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Heiling B, Lehmann T, Müller N, Kloos C, Grimm A, Wolf G, Axer H. What does nerve ultrasound contribute to the evaluation of diabetic polyneuropathy over time? A prospective follow-up observational study of people with type 2 diabetes. Diabetes Res Clin Pract 2025; 222:112115. [PMID: 40113173 DOI: 10.1016/j.diabres.2025.112115] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/17/2025] [Revised: 03/16/2025] [Accepted: 03/17/2025] [Indexed: 03/22/2025]
Abstract
AIMS Studies using peripheral nerve ultrasound have shown moderate nerve enlargement in individuals with diabetic polyneuropathy (DPN). Neither extent, course, consistency, nor clinical significance of this finding is clear. METHOD We examined 156 people with type 2 diabetes mellitus using nerve ultrasound, nerve conduction studies, and clinical scores. 59 received a follow-up examination after one year and 43 patients after two years. RESULTS Increase of cross-sectional area (CSA) of the peripheral nerves in DPN was rather negligible compared to normative cut-off values. The CSA of the median nerve at the forearm and wrist and the ulnar nerve at the upper arm, forearm and wrist were significantly larger in individuals with DPN compared to those without DPN. People with DPN had significant changes in most parameters of nerve conduction studies. Ultrasound measurements over time showed a slight increase in CSA in the median nerve at the carpal tunnel and a slight decrease in the fibular nerve at the fibular head. Ultrasound pattern sum score slightly decreased in the DPN group over two years. CONCLUSIONS Ultrasound measurements alone may be insufficient to detect DPN. Follow up with nerve ultrasound over one or two years was short and did not show substantial changes.
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Affiliation(s)
- Bianka Heiling
- Department of Neurology, Jena University Hospital, Friedrich Schiller University, 07747 Jena, Germany; Clinician Scientist Program OrganAge, Jena University Hospital, 07747 Jena, Germany.
| | - Thomas Lehmann
- Institute of Medical Statistics, Computer and Data Sciences, Jena University Hospital, Jena, Germany
| | - Nicolle Müller
- Department of Internal Medicine III, Jena University Hospital, Friedrich Schiller University, 07747 Jena, Germany
| | - Christof Kloos
- Department of Internal Medicine III, Jena University Hospital, Friedrich Schiller University, 07747 Jena, Germany
| | - Alexander Grimm
- Department of Neurology, Tuebingen University Hospital, 72076 Tuebingen, Germany
| | - Gunter Wolf
- Department of Internal Medicine III, Jena University Hospital, Friedrich Schiller University, 07747 Jena, Germany
| | - Hubertus Axer
- Department of Neurology, Jena University Hospital, Friedrich Schiller University, 07747 Jena, Germany
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Ruffer N, Kleefeld F, Holzer MT, Krusche M, Kötter I, Schneider U, Stenzel W. [Vasculitic involvement of the skeletal muscle and the peripheral nervous system: clinical and neuropathologic perspective]. Z Rheumatol 2025; 84:210-218. [PMID: 39316132 PMCID: PMC11965222 DOI: 10.1007/s00393-024-01567-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/07/2024] [Indexed: 09/25/2024]
Abstract
The peripheral nervous system is a classic target organ in systemic vasculitis. In addition, the skeletal muscle can also be affected. Myalgia, muscle weakness and sensory deficits are typical signs, which can lead to severe functional limitations and impaired of quality of life. Vasculitic involvement of the skeletal muscle (vasculitic myopathy [VM]) and peripheral nerves (vasculitic neuropathy [VN]) occurs predominantly in polyarteritis nodosa and small-vessel vasculitis. VM presents with elevated markers of inflammation and is typically characterized by immobilizing myalgia with normal creatine kinase activity and diffuse or patchy areas of hyperintensity on T2-weighted MRI ("MRI myositis without myositis"). In VN, sensor motor deficits predominantly affect the lower extremity in the area supplied by several peripheral nerves (e.g., mononeuritis multiplex) with acute to subacute history. The histopathological examination of nerve and muscle biopsies is the gold standard for the diagnosis of vasculitic manifestations and has a significant impact on the therapeutic approach.
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Affiliation(s)
- Nikolas Ruffer
- III. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland.
| | - Felix Kleefeld
- Klinik für Neurologie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Marie-Therese Holzer
- III. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland
| | - Martin Krusche
- III. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland
| | - Ina Kötter
- III. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland
- Klinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt, Bad Bramstedt, Deutschland
| | - Udo Schneider
- Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Charité - Universitätsmedizin, Berlin, Deutschland
| | - Werner Stenzel
- Institut für Neuropathologie, Charité - Universitätsmedizin, Berlin, Deutschland
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Heiling B, Kneer K, He W, Lehmann T, Müller N, Kloos C, Grimm A, Axer H. Nerve ultrasound helps to distinguish CIDP patients with diabetes from patients with diabetic polyneuropathy. Sci Rep 2024; 14:30504. [PMID: 39681614 DOI: 10.1038/s41598-024-82235-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/05/2024] [Accepted: 12/03/2024] [Indexed: 12/18/2024] Open
Abstract
Diabetic polyneuropathy (DPN) shares overlapping clinical and electrodiagnostic features with chronic inflammatory demyelinating polyneuropathy (CIDP), which complicates the differential diagnosis of CIDP in diabetic patients. 32 patients with diabetes mellitus and CIDP, 68 patients with CIDP without diabetes, 83 patients with DPN, and 28 diabetic patients without polyneuropathy were examined using clinical scores (Overall Neuropathy Limitation Scale (ONLS), Neuropathy Symptom Score, Neuropathy Deficit Score), nerve conduction studies, and nerve ultrasound (Ultrasound Pattern Sum Score (UPSS)). The ONLS was significantly higher in the CIDP patients with diabetes than in DPN (median [interquartile range]: 4.0 [3.0] vs. 0 [1.0], p < 0.001) as well as the UPSS (4.0 [6.0] vs. 0 [2.9], p < 0.001). Multiple binary logistic regression revealed UPSS and ONLS as statistically significant predictors to differentiate between CIDP with diabetes and DPN. Receiver operating characteristic curve analysis showed the ONLS with an area under the curve (AUC) of 0.918 (95% CI: 0.868-0.0.967, p < 0.001). The UPSS total score had an AUC of 0.826 (95% CI: 0.743-0.909, p < 0.001). An UPSS ≥ 2.5 had a sensitivity of 77.4% and a specificity of 68.7% to detect CIDP. An ONLS ≥ 1.5 had a sensitivity of 87.1% and a specificity of 81.9% to detect CIDP. ROC curve analysis of a composite score of ONLS and UPSS revealed an AUC of 0.959 (95% CI: 0.928-0.991, p < 0.001). CIDP is an important differential diagnosis in people with diabetes mellitus. This study reports that the UPSS is well suited to differentiate between DPN and CIDP.
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Affiliation(s)
- Bianka Heiling
- Department of Neurology, Jena University Hospital, Friedrich Schiller University, 07747, Jena, Germany.
- Clinician Scientist Program OrganAge, Jena University Hospital, 07747, Jena, Germany.
| | - Katharina Kneer
- Department of Neurology, Tuebingen University Hospital, 72076, Tuebingen, Germany
| | - Winnie He
- Department of Neurology, Jena University Hospital, Friedrich Schiller University, 07747, Jena, Germany
| | - Thomas Lehmann
- Center for Clinical Studies, Jena University Hospital, Jena, Germany
| | - Nicolle Müller
- Department of Internal Medicine III, Jena University Hospital, Friedrich Schiller University, 07747, Jena, Germany
| | - Christof Kloos
- Department of Internal Medicine III, Jena University Hospital, Friedrich Schiller University, 07747, Jena, Germany
| | - Alexander Grimm
- Department of Neurology, Tuebingen University Hospital, 72076, Tuebingen, Germany
| | - Hubertus Axer
- Department of Neurology, Jena University Hospital, Friedrich Schiller University, 07747, Jena, Germany
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Kwon EH, Steininger J, Scherbaum R, Gold R, Pitarokoili K, Tönges L. Large-fiber neuropathy in Parkinson's disease: a narrative review. Neurol Res Pract 2024; 6:51. [PMID: 39465424 PMCID: PMC11514528 DOI: 10.1186/s42466-024-00354-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/12/2024] [Accepted: 10/09/2024] [Indexed: 10/29/2024] Open
Abstract
BACKGROUND Numerous studies reported a higher prevalence of polyneuropathy (PNP) in patients with Parkinson's disease (PD) compared to the general population. Importantly, PNP symptoms can aggravate both motor and sensory disturbances in PD patients and negatively impact the disease course. Recent analyses indicate distinct PNP patterns in PD. MAIN TEXT This review aims to provide an overview of the current insights into etiological factors, diagnostic methods, and management strategies of large fiber neuropathy in PD. Despite the higher prevalence, the causes of PNP in PD are still not fully understood. A genetic predisposition can underlie PNP onset in PD. Main research attention is focused on long-term levodopa exposure which is suggested to increase PNP risk by depletion of methylation cofactors such as vitamin B12 and accumulation of homocysteine that altogether can alter peripheral nerve homeostasis. Beyond a potential "iatrogenic" cause, alpha-synuclein deposition has been detected in sural nerve fibers that could contribute to peripheral neuronal degeneration as part of the systemic manifestation of PD. Whereas mild axonal sensory PNP predominates in PD, a considerable proportion of patients also show motor and upper limb nerve involvement. Intriguingly, a correlation between PNP severity and PD severity has been demonstrated. Therefore, PNP screening involving clinical and instrument-based assessments should be implemented in the clinical routine for early detection and monitoring. Given the etiological uncertainty, therapeutic or preventive options remain limited. Vitamin supplementation and use of catechol-O-methyltransferase-inhibitors can be taken into consideration. CONCLUSION PNP is increasingly recognized as a complicating comorbidity of PD patients. Long-term, large-scale prospective studies are required to elucidate the causative factors for the development and progression of PD-associated PNP to optimize treatment approaches. The overall systemic role of "idiopathic" PNP in PD and a putative association with the progression of neurodegeneration should also be investigated further.
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Affiliation(s)
- Eun Hae Kwon
- Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany.
| | - Julia Steininger
- Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
| | - Raphael Scherbaum
- Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
| | - Ralf Gold
- Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
- Neurodegeneration Research, Centre for Protein Diagnostics (ProDi), Ruhr-University, Bochum, Germany
| | - Kalliopi Pitarokoili
- Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
| | - Lars Tönges
- Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
- Neurodegeneration Research, Centre for Protein Diagnostics (ProDi), Ruhr-University, Bochum, Germany
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De Martino Luppi A, Ferreira GE, Borges IS, Antunes DE, Araújo L, dos Santos DF, Nogueira-Barbosa MH, Goulart IMB. Role of multisegmental nerve ultrasound in the diagnosis of leprosy neuropathy. PLoS One 2024; 19:e0305808. [PMID: 39024363 PMCID: PMC11257231 DOI: 10.1371/journal.pone.0305808] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2024] [Accepted: 06/05/2024] [Indexed: 07/20/2024] Open
Abstract
INTRODUCTION/AIMS Leprosy is the most common treatable peripheral neuropathy worldwide. The detection of peripheral nerve impairment is essential for its diagnosis and treatment, in order to prevent stigmatizing deformities and disabilities. This study was performed to identify neural thickening through multisegmental ultrasound (US). METHODS We assessed US measurements of cross-sectional areas (CSAs) of ulnar, median and tibial nerves at two points (in the osteofibrous tunnel and proximal to the tunnel), and also of the common fibular nerve at the fibular head level in 53 leprosy patients (LP), and compared with those of 53 healthy volunteers (HV), as well as among different clinical forms of leprosy. RESULTS US evaluation detected neural thickening in 71.1% (38/53) of LP and a mean number of 3.6 enlarged nerves per patient. The ulnar and tibial were the most frequently affected nerves. All nerves showed significantly higher measurements in LP compared with HV, and also greater asymmetry, with significantly higher values for ulnar and tibial nerves. We found significant CSAs differences between tunnel and pre-tunnel points for ulnar and tibial nerves, with maximum values proximal to the tunnel. All clinical forms of leprosy evaluated showed neural enlargement through US. DISCUSSION Our findings support the role of multisegmental US as a useful method for diagnosing leprosy neuropathy, revealing that asymmetry, regional and non-uniform thickening are characteristics of the disease. Furthermore, we observed that neural involvement is common in different clinical forms of leprosy, reinforcing the importance of including US evaluation of peripheral nerves in the investigation of all leprosy patients.
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Affiliation(s)
- Andrea De Martino Luppi
- National Reference Center for Sanitary Dermatology and Leprosy, Clinics’ Hospital, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
- Radiology Division, Clinics’ Hospital, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
- Postgraduate Program in Health Sciences, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
| | - Guilherme Emílio Ferreira
- Radiology Division, Clinics’ Hospital, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
| | - Isabella Sabião Borges
- National Reference Center for Sanitary Dermatology and Leprosy, Clinics’ Hospital, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
| | - Douglas Eulálio Antunes
- National Reference Center for Sanitary Dermatology and Leprosy, Clinics’ Hospital, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
- Postgraduate Program in Health Sciences, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
| | - Lúcio Araújo
- Department of Mathematics, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
| | - Diogo Fernandes dos Santos
- National Reference Center for Sanitary Dermatology and Leprosy, Clinics’ Hospital, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
- Postgraduate Program in Health Sciences, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
| | - Marcello Henrique Nogueira-Barbosa
- Radiology Division, Department of Medical Imaging, Hematology and Clinical Oncology, Ribeirão Preto Medical School, University of São Paulo Ribeirão Preto, São Paulo, Brazil
| | - Isabela Maria Bernardes Goulart
- National Reference Center for Sanitary Dermatology and Leprosy, Clinics’ Hospital, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
- Postgraduate Program in Health Sciences, School of Medicine, Federal University of Uberlândia (UFU), Uberlândia, MG, Brazil
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Nasr-Eldin YK, Cartwright MS, Hamed A, Ali LH, Abdel-Nasser AM. Neuromuscular Ultrasound in Polyneuropathies. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2024; 43:1181-1198. [PMID: 38504399 DOI: 10.1002/jum.16447] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/29/2023] [Revised: 02/20/2024] [Accepted: 02/25/2024] [Indexed: 03/21/2024]
Abstract
Neuromuscular ultrasound is a painless, radiation-free, high-resolution imaging technique for assessing the peripheral nervous system. It can accurately depict changes in the nerves and muscles of individuals with neuromuscular conditions, and it is therefore a robust diagnostic tool for the assessment of individuals with polyneuropathies. This review will outline the typical ultrasonographic changes found in a wide variety of polyneuropathies. In general, demyelinating conditions result in greater nerve enlargement than axonal conditions, and acquired conditions result in more patchy nerve enlargement compared to diffuse nerve enlargement in hereditary conditions. This review is data-driven, but more nuanced anecdotal findings are also described. The overall goal of this paper is to provide clinicians with an accessible review of the ultrasonographic approaches and findings in a wide variety of polyneuropathies.
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Affiliation(s)
| | - Michael S Cartwright
- Neurology Department, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA
| | - Ahmed Hamed
- Rheumatology and Rehabilitation Department, Minia University, Minia, Egypt
| | - Lamia Hamdy Ali
- Clinical Pathology Department, Minia University, Minia, Egypt
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Kneer K, Straub S, Wittlinger J, Stahl JH, Winter N, Timmann D, Schöls L, Synofzik M, Bender F, Grimm A. Neuropathy in ARSACS is demyelinating but without typical nerve enlargement in nerve ultrasound. J Neurol 2024; 271:2494-2502. [PMID: 38261029 PMCID: PMC11055797 DOI: 10.1007/s00415-023-12159-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/08/2023] [Revised: 12/11/2023] [Accepted: 12/12/2023] [Indexed: 01/24/2024]
Abstract
BACKGROUND To specify peripheral nerve affection in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) by correlating high-resolution nerve ultrasound and nerve conduction studies. METHODS We assessed a cohort of 11 ARSACS patients with standardized nerve conduction studies and high-resolution ultrasound of peripheral nerves and compared nerve ultrasound findings to a healthy control group matched for age, sex, size and weight. RESULTS Mean age of patients was 39.0 (± 14.1) years and disease duration at assessment 30.6 (± 12.5) years. All patients presented with a spasticity, ataxia and peripheral neuropathy. Neuropathy appeared to be primarily demyelinating in 9/11 cases and was not classifiable in 2/11 cases due to not evocable potentials. Nerve ultrasound revealed a normal ultrasound pattern sum score (UPSS) in each ARSACS patient and no significant nerve enlargement compared to the control group. CONCLUSIONS Peripheral neuropathy in ARSACS showed primarily demyelinating rather than axonal characteristics and presented without nerve enlargement. As demyelinating neuropathies do commonly present enlarged nerves we recommend further genetic testing of the SACS gene in patients who present with this combination of demyelinating neuropathy without nerve enlargement. ARSACS cases that initially presented only with neuropathy without spasticity or ataxia and therefore were misdiagnosed as Charcot-Marie-Tooth disease are supporting this suggestion.
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Affiliation(s)
- Katharina Kneer
- Department of Epileptology, Center of Neurology, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany.
- Hertie Institute for Clinical Brain Research, Eberhard-Karls University Tübingen, Tübingen, Germany.
| | - Stephanie Straub
- Department of Epileptology, Center of Neurology, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany
- Hertie Institute for Clinical Brain Research, Eberhard-Karls University Tübingen, Tübingen, Germany
| | - Julia Wittlinger
- Department of Epileptology, Center of Neurology, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany
- Hertie Institute for Clinical Brain Research, Eberhard-Karls University Tübingen, Tübingen, Germany
| | - Jan-Hendrik Stahl
- Department of Epileptology, Center of Neurology, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany
- Hertie Institute for Clinical Brain Research, Eberhard-Karls University Tübingen, Tübingen, Germany
| | - Natalie Winter
- Department of Epileptology, Center of Neurology, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany
- Hertie Institute for Clinical Brain Research, Eberhard-Karls University Tübingen, Tübingen, Germany
| | - Dagmar Timmann
- Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), Essen University Hospital, University of Duisburg-Essen, Essen, Germany
| | - Ludger Schöls
- Hertie Institute for Clinical Brain Research, Eberhard-Karls University Tübingen, Tübingen, Germany
- Department of Neurodegenerative Diseases, Center of Neurology, University of Tuebingen, Tuebingen, Germany
- German Center for Neurodegenerative Diseases (DZNE), Tuebingen, Germany
| | - Matthis Synofzik
- Hertie Institute for Clinical Brain Research, Eberhard-Karls University Tübingen, Tübingen, Germany
- Department of Neurodegenerative Diseases, Center of Neurology, University of Tuebingen, Tuebingen, Germany
- German Center for Neurodegenerative Diseases (DZNE), Tuebingen, Germany
| | - Friedemann Bender
- Department of Neurodegenerative Diseases, Center of Neurology, University of Tuebingen, Tuebingen, Germany
- Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), Essen University Hospital, University of Duisburg-Essen, Essen, Germany
- German Center for Neurodegenerative Diseases (DZNE), Tuebingen, Germany
- Kinder- Und Jugend Psychiatrie Klink Esslingen, Esslingen, Germany
| | - Alexander Grimm
- Department of Epileptology, Center of Neurology, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany
- Hertie Institute for Clinical Brain Research, Eberhard-Karls University Tübingen, Tübingen, Germany
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Li D, Yu H, Zhou M, Fan W, Guan Q, Li L. Case report: Chronic inflammatory demyelinating polyneuropathy superimposed on Charcot-Marie-tooth type 1A disease after SARS-CoV-2 vaccination and COVID-19 infection. Front Neurol 2024; 15:1358881. [PMID: 38651106 PMCID: PMC11033519 DOI: 10.3389/fneur.2024.1358881] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2023] [Accepted: 03/28/2024] [Indexed: 04/25/2024] Open
Abstract
Background There is growing evidence that severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) or COVID-19 infection is associated with the development of immune mediated neuropathies like chronic inflammatory demyelinating polyneuropathy (CIDP), but the impact of SARS-CoV-2 vaccination and COVID-19 infection on genetic disorders such as Charcot-MarieTooth (CMT) remains unclear. Case presentation A 42-year-old male with occulted CMT neuropathy type lA (CMT1A) who developed limb numbness and weakness after the second SARS-CoV-2-vaccination was confirmed by identifying characteristic repeats in the p11.2 region of chromosome 17. Due to the progressive deterioration of muscle strength over 8 weeks, limb atrophy, moderately elevated protein counts in the cerebrospinal fluid, and significant improvement with intravenous human immunoglobulin, which were characteristic of acquired inflammatory neuropathies, he was eventually diagnosed with CIDP superimposed on CMT1A. However, after a three-month plateau, the patient contracted COVID-19, which led to repeated and worsening symptoms of limb weakness and atrophy, thus was diagnosed with a recurrence of CIDP and treated with Intravenous immunoglobulin and methylprednisolone 500 mg/d for 5 consecutive days, followed by oral prednisone and mycophenolate mofetil tablets. On 2 month follow-up, he exhibited remarkable clinical improvement and could walk independently with rocking gait. After 1 year of follow-up, the patient's condition was stable without further change. Conclusion Our case indicates that CMT1A can deteriorate after SARS-CoV-2 vaccination. Thus, SARS-CoV-2 vaccination should be considered a potential predisposing factor for CMT1A worsening. The possible superposition of CMTIA and CIDP in the context of SARS-CoV-2 infection or immunity suggests that any clinical exacerbation in patients with CMT1A should be carefully evaluated to rule out treatable superposition inflammation. In addition, electrophysiological and imaging examination of the proximal nerves, such as the axillary nerve, is helpful for the diagnosis of CIDP.
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Affiliation(s)
| | | | | | | | | | - Li Li
- Department of Neurology, Ningbo No 2 Hospital, Ningbo, Zhejiang, China
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9
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Nasr-Eldin YK, Cartwright MS, Hamed A, Ali LH, Abdel-Nasser AM. Evaluation of Serum Neurofilament Light Chain and Nerve Ultrasound in Diabetic Neuropathy. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2024; 43:683-696. [PMID: 38124499 DOI: 10.1002/jum.16393] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/06/2023] [Revised: 11/14/2023] [Accepted: 11/28/2023] [Indexed: 12/23/2023]
Abstract
OBJECTIVE To assess the role of serum neurofilament light chain (NfL) levels in individuals with diabetic polyneuropathy (DPN) compared with controls, as well as to highlight the different sonographic changes in DPN and determine if NfL correlates with sonographic, clinical, and functional parameters. METHODS Diabetic individuals with signs or symptoms consistent with peripheral nerve involvement were recruited. They were evaluated by examination, functional neuropathy severity scores, laboratory assessments (including NfL), nerve conduction studies (NCS), and ultrasound. Ultrasound was performed of the bilateral median, ulnar, tibial, fibular, sural, and vagus nerves, and cervical roots 5 and 6. Results were compared with age, sex, and body mass index matched healthy controls. RESULTS A total of 320 nerves from 20 patients and 480 nerves from 30 controls were evaluated. NfL was significantly elevated in those with diabetes with a mean and standard deviation of 6.95 ± 2.95 pg/mL in the diabetic group and 2.83 ± 0.77 pg/mL in controls (P < .001). Nerve cross-sectional area and serum NfL levels correlated significantly with clinical and functional parameters and with each other (P < .05). CONCLUSION Individuals with DPN have significantly higher NfL levels than healthy controls, and NfL levels correlate with ultrasonographic parameters. These findings may be useful for the diagnosis, prognosis, and disease monitoring of those with DPN, though further exploration is needed.
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Affiliation(s)
| | - Michael S Cartwright
- Neurology Department, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA
| | - Ahmed Hamed
- Rheumatology and Rehabilitation Department, Minia University, Minya, Egypt
| | - Lamia Hamdy Ali
- Clinical Pathology Department, Minia University, Minya, Egypt
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10
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Park JY, Lee MJ, Kim HJ, Nam JW. Evaluation of peripheral nerve injury according to the severity of damage using 18F-FDG PET/MRI in a rat Model of sciatic nerve injury. Neurol Res 2024; 46:356-366. [PMID: 38402903 DOI: 10.1080/01616412.2024.2321774] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2023] [Accepted: 02/17/2024] [Indexed: 02/27/2024]
Abstract
OBJECTIVES We ascertained that the PET scan may be a valuable imaging modality for the noninvasive, objective diagnosis of neuropathic pain caused by peripheral nerve injury through the previous study. This study aimed to assess peripheral nerve damage according to severity using18F-FDG PET/MRI of the rat sciatic nerve. METHODS Eighteen rats were divided into three groups: 30-second (G1), 2-minute (G2), and 5-minute (G3) crushing injuries. The severity of nerve damage was measured in the third week after the crushing injury using three methods: the paw withdrawal threshold test (RevWT), standardized uptake values on PET (SUVR), and intensity analysis on immunohistochemistry (IntR). RESULTS There were significant differences between G1 and G3 in both SUVR and IntR (p = 0.012 and 0.029, respectively), and no significant differences in RevWT among the three groups (p = 0.438). There was a significant difference in SUVR (p = 0.012), but no significant difference in IntR between G1 and G2 (p = 0.202). There was no significant difference between G2 and G3 in SUVR and IntR (p = 0.810 and 0.544, respectively). DISCUSSION Although PET did not show results consistent with those of immunohistochemistry in all respects, this study demonstrated that PET uptake tended to increase with severe nerve damage. If this research is supplemented by further experiments, PET/MRI can be used as an effective diagnostic modality.
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Affiliation(s)
- Jong Yeol Park
- Department of Oral and Maxillofacial Surgery, College of Dentistry, Yonsei University, Seoul, Republic of Korea
| | - Mi Jee Lee
- Department of Oral and Maxillofacial Surgery, College of Dentistry, Yonsei University, Seoul, Republic of Korea
| | - Hyung Jun Kim
- Department of Oral and Maxillofacial Surgery, College of Dentistry, Yonsei University, Seoul, Republic of Korea
| | - Jung Woo Nam
- Department of Oral and Maxillofacial Surgery, Sanbon Dental Hospital, Wonkwang University, Gunpo-si, Republic of Korea
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11
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Laure Inghilleri M, Alonso S, Moron H, Ruiz H, Bastide S, Coudray S. The value of ultrasound-guidance of nerves and muscles for patient tolerance and parameters electrodiagnostic studies. Clin Neurophysiol Pract 2024; 9:78-84. [PMID: 38357416 PMCID: PMC10864755 DOI: 10.1016/j.cnp.2024.01.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/29/2023] [Revised: 01/05/2024] [Accepted: 01/09/2024] [Indexed: 02/16/2024] Open
Abstract
Objective To assess impact of ultrasound guidance (USG) on patient's perception of nerve conduction studies (NCS). Methods In this single-center, randomized, sham-controlled, parallel, single-blind trial, we evaluated ultrasound (US) in identifying NCS stimulation site. Consecutive adults (18-80 old) without neuropathy referred for NCS were electronically randomized 1:1 to USG or Sham US. The primary outcome was sensory supramaximal intensity (SSMI) for each site/nerve; motor supramaximal intensity (MSMI), amplitudes, number of non-routine muscle punctured, Visual Analogue Scale (VAS), satisfaction were secondary outcomes. Results 290 participants were randomized, with 145 in the USG and 144 Sham US groups, respectively. No difference in SSMI, CMAP or SNAP, VAS, satisfaction was recorded. With USG, the median at the elbow and fibular MMSI were lower (p = 0.04; p = 0.02). With normal NCS or overweight and obese subgroups patients had lower median SSMI (p = 0.05/ p = 0.02), higher median and sural SNAP with normal NCS (p = 0.04; p = 0.007) and the sural SNAP for the expert US subgroup (p = 0.02). Conclusions USG is useful for nerves, that are anatomically variable or in obesity. The sural SNAP gain with US in the normal NCS subgroup could facilitate routine NCS. Significance In standard NCS the USG does not modify the patient's tolerance.Trial Registration: clinicaltrials.gov (NCT03868189).
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Affiliation(s)
- Marie Laure Inghilleri
- Neuromuscular Disorder Unit, Department of Neurophysiology, CHU Nîmes, Univ Montpellier, Nîmes, France
| | - Sandrine Alonso
- Department of Biostatistics, Clinical Epidemiology, Public Health and Innovation in Methodology (BESPIM), CHU Nîmes, Univ Montpellier, Nîmes, France
| | - Hélène Moron
- Neuromuscular Disorder Unit, Department of Neurophysiology, CHU Nîmes, Univ Montpellier, Nîmes, France
| | - Hector Ruiz
- Neuromuscular Disorder Unit, Department of Neurophysiology, CHU Nîmes, Univ Montpellier, Nîmes, France
| | - Sophie Bastide
- Department of Biostatistics, Clinical Epidemiology, Public Health and Innovation in Methodology (BESPIM), CHU Nîmes, Univ Montpellier, Nîmes, France
| | - Sarah Coudray
- Neuromuscular Disorder Unit, Department of Neurophysiology, CHU Nîmes, Univ Montpellier, Nîmes, France
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12
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Telleman JA, Sneag DB, Visser LH. The role of imaging in focal neuropathies. HANDBOOK OF CLINICAL NEUROLOGY 2024; 201:19-42. [PMID: 38697740 DOI: 10.1016/b978-0-323-90108-6.00001-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/05/2024]
Abstract
Electrodiagnostic testing (EDX) has been the diagnostic tool of choice in peripheral nerve disease for many years, but in recent years, peripheral nerve imaging has been used ever more frequently in daily clinical practice. Nerve ultrasound and magnetic resonance (MR) neurography are able to visualize nerve structures reliably. These techniques can aid in localizing nerve pathology and can reveal significant anatomical abnormalities underlying nerve pathology that may have been otherwise undetected by EDX. As such, nerve ultrasound and MR neurography can significantly improve diagnostic accuracy and can have a significant effect on treatment strategy. In this chapter, the basic principles and recent developments of these techniques will be discussed, as well as their potential application in several types of peripheral nerve disease, such as carpal tunnel syndrome (CTS), ulnar neuropathy at the elbow (UNE), radial neuropathy, brachial and lumbosacral plexopathy, neuralgic amyotrophy (NA), fibular, tibial, sciatic, femoral neuropathy, meralgia paresthetica, peripheral nerve trauma, tumors, and inflammatory neuropathies.
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Affiliation(s)
- Johan A Telleman
- Department of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Tilburg, The Netherlands
| | - Darryl B Sneag
- Department of Radiology and Imaging, Hospital for Special Surgery, New York, NY, United States
| | - Leo H Visser
- Department of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Tilburg, The Netherlands.
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13
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Bürkle E, Ruff C, Lindig T, Nägele T, Hauser TK, Grimm A, Winter N. [Choosing the right imaging for the diagnostics and assessment of the course of peripheral nerve injuries]. DER NERVENARZT 2023; 94:1087-1096. [PMID: 37848647 DOI: 10.1007/s00115-023-01550-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 08/25/2023] [Indexed: 10/19/2023]
Abstract
BACKGROUND Nerve injuries are a frequent problem in routine clinical practice and require intensive interdisciplinary care. OBJECTIVE The current status of imaging to confirm the diagnosis of nerve injuries is described. The role of high-resolution ultrasound and magnetic resonance imaging (MRI) in the diagnostics and follow-up of peripheral nerve injuries is elaborated. MATERIAL AND METHODS Review of the current state of imaging to confirm the diagnosis of nerve injuries. RESULTS Depending on the suspected site of damage, the primary domain of magnetic resonance (MR) imaging (MR neurography) is injuries in the region of the spine, nerve roots, brachial plexus and lumbar plexus, pelvis and proximal thigh. In contrast, in other peripheral nerve lesions of the extremities the advantages of high-resolution nerve ultrasound in a dynamic setting predominate. The MR neurography is indicated here, especially in the frequent bottleneck syndromes and only in very isolated and selected cases. CONCLUSION In addition to a correct anatomical assignment, the timely decision for a possible intervention and the appropriate concomitant treatment are an important basis for a favorable prognosis of nerve injuries. Imaging techniques should therefore be used early in the diagnostics and follow-up controls of peripheral nerve injuries.
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Affiliation(s)
- Eva Bürkle
- Abteilung für diagnostische und interventionelle Neuroradiologie, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Deutschland
| | - Christer Ruff
- Abteilung für diagnostische und interventionelle Neuroradiologie, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Deutschland.
| | - Tobias Lindig
- Abteilung für diagnostische und interventionelle Neuroradiologie, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Deutschland
| | - Thomas Nägele
- Abteilung für diagnostische und interventionelle Neuroradiologie, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Deutschland
- Radiologie Sport Stuttgart, Stuttgart-Bad Cannstatt, Deutschland
| | - Till-Karsten Hauser
- Abteilung für diagnostische und interventionelle Neuroradiologie, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Deutschland
| | - Alexander Grimm
- Abteilung für Neurologie, Universitätsklinikum Tübingen, Tübingen, Deutschland
| | - Natalie Winter
- Abteilung für Neurologie, Universitätsklinikum Tübingen, Tübingen, Deutschland
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14
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Hartinger S, Hammersen J, Leistner NA, Lawson McLean A, Risse C, Senft C, Schütze S, Heiling B, Schwab M, Mäurer I. The role of neuromuscular ultrasound in diagnostics of peripheral neuropathies induced by cytostatic agents or immunotherapies. Acta Neuropathol Commun 2023; 11:187. [PMID: 38012771 PMCID: PMC10683078 DOI: 10.1186/s40478-023-01685-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2023] [Accepted: 11/07/2023] [Indexed: 11/29/2023] Open
Abstract
A relevant number of cancer patients who receive potentially neurotoxic cytostatic agents develop a chemotherapy-induced peripheral neuropathy over time. Moreover, the increasing use of immunotherapies and targeted agents leads to a raising awareness of treatment-associated peripheral neurotoxicity, e.g., axonal and demyelinating neuropathies such as Guillain-Barré-like syndromes. To date, the differentiation of these phenomena from concurrent neurological co-morbidities or (para-)neoplastic nerve affection as well as their longitudinal monitoring remain challenging. Neuromuscular ultrasound (NMUS) is an established diagnostic tool for peripheral neuropathies. Performed by specialized neurologists, it completes clinical and neurophysiological diagnostics especially in differentiation of axonal and demyelinating neuropathies. No generally approved biomarkers of treatment-induced peripheral neurotoxicity have been established so far. NMUS might significantly extend the repertoire of diagnostic and neuromonitoring methods in this growing patient group in short term. In this article, we present enlargements of the dorsal roots both in cytostatic and in immunotherapy-induced neurotoxicity for the first time. We discuss related literature regarding new integrative applications of NMUS for cancer patients by reference to two representative case studies. Moreover, we demonstrate the integration of NMUS in a diagnostic algorithm for suspected peripheral neurotoxicity independently of a certain cancer treatment regimen emphasizing the emerging potential of NMUS for clinical routine in this interdisciplinary field and prospective clinical trials.
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Affiliation(s)
- Stefanie Hartinger
- Department of Neurology, Jena University Hospital, Friedrich Schiller University Jena, Am Klinikum 1, 07747, Jena, Germany.
- Neurooncological Center, Jena University Hospital, Friedrich Schiller University Jena, Jena, Germany.
| | - Jakob Hammersen
- Klinik für Innere Medizin II, Hämatologie, Internistische Onkologie und Palliativmedizin, Universitätsklinikum Jena, Friedrich-Schiller-Universität Jena, Jena, Deutschland
| | - Niklas A Leistner
- Department of Neurology, Jena University Hospital, Friedrich Schiller University Jena, Am Klinikum 1, 07747, Jena, Germany
| | - Anna Lawson McLean
- Neurooncological Center, Jena University Hospital, Friedrich Schiller University Jena, Jena, Germany
- Department of Neurosurgery, Jena University Hospital, Friedrich Schiller University Jena, Jena, Germany
| | - Clemens Risse
- Department of Neurology, Jena University Hospital, Friedrich Schiller University Jena, Am Klinikum 1, 07747, Jena, Germany
| | - Christian Senft
- Neurooncological Center, Jena University Hospital, Friedrich Schiller University Jena, Jena, Germany
- Department of Neurosurgery, Jena University Hospital, Friedrich Schiller University Jena, Jena, Germany
| | - Stefanie Schütze
- Department of Gynecology and Reproductive Medicine, Jena University Hospital, Friedrich Schiller University Jena, Jena, Germany
| | - Bianka Heiling
- Department of Neurology, Jena University Hospital, Friedrich Schiller University Jena, Am Klinikum 1, 07747, Jena, Germany
| | - Matthias Schwab
- Department of Neurology, Jena University Hospital, Friedrich Schiller University Jena, Am Klinikum 1, 07747, Jena, Germany
| | - Irina Mäurer
- Department of Neurology, Jena University Hospital, Friedrich Schiller University Jena, Am Klinikum 1, 07747, Jena, Germany
- Neurooncological Center, Jena University Hospital, Friedrich Schiller University Jena, Jena, Germany
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15
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Serhal A, Lee SK, Michalek J, Serhal M, Omar IM. Role of high-resolution ultrasound and magnetic resonance neurography in the evaluation of peripheral nerves in the upper extremity. J Ultrason 2023; 23:e313-e327. [PMID: 38020515 PMCID: PMC10668945 DOI: 10.15557/jou.2023.0037] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2023] [Accepted: 08/30/2023] [Indexed: 12/01/2023] Open
Abstract
Upper extremity entrapment neuropathies are common conditions in which peripheral nerves are prone to injury at specific anatomical locations, particularly superficial regions or within fibro-osseous tunnels, resulting in pain and potential disability. Although neuropathy is primarily diagnosed clinically by physical examination and electrophysiology, imaging evaluation with ultrasound and magnetic resonance neurography are valuable complementary non-invasive and accurate tools for evaluation and can help define the site and cause of nerve dysfunction which ultimately leads to precise and timely treatment. Ultrasound, which has higher spatial resolution, can quickly and comfortably characterize the peripheral nerves in real time and can evaluate for denervation related muscle atrophy. Magnetic resonance imaging on the other hand provides excellent contrast resolution between the nerves and adjacent tissues, also between pathologic and normal segments of peripheral nerves. It can also assess the degree of muscle denervation and atrophy. As a prerequisite for nerve imaging, radiologists and sonographers should have a thorough knowledge of anatomy of the peripheral nerves and their superficial and deep branches, including variant anatomy, and the motor and sensory territories innervated by each nerve. The purpose of this illustrative article is to review the common neuropathy and nerve entrapment syndromes in the upper extremities focusing on ultrasound and magnetic resonance neurography imaging.
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Affiliation(s)
- Ali Serhal
- Department of Radiology, Northwestern University, Chicago, USA
| | | | - Julia Michalek
- Department of Radiology, Northwestern Memorial Hospital, Chicago, USA
| | - Muhamad Serhal
- Department of Radiology, Northwestern University, Chicago, USA
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16
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Senarai T, Suwannakhan A, Pratipanawatr T, Yammine K, Yurasakpong L, Sathapornsermsuk T, Janta S, Kittiboonya A. Normative Reference Values of the Tibial Nerve in Healthy Individuals Using Ultrasonography: A Systematic Review and Meta-Analysis. J Clin Med 2023; 12:6186. [PMID: 37834829 PMCID: PMC10573196 DOI: 10.3390/jcm12196186] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2023] [Revised: 09/23/2023] [Accepted: 09/23/2023] [Indexed: 10/15/2023] Open
Abstract
BACKGROUND High-resolution ultrasound of the tibial nerve has been used for screening of several neurologic disorders, but normative reference values of tibial nerve cross-sectional areas (CSA) have not been well established. Thus, the present meta-analysis was performed to generate normative estimates of tibial nerve CSA at various sites of the lower limb based on ultrasonography. METHODS Google Scholar, Scopus and PubMed were searched for potential studies. Studies were required to report tibial nerve CSA in healthy individuals to be included. A random-effect meta-analysis was performed to calculate tibial nerve CSA values. Subgroup and statistical analyses were performed to study covariates. RESULTS Forty-eight eligible articles consisting of 2695 limbs were included. The average tibial nerve CSA was found to be 10.9 mm2 at the ankle (95% CI: 9.9-11.8) and should not exceed 11.8 mm2 in healthy adults. At the popliteal fossa, the overall CSA was 21.7 mm2 (95% CI: 17.5-25.8) in healthy adults. At both sites, the average tibial nerve CSA was significantly larger in adults than in children, and the differences by geographical region were not statistically significant. At the ankle, tibial nerve CSA increased with age and body mass index, while at the popliteal fossa it increased with age and weight. CONCLUSIONS our findings indicate that the tibial nerve varied not only along its course but also among sub-variables. Establishing normal references values of tibial nerve CSA is helpful to differentiate healthy from diseased tibial nerves such as in diabetic peripheral neuropathy or tarsal tunnel syndrome.
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Affiliation(s)
- Thanyaporn Senarai
- Microscopy Unit, Department of Anatomy, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand;
| | - Athikhun Suwannakhan
- Department of Anatomy, Faculty of Science, Mahidol University, Bangkok 10400, Thailand;
- In Silico and Clinical Anatomy Research Group (iSCAN), Bangkok 10400, Thailand
| | - Thongchai Pratipanawatr
- Department of Internal Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand;
| | - Kaissar Yammine
- Department of Orthopedic and Trauma Surgery, Lebanese American University Medical Center—Rizk Hospital, Beirut 11-3288, Lebanon;
- The Center for Evidence-Based Anatomy, Sports and Orthopedic Research, Lebanese American University, Byblos 1102-2801, Lebanon
| | - Laphatrada Yurasakpong
- Department of Anatomy, Faculty of Science, Mahidol University, Bangkok 10400, Thailand;
- In Silico and Clinical Anatomy Research Group (iSCAN), Bangkok 10400, Thailand
| | | | - Sirorat Janta
- Anatomy Unit, Department of Medical Science, Faculty of Science, Rangsit University, Pathumthani 12000, Thailand;
| | - Achiraya Kittiboonya
- Centre for Anatomy and Human Identification (CAHID), School of Science and Engineering, University of Dundee, Dundee DD1 4HN, UK;
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17
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Iguchi N, Mano T, Iwasa N, Kikutsuji N, Saito K, Sugie K. An Ultrasonographic Evaluation for the Early Detection of Nerve Root Changes in Herpes Zoster-associated Motor Paresis. Intern Med 2023; 62:903-907. [PMID: 35989267 PMCID: PMC10076138 DOI: 10.2169/internalmedicine.0039-22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/28/2022] [Accepted: 07/03/2022] [Indexed: 11/06/2022] Open
Abstract
We herein report a case of herpes zoster complicated by right-arm paralysis, wherein cervical nerve root ultrasonography enabled the early diagnosis and a therapeutic efficacy evaluation. A 71-year-old man developed progressive weakness in the muscles innervated by the right C5-6 nerve root following the appearance of a painful rash. Cervical nerve root ultrasonography revealed C5-6 nerve root inflammatory swelling. Methylprednisolone pulse therapy and subsequent oral prednisolone therapy gradually improved the muscle weakness. At three weeks following admission, ultrasonography revealed C5-6 nerve root inflammatory swelling improvement. Ultrasonography may aid in the early detection of nerve root inflammatory swelling and help monitor treatment efficacy.
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Affiliation(s)
| | - Tomoo Mano
- Department of Neurology, Nara Medical University, Japan
- Department of Rehabilitation Medicine, Nara Medical University, Japan
| | - Naoki Iwasa
- Department of Neurology, Nara Medical University, Japan
| | | | - Kozue Saito
- Department of Neurology, Nara Medical University, Japan
| | - Kazuma Sugie
- Department of Neurology, Nara Medical University, Japan
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18
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El Houjeiry E, Coudray S, Thouvenot E, Ion IM. Spinal cord lesion mimicking a dysimmune myelitis revealing CANVAS syndrome. J Spinal Cord Med 2023; 46:332-336. [PMID: 35235501 PMCID: PMC9987767 DOI: 10.1080/10790268.2022.2033936] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/19/2022] Open
Abstract
CONTEXT Posterior spinal cord lesions are found in patients with ganglionopathy. These are normally found in later stages of the neuronopathy as a consequence of dorsal root ganglia degeneration. Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome (CANVAS) is an emerging neurological disorder. Myelitis lesions have been described in confirmed CANVAS cases. FINDINGS We describe a case of a 68-year-old woman with slowly progressive ataxia with paresthesia. Laboratory tests were normal. Total spine MRI showed a C4 posterior spinal cord lesion. Lumbar puncture was positive for oligoclonal bands with normal IgG index and protein level. Paraneoplastic antibodies were not detected. Electromyography showed nonlength dependent sensory neuropathy. The patient was treated with intravenous immunoglobulin for suspected dysimmune myelitis. Over 6 years, she progressively developed other neurological manifestations evoking CANVAS. Nerve conduction study showed isolated sensory impairment over the years and peripheral nerve ultrasound revealed abnormally small nerves. Further genetic testing confirmed the diagnosis. CONCLUSION This is the first case of CANVAS syndrome presenting initially with an isolated spinal cord lesion mimicking dysimmune myelitis. The purpose of this case report is to add to the current literature about this evolving neurological syndrome and to aid clinicians in their diagnostic approach in clinical practice.
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Affiliation(s)
| | - Sarah Coudray
- Department of Neurophysiology, Nimes University Hospital, Nimes, France
| | - Eric Thouvenot
- Department of Neurology, Nimes University Hospital, Nimes, France.,IGF, Univ. Montpellier, CNRS, INSERM, Montpellier, France
| | - Ioana Maria Ion
- Department of Neurology, Nimes University Hospital, Nimes, France
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Tozza S, Spina E, Iovino A, Iodice R, Dubbioso R, Ruggiero L, Nolano M, Manganelli F. Value of Antibody Determinations in Chronic Dysimmune Neuropathies. Brain Sci 2022; 13:37. [PMID: 36672019 PMCID: PMC9856104 DOI: 10.3390/brainsci13010037] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2022] [Revised: 12/12/2022] [Accepted: 12/21/2022] [Indexed: 12/28/2022] Open
Abstract
Chronic dysimmune neuropathies encompass a group of neuropathies that share immune-mediated pathomechanism. Chronic dysimmune antibody-related neuropathies include anti-MAG neuropathy, multifocal motor neuropathy, and neuropathies related to immune attack against paranodal antigens. Such neuropathies exhibit distinguishing pathomechanism, clinical and response to therapy features with respect to chronic inflammatory demyelinating polyradiculoneuropathy and its variants, which represent the most frequent form of chronic dysimmune neuropathy. This narrative review provides an overview of pathomechanism; clinical, electrophysiological, and biochemical features; and treatment response of the antibody-mediated neuropathies, aiming to establish when and why to look for antibodies in chronic dysimmune neuropathies.
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Affiliation(s)
- Stefano Tozza
- Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II of Naples, 80131 Naples, Italy
| | - Emanuele Spina
- Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II of Naples, 80131 Naples, Italy
| | - Aniello Iovino
- Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II of Naples, 80131 Naples, Italy
| | - Rosa Iodice
- Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II of Naples, 80131 Naples, Italy
| | - Raffaele Dubbioso
- Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II of Naples, 80131 Naples, Italy
| | - Lucia Ruggiero
- Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II of Naples, 80131 Naples, Italy
| | - Maria Nolano
- Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II of Naples, 80131 Naples, Italy
- Neurology Department, Skin Biopsy Laboratory, Istituti Clinici Scientifici Maugeri IRCCS, 82037 Telese Terme, Italy
| | - Fiore Manganelli
- Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II of Naples, 80131 Naples, Italy
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Dhanapalaratnam R, Issar T, Poynten AM, Milner K, Kwai NCG, Krishnan AV. Diagnostic accuracy of nerve ultrasonography for the detection of peripheral neuropathy in type 2 diabetes. Eur J Neurol 2022; 29:3571-3579. [PMID: 36039540 PMCID: PMC9826521 DOI: 10.1111/ene.15534] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2022] [Revised: 07/05/2022] [Accepted: 07/13/2022] [Indexed: 01/11/2023]
Abstract
BACKGROUND AND PURPOSE Nerve conduction studies (NCS) are the current objective measure for diagnosis of peripheral neuropathy in type 2 diabetes but do not assess nerve structure. This study investigated the utility of peripheral nerve ultrasound as a marker of the presence and severity of peripheral neuropathy in type 2 diabetes. METHODS A total of 156 patients were recruited, and nerve ultrasound was undertaken on distal tibial and distal median nerves. Neuropathy severity was graded using the modified Toronto Clinical Neuropathy Scale (mTCNS) and Total Neuropathy Score (TNS). Studies were undertaken by a single ultrasonographer blinded to nerve conduction results. RESULTS A stepwise increase in tibial nerve cross-sectional area (CSA) was noted with increasing TNS grade (p < 0.001) and each mTCNS quartile (p < 0.001). Regression analysis demonstrated a correlation between tibial nerve CSA and neuropathy severity (p < 0.001). Using receiver operator curve analysis, tibial nerve CSA of >12.88 mm yielded a sensitivity of 70.5% and specificity of 85.7% for neuropathy detection. Binary logistic regression revealed that tibial nerve CSA was a predictor of abnormal sural sensory nerve action potential amplitude (odds ratio = 1.239, 95% confidence interval [CI] = 1.142-1.345) and abnormal neuropathy score (odds ratio = 1.537, 95% confidence interval [CI] = 1.286-1.838). CONCLUSIONS Tibial nerve ultrasound has good specificity and sensitivity for neuropathy diagnosis in type 2 diabetes. The study demonstrates that tibial nerve CSA correlates with neuropathy severity. Future serial studies using both ultrasound and NCS may be useful in determining whether changes in ultrasound occur prior to development of nerve conduction abnormalities and neuropathic symptoms.
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Affiliation(s)
- Roshan Dhanapalaratnam
- Prince of Wales Clinical School, University of New South WalesSydneyNew South WalesAustralia
- Institute of Neurological Sciences, Prince of Wales HospitalSydneyNew South WalesAustralia
| | - Tushar Issar
- Prince of Wales Clinical School, University of New South WalesSydneyNew South WalesAustralia
| | - Ann M. Poynten
- Prince of Wales Clinical School, University of New South WalesSydneyNew South WalesAustralia
- Department of EndocrinologyPrince of Wales HospitalSydneyNew South WalesAustralia
| | - Kerry‐Lee Milner
- Prince of Wales Clinical School, University of New South WalesSydneyNew South WalesAustralia
- Department of EndocrinologyPrince of Wales HospitalSydneyNew South WalesAustralia
| | - Natalie C. G. Kwai
- School of Medical SciencesUniversity of SydneySydneyNew South WalesAustralia
| | - Arun V. Krishnan
- Prince of Wales Clinical School, University of New South WalesSydneyNew South WalesAustralia
- Institute of Neurological Sciences, Prince of Wales HospitalSydneyNew South WalesAustralia
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Zaottini F, Picasso R, Pistoia F, Sanguinetti S, Pansecchi M, Tovt L, Viglino U, Cabona C, Garnero M, Benedetti L, Martinoli C. High-resolution ultrasound of peripheral neuropathies in rheumatological patients: An overview of clinical applications and imaging findings. Front Med (Lausanne) 2022; 9:984379. [PMID: 36388946 PMCID: PMC9661426 DOI: 10.3389/fmed.2022.984379] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2022] [Accepted: 09/12/2022] [Indexed: 11/13/2022] Open
Abstract
Peripheral neuropathies are surprisingly common and can be associated with a number of conditions, including rheumatological diseases. Whether the co-existence of peripheral neuropathies with rheumatological disorders is coincidental or related to a common pathogenic mechanism, these disabling conditions can affect the outcome of rheumatological patients and should be targeted with specific treatment. The clinical presentation of peripheral neuropathy can be multifaceted and difficult to recognize in polysymptomatic patients. However, physicians adopting state-of-art diagnostic strategies, including nerve imaging, may improve the detection rate and management of neuropathies. In particular, a diagnostic approach relying exclusively on clinical history and nerve conduction studies may not be sufficient to disclose the etiology of the nerve damage and its anatomical location and thus requires integration with morphological studies. High-Resolution Ultrasound (HRUS) is increasingly adopted to support the diagnosis and follow-up of both joint disorders in rheumatology and peripheral neuropathies of different etiologies. In this review, the different types of nerve disorders associated with the most common syndromes of rheumatological interest are discussed, focusing on the distinctive sonographic features.
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Affiliation(s)
- Federico Zaottini
- San Martino Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, Genoa, Italy
| | - Riccardo Picasso
- San Martino Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, Genoa, Italy
- *Correspondence: Riccardo Picasso,
| | - Federico Pistoia
- Dipartimento di Medicina Sperimentale, Scuola di Scienze Mediche e Farmaceutiche, Università di Genova, Genoa, Italy
| | - Sara Sanguinetti
- Dipartimento di Medicina Sperimentale, Scuola di Scienze Mediche e Farmaceutiche, Università di Genova, Genoa, Italy
| | - Michelle Pansecchi
- Dipartimento di Scienze della Salute, Scuola di Scienze Mediche e Farmaceutiche, Università di Genova, Genoa, Italy
| | - Luca Tovt
- Dipartimento di Scienze della Salute, Scuola di Scienze Mediche e Farmaceutiche, Università di Genova, Genoa, Italy
| | - Umberto Viglino
- Dipartimento di Scienze della Salute, Scuola di Scienze Mediche e Farmaceutiche, Università di Genova, Genoa, Italy
| | - Corrado Cabona
- San Martino Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, Genoa, Italy
- Eye Clinic, Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Science, School of Medical and Pharmaceutical Sciences, University of Genoa, Genoa, Italy
| | - Martina Garnero
- San Martino Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, Genoa, Italy
- Eye Clinic, Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Science, School of Medical and Pharmaceutical Sciences, University of Genoa, Genoa, Italy
| | - Luana Benedetti
- San Martino Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, Genoa, Italy
- Eye Clinic, Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Science, School of Medical and Pharmaceutical Sciences, University of Genoa, Genoa, Italy
| | - Carlo Martinoli
- San Martino Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, Genoa, Italy
- Dipartimento di Scienze della Salute, Scuola di Scienze Mediche e Farmaceutiche, Università di Genova, Genoa, Italy
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22
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Marin A, Savescu M, Marin G, Dricu A, Parasca S, Giuglea C. Evaluation of muscle atrophy after sciatic nerve defect repair – experimental model. ROMANIAN JOURNAL OF MILITARY MEDICINE 2022. [DOI: 10.55453/rjmm.2022.125.3.10] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/07/2022]
Abstract
Peripheral nerve injuries are one of the most important causes for disability generating considerable costs around the world. Appropriate assessment of the extent of peripheral nerve lesions and the choice of the right therapeutic protocol remain some of the biggest challenges. Non-invasive neuroimaging approaches are suitable in managing peripheral nerve repairs, providing in the same time accuracy details in structural neural detection, with minimal discomfort at a low cost. Medical imaging technology development has led to progress in examination of peripheral nervous system, using a series of tools and methods, such as: ultrasonography (US), positron emission tomography (PET), magnetic resonance imaging (MRI) and magnetic diffusion tensor imaging (DTI). In this study, we evaluated the regeneration process in Wistar rats after sciatic nerve defects repair with 4 different techniques (i.e. nerve graft, rat aorta used as a nerve conduit, rat aorta filled with platelet rich plasma (PRP) and rat aorta filled with stem cells) by using MRI investigation alongside the clinical evaluation. Our results showed that among the 4 batches, the PRP batch had the best results in muscle atrophy condition (both on MRI scan and on gastrocnemius index); on the second place the stem cell batch, then the nerve graft batch and finally the aortic conduit batch. MRI proved to be a reliable non-invasive monitoring method and showed good result in correlation with the footprint test (sciatic functional index) and the gastrocnemius index
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23
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Ultrasound of cervical nerve root enlargement in polyneuropathy is not confounded by neuroforaminal stenoses. Clin Neurophysiol 2022; 141:34-41. [DOI: 10.1016/j.clinph.2022.06.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2022] [Revised: 06/21/2022] [Accepted: 06/25/2022] [Indexed: 11/22/2022]
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Use of Neuromuscular Ultrasound in the Diagnosis of Disulfiram Polyneuropathy. J Clin Neuromuscul Dis 2022; 23:201-204. [PMID: 35608643 DOI: 10.1097/cnd.0000000000000380] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
ABSTRACT We present the case of a 37-year-old woman with alcohol use disorder, who developed leg cramping, bilateral foot drop, and hand weakness 3 months after starting disulfiram. This was accompanied by an 18-pound involuntary weight loss. Electrophysiologic findings showed a motor predominant axonal neuropathy. Neuromuscular ultrasound showed normal to small cross-sectional area of all nerves studied. This case is discussed, and the ultrasound findings are compared with another reported case.
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25
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Crump NH, Cartwright MS. A Retrospective Study of Ultrasound Accuracy for the Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy. J Clin Neurophysiol 2022; 39:312-316. [PMID: 33009042 DOI: 10.1097/wnp.0000000000000782] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
Abstract
INTRODUCTION Ultrasound is emerging as a useful tool for the evaluation of immune-mediated neuropathies because it can provide high-resolution anatomic information to complement electrodiagnostic data. Nerve enlargements are commonly found in chronic inflammatory demyelinating polyneuropathy (CIDP), and their presence likely useful in diagnosis, particularly if multifocal. METHODS In this study, the authors undertook a retrospective chart review to identify ultrasound findings in patients with CIDP previously studied in a single busy neurodiagnostic laboratory. RESULTS Of the 50 cases identified from 2000 to 2017, individuals with a confirmed diagnosis of CIDP (21 cases) were more likely to have multiple sites of enlargement, as well as more pronounced nerve enlargement, than patients who were subsequently found to have an alternate cause of neuropathy (22 cases). The presence of any moderately enlarged nerve segment predicted definite CIDP with sensitivity of 81% and specificity 77%. CONCLUSION This study demonstrates that ultrasound can be of diagnostic utility in patients with suspected CIDP, even when conducted in a nonstandardized real-world setting.
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Affiliation(s)
- Nicholas H Crump
- Departments of Neurology and Medicine, Austin Health and University of Melbourne, Victoria, Australia ; and
| | - Michael S Cartwright
- Department of Neurology, Wake Forest School of Medicine, Winston-Salem, North Carolina, U.S.A
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26
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Chen Z, Saini M, Neo SXM, Ng PS, Koh JS, Prasad K, Verma K, Davila S, Lim WK, Phua Z, Li MM, Kang C, Tay KSS, Chai JYH. Acute to Subacute Atraumatic Entrapment Neuropathies in Patients With CMT1A: A Report of a Distinct Phenotypic Variant of CMT1A. Front Neurol 2022; 13:826634. [PMID: 35280294 PMCID: PMC8914073 DOI: 10.3389/fneur.2022.826634] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/01/2021] [Accepted: 01/31/2022] [Indexed: 11/13/2022] Open
Abstract
Charcot-Marie-Tooth type 1A (CMT1A) is typically characterised as a childhood-onset, symmetrical, length-dependent polyneuropathy with a gradual progressive clinical course. Acute to subacute neurological deterioration in CMT1A is rare, and has been reported secondary to overlap pathologies including inflammatory neuropathy. We identified two patients with CMT1A who presented with acute to subacute, atraumatic, entrapment neuropathies as an initial symptom. A superimposed inflammatory neuropathy was excluded. Both patients had a diffuse demyelinating polyneuropathy, with markedly low motor nerve conduction velocities (<20 m/s). In both patients, we demonstrated symptomatic and asymptomatic partial conduction blocks at multiple entrapment sites. Nerve ultrasound findings in our patients demonstrated marked diffuse nerve enlargement, more pronounced at non-entrapment sites compared to entrapment sites. We discuss ways to distinguish this condition from its other differentials. We propose pathophysiological mechanisms underlying this condition. We propose that CMT1A with acute to subacute, atraumatic, entrapment neuropathies to be a distinct phenotypic variant of CMT1A.
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Affiliation(s)
- Zhiyong Chen
- Department of Neurology, National Neuroscience Institute, Singapore, Singapore
- *Correspondence: Zhiyong Chen
| | - Monica Saini
- Department of Neurology, National Neuroscience Institute, Singapore, Singapore
| | - Shermyn X. M. Neo
- Department of Neurology, National Neuroscience Institute, Singapore, Singapore
| | - Peng-Soon Ng
- Department of Neurology, National Neuroscience Institute, Singapore, Singapore
| | - Jasmine S. Koh
- Department of Neurology, National Neuroscience Institute, Singapore, Singapore
| | - Kalpana Prasad
- Department of Neurology, National Neuroscience Institute, Singapore, Singapore
| | - Kamal Verma
- Department of Neurology, National Neuroscience Institute, Singapore, Singapore
| | - Sonia Davila
- Singhealth Duke-National University of Singapore (NUS) Institute of Precision Medicine, Singapore, Singapore
- Cardiovascular and Metabolic Disorders, Duke-National University of Singapore (NUS) Medical School, Singapore, Singapore
- SingHealth Duke-National University of Singapore (NUS) Genomic Medicine Centre, Singapore, Singapore
| | - Weng Khong Lim
- Singhealth Duke-National University of Singapore (NUS) Institute of Precision Medicine, Singapore, Singapore
- SingHealth Duke-National University of Singapore (NUS) Genomic Medicine Centre, Singapore, Singapore
- Cancer and Stem Cell Biology Program, Duke-National University of Singapore (NUS) Medical School, Singapore, Singapore
| | - Ziqun Phua
- Neurodiagnostic Laboratory, National Neuroscience Institute, Singapore, Singapore
| | - Michelle M. Li
- Neurodiagnostic Laboratory, National Neuroscience Institute, Singapore, Singapore
| | - Corrine Kang
- Clinical Measurement Unit, Changi General Hospital, Singapore, Singapore
| | - Karine S. S. Tay
- Neuromuscular Laboratory, National Neuroscience Institute, Singapore, Singapore
| | - Josiah Y. H. Chai
- Department of Neurology, National Neuroscience Institute, Singapore, Singapore
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27
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Jorgensen SP, Cartwright MS, Norbury J. Neuromuscular Ultrasound: Indications in the Electrodiagnostic Laboratory. Am J Phys Med Rehabil 2022; 101:78-88. [PMID: 33990480 DOI: 10.1097/phm.0000000000001790] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
ABSTRACT Accurate assessment of neuromuscular disorders is critical to facilitate timely treatment and achieve the best outcomes. Historically, electrodiagnostic studies have filled this role, but recently, neuromuscular ultrasound is being used in the electrodiagnostic laboratory. This review discusses the uses of neuromuscular ultrasound in the electrodiagnostic laboratory that have strong evidence, emphasizing those that could be adopted in a typical electrodiagnostic laboratory with a reasonable level of equipment and training. The evidence currently supports using neuromuscular ultrasound to diagnose carpal tunnel syndrome and ulnar neuropathies at the elbow and as a supplementary test when electrodiagnostic studies are suspected to be falsely negative or in axonal nonlocalizing lesions. Neuromuscular ultrasound can identify the causes of focal mononeuropathies, which can change treatment in specific cases. It is sensitive at identifying fasciculations and providing complementary evidence of autoimmune demyelinating polyneuropathies. It is particularly helpful in assessing nerves after trauma. Neuromuscular ultrasound is likely to prove even more useful in the electrodiagnostic laboratory as the technology continues to advance.
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Affiliation(s)
- Shawn P Jorgensen
- From the Department of Physical Medicine and Rehabilitation, Albany Medical College, Albany, New York (SPJ); Department of Family Medicine, Larner Medical College at the University of Vermont, Burlington, Vermont (SPJ); Adirondack Rehabilitation Medicine, PLLC, Queensbury, New York (SPJ); Department of Neurology, Wake Forest University School of Medicine, Winston-Salem, North Carolina (MSC); and Division of Physical Medicine and Rehabilitation, Department of Neurology, Texas Tech University Health Sciences Center, Lubbock, Texas (JN)
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28
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Bulinski C, Viard M, Vlazak A, Habig K, Juenemann M, Best C, Schirotzek I, Kaps M, Krämer HH. Neuromuscular sonography detects changes in muscle echotexture and nerve diameter in ICU patients within 24 h. J Ultrasound 2021; 25:535-545. [PMID: 34870825 PMCID: PMC9402876 DOI: 10.1007/s40477-021-00621-8] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2021] [Accepted: 08/25/2021] [Indexed: 11/03/2022] Open
Abstract
Purpose During an ICU stay, changes in muscles and nerves occur that is accessible via neuromuscular sonography. Methods 17 patients recruited from the neurological and neurosurgical ICU (six women; 66 ± 3 years) and 7 healthy controls (three women, 75 ± 3 years) were included. Muscle sonography (rectus abdominis, biceps, rectus femoris and tibialis anterior muscles) using gray-scale values (GSVs), and nerve ultrasound (peroneal, tibial and sural nerves) analyzing the cross-sectional area (CSA) were performed on days 1 (t1), 3 (t2), 5 (t3), 8 (t4), and 16 (t5) after admission. Results Time course analysis revealed that GSVs were significantly higher within the patient group for all of the investigated muscles (rectus abdominis: F = 7.536; p = 0.011; biceps: F = 14.761; p = 0.001; rectus femoris: F = 9.455; p = 0.005; tibialis anterior: F = 7.282; p = 0.012). The higher GSVs were already visible at t1 or, at the latest, at t2 (tibialis anterior muscles). CSA was enlarged in all of the investigated nerves in the patient group (peroneal nerve: F = 7.129; p = 0.014; tibial nerve: F = 28.976, p < 0.001; sural nerve: F = 13.051; p = 0.001). The changes were visible very early (tibial nerve: t1; peroneal nerve: t2). The CSA of the motor nerves showed an association with the ventilation time and days within the ICU (t1 through t4; p < 0.05). Discussion We detected very early changes in the muscles and nerves of ICU-patients. Nerve CSA might be a useful parameter to identify patients who are at risk for difficult weaning. Therefore our observations might be severity signs of neuromuscular suffering for the most severe patients.
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Affiliation(s)
- Catherine Bulinski
- Department of Neurology, Justus Liebig University, Klinikstrasse 33, 35392, Giessen, Germany
| | - Maxime Viard
- Department of Neurology, Justus Liebig University, Klinikstrasse 33, 35392, Giessen, Germany
| | - Alexander Vlazak
- Department of Neurology, Justus Liebig University, Klinikstrasse 33, 35392, Giessen, Germany
| | - Kathrin Habig
- Department of Neurology, Justus Liebig University, Klinikstrasse 33, 35392, Giessen, Germany
| | - Martin Juenemann
- Department of Neurology, Justus Liebig University, Klinikstrasse 33, 35392, Giessen, Germany
| | - Christoph Best
- Department of Neurology, Phillipps University, Baldingerstrasse 1, Marburg, Germany
| | - Ingo Schirotzek
- Department of Neurology, Justus Liebig University, Klinikstrasse 33, 35392, Giessen, Germany
| | - Manfred Kaps
- Department of Neurology, Justus Liebig University, Klinikstrasse 33, 35392, Giessen, Germany
| | - Heidrun H Krämer
- Department of Neurology, Justus Liebig University, Klinikstrasse 33, 35392, Giessen, Germany.
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29
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Stenør CPM, Mahdaoui SE, Wolfram N. Ultrasonic evidence of mononeuritis multiplex caused by Lyme neuroborreliosis. Muscle Nerve 2021; 65:E4-E6. [PMID: 34644405 DOI: 10.1002/mus.27440] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2021] [Revised: 10/04/2021] [Accepted: 10/09/2021] [Indexed: 11/10/2022]
Affiliation(s)
- Christian P M Stenør
- Department of Neurology, Herlev & Gentofte Hospital, Herlev, Denmark.,Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark
| | - Sahla El Mahdaoui
- Department of Neurology, Herlev & Gentofte Hospital, Herlev, Denmark
| | - Nils Wolfram
- Department of Neurophysiology, Rigshospitalet Glostrup, Glostrup, Denmark
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30
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Hannaford A, Vucic S, Kiernan MC, Simon NG. Review Article "Spotlight on Ultrasonography in the Diagnosis of Peripheral Nerve Disease: The Evidence to Date". Int J Gen Med 2021; 14:4579-4604. [PMID: 34429642 PMCID: PMC8378935 DOI: 10.2147/ijgm.s295851] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Accepted: 07/27/2021] [Indexed: 11/23/2022] Open
Abstract
Neuromuscular ultrasound is rapidly becoming incorporated into clinical practice as a standard tool in the assessment of peripheral nerve diseases. Ultrasound complements clinical phenotyping and electrodiagnostic evaluation, providing critical structural anatomical information to enhance diagnosis and identify structural pathology. This review article examines the evidence supporting neuromuscular ultrasound in the diagnosis of compressive mononeuropathies, traumatic nerve injury, generalised peripheral neuropathy and motor neuron disease. Extending the sonographic evaluation of nerves beyond simple morphological measurements has the potential to improve diagnostics in peripheral neuropathy, as well as advancing the understanding of pathological mechanisms, which in turn will promote precise therapies and improve therapeutic outcomes.
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Affiliation(s)
- Andrew Hannaford
- Westmead Clinical School, Westmead Hospital, University of Sydney, Sydney, Australia
| | - Steve Vucic
- Westmead Clinical School, Westmead Hospital, University of Sydney, Sydney, Australia
| | - Matthew C Kiernan
- Brain and Mind Centre, University of Sydney, University of Sydney and Department of Neurology, Royal Prince Alfred Hospital, Sydney, Australia
| | - Neil G Simon
- Northern Beaches Clinical School, Macquarie University, Sydney, Australia
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31
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Seeliger T, Bönig L, Gingele S, Prenzler NK, Thiele T, Ernst D, Witte T, Stangel M, Skripuletz T, Körner S. Nerve ultrasound findings in Sjögren's syndrome-associated neuropathy. J Neuroimaging 2021; 31:1156-1165. [PMID: 34270142 DOI: 10.1111/jon.12907] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2021] [Revised: 06/30/2021] [Accepted: 06/30/2021] [Indexed: 11/29/2022] Open
Abstract
BACKGROUND AND PURPOSE The phenotype of Sjögren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjögren's syndrome-associated neuropathy remains an underdiagnosed entity with only few insights considering the pathomechanisms of nerve damage. Nerve ultrasound has proven to be a useful and efficient tool in detecting nerve damage of autoimmune origin. We, therefore, aimed to evaluate this method for Sjögren's syndrome-associated neuropathy. METHODS Patients with Sjögren's syndrome and clinical signs of neuropathy underwent sonographic examination of both median and ulnar nerves. Nerve thickening was classified for cross-sectional areas of >12 mm² at the median nerve and for >10 mm² at the ulnar nerve. Fascicle thickening was documented for cross-sectional areas ≥5 mm² at the median and ≥3 mm² at the ulnar nerve. RESULTS Forty-three patients were included in the analysis (median age 60 years [interquartile range 53-73 years], female rate 60%). 31/43 patients (72%) showed abnormalities on nerve ultrasound, while nerve thickening was found more frequently than fascicle thickening (90% vs. 52% of patients with sonographic abnormalities, respectively). Abnormal findings were observed more frequently at the median nerve and in proximal localization. Abnormal findings on nerve conduction studies were evident in 36/43 patients (84%). Nerve conduction studies revealed a tendency of demyelinating nerve damage patterns being associated with abnormal findings on nerve ultrasound. CONCLUSIONS In addition to nerve conduction studies, nerve ultrasound may have a supporting role in the diagnosis of Sjögren's syndrome-associated neuropathy. Also, our data support an immune-mediated inflammatory demyelinating pathogenesis of Sjögren's syndrome-associated neuropathy.
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Affiliation(s)
- Tabea Seeliger
- Department of Neurology, Hannover Medical School, Hannover, Germany
| | - Lena Bönig
- Department of Neurology, Hannover Medical School, Hannover, Germany
| | - Stefan Gingele
- Department of Neurology, Hannover Medical School, Hannover, Germany
| | - Nils K Prenzler
- Department of Otolaryngology, Hannover Medical School, Hannover, Germany
| | - Thea Thiele
- Department of Rheumatology & Immunology, Hannover Medical School, Hannover, Germany
| | - Diana Ernst
- Department of Rheumatology & Immunology, Hannover Medical School, Hannover, Germany
| | - Torsten Witte
- Department of Rheumatology & Immunology, Hannover Medical School, Hannover, Germany
| | - Martin Stangel
- Department of Neurology, Hannover Medical School, Hannover, Germany
| | | | - Sonja Körner
- Department of Neurology, Hannover Medical School, Hannover, Germany
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32
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Mahran SA, Galluccio F, Khedr TM, Elsonbaty A, Allam AES, Garcia Martos A, Osman DMM, Matucci-Cerinic M, Guiducci S, Galal MAA. Peripheral neuropathy in systemic lupus erythematosus: what can neuromuscular ultrasonography (NMUS) tell us? A cross-sectional study. Lupus Sci Med 2021; 8:e000521. [PMID: 34312211 PMCID: PMC8314698 DOI: 10.1136/lupus-2021-000521] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2021] [Accepted: 06/18/2021] [Indexed: 11/18/2022]
Abstract
OBJECTIVE To evaluate peripheral nerve involvement in patients with SLE with neuromuscular ultrasonography (NMUS) and understand its role in investigating SLE-related peripheral neuropathy. METHODS This is an observational cross-sectional study on patients with SLE and healthy controls. Five nerves in each patient were examined bilaterally with NMUS, and the cross-sectional area (CSA) of each nerve at certain sites was estimated. The mean CSA at each site, for each nerve, in each group was statistically analysed and compared between groups. RESULTS 370 nerves were evaluated in 37 patients. By nerve conduction study (NCS), 36 patients had polyneuropathy (80.6% mixed type, 19.4% sensory). Significant mean CSA enlargement was present among the ulnar nerve at the Guyon's canal and mid-humerus (both p=0.001); tibial nerve at the distal leg and proximal to the tarsal tunnel (p=0.003 and p=0.001, respectively); and peroneal nerve at the popliteal fossa (p=0.042). The mean CSA showed high specificity compared with NCS. CONCLUSION Our study shows that CSA could be a complementary tool to NCS for studying peripheral neuropathy in SLE. Furthermore, NMUS provides data on the different pathophysiological aspects of nerve involvement in SLE. Future studies using more than one sonographic parameter in combination with NCS and nerve histopathology are recommended to further investigate SLE-related neuropathy.Trial registration number NCT04527172.
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Affiliation(s)
- Safaa A Mahran
- Department of Rheumatology, Rehabilitation and Physical Medicine, Assiut University, Assiut, Egypt
| | - Felice Galluccio
- Medical-Geriatric Department, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy
- MoMaRC Morphological Madrid Research Center, Ultradissection group, Madrid, Spain
| | - Tayseer Mohamed Khedr
- Department of Rheumatology, Rehabilitation and Physical Medicine, Assiut University, Assiut, Egypt
| | - Amira Elsonbaty
- Department of Rheumatology, Rehabilitation and Physical Medicine, Assiut University, Assiut, Egypt
| | - Abdallah El-Sayed Allam
- MoMaRC Morphological Madrid Research Center, Ultradissection group, Madrid, Spain
- Department of Physical Medicine, Rheumatology and Rehabilitation, Tanta University Faculty of Medicine, Tanta, Egypt
| | | | - Doaa M M Osman
- Department of Public Health and Community, Faculty of Medicine, Assiut University, Assiut, Egypt
| | - Marco Matucci-Cerinic
- Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Firenze, Italy
| | - Serena Guiducci
- Medical-Geriatric Department, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy
- Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Firenze, Italy
| | - Marwa A A Galal
- Department of Rheumatology, Rehabilitation and Physical Medicine, Assiut University, Assiut, Egypt
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34
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Telleman JA, Herraets IJT, Goedee HS, van Eijk RPA, Verhamme C, Eftimov F, Lieba-Samal D, Asseldonk JTV, van den Berg LH, van der Pol WL, Visser LH. Prognostic value of nerve ultrasonography: A prospective multicenter study on the natural history of chronic inflammatory neuropathies. Eur J Neurol 2021; 28:2327-2338. [PMID: 33909329 DOI: 10.1111/ene.14885] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2021] [Revised: 04/19/2021] [Accepted: 04/22/2021] [Indexed: 02/06/2023]
Abstract
BACKGROUND AND OBJECTIVE Nerve ultrasound is a promising new tool in chronic inflammatory neuropathies. The aim of this study was to determine its prognostic value in a prospective multicenter cohort study including incident and prevalent patients with CIDP and MMN. METHODS We enrolled 126 patients with CIDP, and 72 with MMN; 71 were treatment-naive. Patients with chronic idiopathic axonal polyneuropathy (CIAP; n = 35) were considered as disease controls. Standardized neurological examination, questionnaires, and nerve ultrasonography were obtained at time of inclusion and 1-year follow-up. Nerve size development over time and correlation between nerve size and clinical outcome measures were determined using linear mixed effects models. RESULTS Nerve size development over time was heterogeneous. Only in MMN was there a correlation between C5 nerve root size and deterioration of grip strength (-1.3 kPa/mm2 (95% confidence interval [CI] -2.3 to -0.2). No other significant correlations between nerve size and clinical outcome measures were found. In MMN, presence of nerve enlargement at inclusion predicted deterioration of grip strength, and MMN patients with enlargement confined to the brachial plexus seemed to have more favorable outcomes. No other predictive effects of sonographic nerve size were found. CONCLUSIONS The present study indicates that the natural course of nerve size development in CIDP and MMN is heterogeneous, and that the prognostic value of sonographic nerve enlargement is limited. It had some predictive effect in patients with MMN. Further research in specific subgroups of chronic inflammatory neuropathy is necessary to determine the usefulness of nerve ultrasonography after the diagnostic phase.
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Affiliation(s)
- Johan A Telleman
- Department of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Tilburg, The Netherlands.,Department of Neurology and Neurosurgery, UMC Utrecht Brain Center, University Utrecht, Utrecht, The Netherlands
| | - Ingrid J T Herraets
- Department of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Tilburg, The Netherlands.,Department of Neurology and Neurosurgery, UMC Utrecht Brain Center, University Utrecht, Utrecht, The Netherlands
| | - Hendrik Stephan Goedee
- Department of Neurology and Neurosurgery, UMC Utrecht Brain Center, University Utrecht, Utrecht, The Netherlands
| | - Ruben P A van Eijk
- Department of Neurology and Neurosurgery, UMC Utrecht Brain Center, University Utrecht, Utrecht, The Netherlands.,Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands
| | - Camiel Verhamme
- Amsterdam University Medical Center, Amsterdam Neuroscience, University of Amsterdam, Amsterdam, The Netherlands
| | - Filip Eftimov
- Amsterdam University Medical Center, Amsterdam Neuroscience, University of Amsterdam, Amsterdam, The Netherlands
| | - Doris Lieba-Samal
- Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Jan Thies van Asseldonk
- Department of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Tilburg, The Netherlands
| | - Leonard H van den Berg
- Department of Neurology and Neurosurgery, UMC Utrecht Brain Center, University Utrecht, Utrecht, The Netherlands
| | - Willem Ludo van der Pol
- Department of Neurology and Neurosurgery, UMC Utrecht Brain Center, University Utrecht, Utrecht, The Netherlands
| | - Leo H Visser
- Department of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Tilburg, The Netherlands
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Abstract
Peripheral nerve imaging is a helpful and sometimes essential adjunct to clinical history, physical examination, and electrodiagnostic studies. Advances in imaging technology have allowed the visualization of nerve structures and their surrounding tissues. The clinical applications of ultrasound and magnetic resonance imaging (MRI) in the evaluation of peripheral nerve disorders are growing exponentially. This article reviews basics of ultrasound and MRI as they relate to nerve imaging, reviews advantages and limitations of each imaging modality, reviews the applications of ultrasound and MRI in disorders of peripheral nerve, and discusses emerging advances in the field.
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Affiliation(s)
- Natalia L Gonzalez
- Department of Neurology, Neuromuscular Division, Duke University, Duke University Hospital, 3403 DUMC, Duke South Clinic 1L, Durham, NC 27710, USA.
| | - Lisa D Hobson-Webb
- Department of Neurology, Neuromuscular Division, Duke University, Duke University Hospital, 3403 DUMC, Duke South Clinic 1L, Durham, NC 27710, USA
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Shen J, Yang F, Chen W, Wang F, Liang C, Qiu Y, Xu W. The Efficacy of Ultrasound for Visualizing Radial Nerve Lesions with Coexistent Plate Fixation of Humeral Shaft Fractures. Injury 2021; 52:516-523. [PMID: 33257017 DOI: 10.1016/j.injury.2020.11.042] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/01/2020] [Revised: 11/14/2020] [Accepted: 11/15/2020] [Indexed: 02/02/2023]
Abstract
INTRODUCTION Ultrasound has been commonly employed for depicting the morphology of the lesions in patients with radial nerve neuropathy, including entrapment, tumor, trauma, and iatrogenic injury. However, few studies have evaluated the efficacy of ultrasound for visualizing radial nerve lesions with coexistent plate fixation of humeral shaft fractures. This study aimed to address this special clinical issue. METHODS We retrospectively examined the efficacy of ultrasound for visualizing radial nerve lesions with coexistent plate fixation of humeral shaft fractures based on intraoperative findings in patients who were treated in our hospital from January 2007 to June 2019. RESULTS Forty-six patients were included, and there was a 100% concordance between the ultrasound and intraoperative findings on radial nerve lesions. Ultrasonography revealed four types of lesions: radial nerve in continuity in thirty-one patients, neuroma in continuity in four patients, radial nerve stuck under the plate in three patients, and radial nerve transection in eight patients. The lesion radial nerve in continuity comprised two situations according to intraoperative electrodiagnostic test results, which could not be differentiated by ultrasonography, radial nerve in continuity treated with neurolysis in twenty-five patients and radial nerve in continuity treated with nerve graft in six patients. CONCLUSION Ultrasonography can accurately depict radial nerve lesions with coexistent plate fixation of humeral shaft fractures. It provides a basis for determining the extent of nerve damage in all patients except those with the lesion radial nerve in continuity, which is conducive to making treatment decisions as early as possible.
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Affiliation(s)
- Jun Shen
- Department of Hand Surgery, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China
| | - Fangjing Yang
- Department of Hand Surgery, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China
| | - Weimin Chen
- Department of Ultrasound in Medicine, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China
| | - Fei Wang
- Department of Hand and Upper Extremity Surgery, Jing' an District Central Hospital, Shanghai, China
| | - Chunmin Liang
- Limb Function Reconstruction Center, Jing' an District Central Hospital, Shanghai, China
| | - Yanqun Qiu
- Department of Hand Surgery, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China; Department of Hand and Upper Extremity Surgery, Jing' an District Central Hospital, Shanghai, China; Limb Function Reconstruction Center, Jing' an District Central Hospital, Shanghai, China; National Clinical Research Center for Aging and Medicine, Fudan University, Shanghai, China; Key Laboratory of Hand Reconstruction, Ministry of Health, Shanghai, China; Shanghai Key Laboratory of Peripheral Nerve and Microsurgery, Shanghai, China; State Key Laboratory of Medical Neurobiology, Fudan University, Shanghai, China; Research unit of synergistic reconstruction of upper and lower limbs after brain injury, Chinese Academy of Medical Sciences, Shanghai, China.
| | - Wendong Xu
- Department of Hand Surgery, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China; Department of Hand and Upper Extremity Surgery, Jing' an District Central Hospital, Shanghai, China; Limb Function Reconstruction Center, Jing' an District Central Hospital, Shanghai, China; National Clinical Research Center for Aging and Medicine, Fudan University, Shanghai, China; Key Laboratory of Hand Reconstruction, Ministry of Health, Shanghai, China; Shanghai Key Laboratory of Peripheral Nerve and Microsurgery, Shanghai, China; State Key Laboratory of Medical Neurobiology, Fudan University, Shanghai, China; Research unit of synergistic reconstruction of upper and lower limbs after brain injury, Chinese Academy of Medical Sciences, Shanghai, China
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High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome-A Comparative Study. Diagnostics (Basel) 2021; 11:diagnostics11020264. [PMID: 33572067 PMCID: PMC7915164 DOI: 10.3390/diagnostics11020264] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2020] [Revised: 01/26/2021] [Accepted: 02/05/2021] [Indexed: 12/13/2022] Open
Abstract
Background: High-resolution nerve ultrasound (HRUS) has been proven to be a valuable tool in the diagnosis of immune-mediated neuropathies, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is an important differential diagnosis of CIDP. Until now, there have been no studies that could identify specific HRUS abnormalities in POEMS syndrome patients. Thus, the aim of this study was to assess possible changes and compare findings with CIDP patients. Methods: We retrospectively analyzed HRUS findings in three POEMS syndrome and ten CIDP patients by evaluating cross-sectional nerve area (CSA), echogenicity and additionally calculating ultrasound pattern scores (UPSA, UPSB, UPSC and UPSS) and homogeneity scores (HS). Results: CIDP patients showed greater CSA enlargement and higher UPSS (median 14 vs. 11), UPSA (median 11.5 vs. 8) and HS (median 5 vs. 3) compared with POEMS syndrome patients. However, every POEMS syndrome patient illustrated enlarged nerves exceeding reference values, which were not restricted to entrapment sites. In CIDP and POEMS syndrome, heterogeneous enlargement patterns could be identified, such as inhomogeneous, homogeneous and regional nerve enlargement. HRUS in CIDP patients visualized both increased and decreased echointensity, while POEMS syndrome patients pictured hypoechoic nerves with hyperechoic intraneural connective tissue. Discussion: This is the first study to demonstrate HRUS abnormalities in POEMS syndrome outside of common entrapment sites. Although nerve enlargement was more prominent in CIDP, POEMS syndrome patients revealed distinct echogenicity patterns, which might aid in its differentiation from CIDP. Future studies should consider HRUS and its possible role in determining diagnosis, prognosis and treatment response in POEMS syndrome.
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38
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Telleman JA, Herraets IJT, Goedee HS, van Asseldonk JT, Visser LH. Ultrasound scanning in the diagnosis of peripheral neuropathies. Pract Neurol 2021; 21:186-195. [DOI: 10.1136/practneurol-2020-002645] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/18/2020] [Indexed: 12/13/2022]
Abstract
Nerve ultrasound scanning has become a valuable diagnostic tool in the routine workup of peripheral nerve disorders, effectively complementing conventional electrodiagnostic studies. The most relevant sonographic features are nerve size and structural integrity. Several peripheral neuropathies show characteristic and distinct patterns of nerve enlargement, allowing their early and accurate identification, and reducing test-burden and diagnostic delay for patients. In mononeuropathies such as carpal tunnel syndrome and ulnar neuropathy at the elbow, nerve enlargement develops only at specific sites of entrapment, while in polyneuropathy the nerve enlargement may be multifocal, regional or even diffuse. Nerve ultrasound scanning can reliably identify chronic inflammatory neuropathies, even when extensive electrodiagnostic studies fail, and it should therefore be embedded in routine diagnostic workup of peripheral neuropathies. In this paper we describe a potential diagnostic strategy to achieve this.
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Nerve Ultrasound as Helpful Tool in Polyneuropathies. Diagnostics (Basel) 2021; 11:diagnostics11020211. [PMID: 33572591 PMCID: PMC7910962 DOI: 10.3390/diagnostics11020211] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2020] [Revised: 01/26/2021] [Accepted: 01/27/2021] [Indexed: 02/06/2023] Open
Abstract
Background: Polyneuropathies (PNP) are a broad field of diseases affecting millions of people. While the symptoms presented are mostly similar, underlying causes are abundant. Thus, early identification of treatable causes is often difficult. Besides clinical data and basic laboratory findings, nerve conduction studies are crucial for etiological classification, yet limited. Besides Magnetic Resonance Imaging (MRI), high-resolution nerve ultrasound (HRUS) has become a noninvasive, fast, economic and available tool to help distinguish different types of nerve alterations in neuropathies. Methods: We aim to describe typical ultrasound findings in PNP and patterns of morphological changes in hereditary, immune-mediated, diabetic, metabolic and neurodegenerative PNP. Literature research was performed in PubMed using the terms ‘nerve ultrasound’, neuromuscular ultrasound, high-resolution nerve ultrasound, peripheral nerves, nerve enlargement, demyelinating, hereditary, polyneuropathies, hypertrophy’. Results: Plenty of studies over the past 20 years investigated the value of nerve ultrasound in different neuropathies. Next to nerve enlargement, patterns of nerve enlargement, echointensity, vascularization and elastography have been evaluated for diagnostic terms. Furthermore, different scores have been developed to distinguish different etiologies of PNP. Conclusions: Where morphological alterations of the nerves reflect underlying pathologies, early nerve ultrasound might enable a timely start of available treatment and also facilitate follow up of therapy success.
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40
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Neuromuscular Ultrasound in the Pediatric Population. Diagnostics (Basel) 2020; 10:diagnostics10121012. [PMID: 33255940 PMCID: PMC7760629 DOI: 10.3390/diagnostics10121012] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/10/2020] [Revised: 11/19/2020] [Accepted: 11/24/2020] [Indexed: 12/15/2022] Open
Abstract
The diagnosis and evaluation of neuromuscular disorders traditionally involves electrodiagnostic (EDx) testing, including nerve conduction studies (NCSs) and electromyography (EMG). These tools can cause pain and discomfort, an important consideration when performed on children. Neuromuscular ultrasound is noninvasive, cost-effective, and increasingly utilized for the detection of neuromuscular pathology. Studies investigating the performance and clinical implementation of ultrasound have primarily been performed in adult populations. Ultrasound in children has the potential to guide EDx testing and ultimately improve diagnostic efficiency and accuracy. This review aims to describe key features of neuromuscular ultrasound in the pediatric population based on the available studies, including our own institutional experience.
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Gardill K, Schüpbach M. [Pronounced proximally accentuated changes in high-voltage stimulation and nerve ultrasound in long-standing therapy-naive CIDP variants]. DER NERVENARZT 2020; 92:161-165. [PMID: 33112965 DOI: 10.1007/s00115-020-01020-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 10/02/2020] [Indexed: 11/29/2022]
Abstract
The differential diagnosis of chronic demyelinating polyneuropathy particularly includes inflammatory (CIDP) and hereditary causes. Using the example of a 63-year-old patient, we show the diagnostic procedure with conventional electrophysiological diagnostics and additionally by the use of proximal nerve conduction studies with high-voltage stimulation (HVS) and the direct morphological examination by high-resolution nerve ultrasound. In the present case, the focal accentuation of the changes in HVS and the equally pronounced focal thickening of the most affected ulnar nerve in ultrasound confirmed the diagnosis of CIDP instead of hereditary neuropathy.
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Affiliation(s)
- Klaus Gardill
- Neurologische Praxis, Mühledorfstraße 21, 3018, Bern, Schweiz.
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42
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Cardellini D, Zanette G, Taioli F, Bertolasi L, Ferrari S, Cavallaro T, Fabrizi GM. CIDP, CMT1B, or CMT1B plus CIDP? Neurol Sci 2020; 42:1127-1130. [PMID: 33070202 DOI: 10.1007/s10072-020-04789-5] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/25/2020] [Accepted: 10/01/2020] [Indexed: 11/26/2022]
Abstract
Charcot-Marie-Tooth disease type 1 (CMT1) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have distinct clinical and neurophysiological features that result from dysmyelination in CMT1 and macrophage-mediated segmental demyelination in CIDP. CMT1 may occur in genetically isolated cases with atypical presentations that converge phenotypically with CIDP; in rare cases, however, CMT1 may be complicated by superimposed CIDP. We report the case of a patient harboring a de novo heterozygous null mutation of the myelin protein zero (MPZ) gene and affected by subclinical CMT1B who became symptomatic due to superimposed CIDP. Peripheral nerve high-resolution ultrasound (HRUS) aided in establishing the coexistence of CMT1B and CIDP; the diagnosis was further supported by favorable clinical, neurophysiological, and ultrasound responses to immunoglobulin therapy.
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Affiliation(s)
- Davide Cardellini
- Section of Neurology, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
| | - Giampietro Zanette
- Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy
| | - Federica Taioli
- Section of Neurology, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
- Section of Neurology B, Department of Neuroscience, University Hospital G.B. Rossi, AOUI Verona, P.le LA Scuro, 10 37134, Verona, VR, Italy
| | - Laura Bertolasi
- Section of Neurology B, Department of Neuroscience, University Hospital G.B. Rossi, AOUI Verona, P.le LA Scuro, 10 37134, Verona, VR, Italy
| | - Sergio Ferrari
- Section of Neurology B, Department of Neuroscience, University Hospital G.B. Rossi, AOUI Verona, P.le LA Scuro, 10 37134, Verona, VR, Italy
| | - Tiziana Cavallaro
- Section of Neurology B, Department of Neuroscience, University Hospital G.B. Rossi, AOUI Verona, P.le LA Scuro, 10 37134, Verona, VR, Italy
| | - Gian Maria Fabrizi
- Section of Neurology, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy.
- Section of Neurology B, Department of Neuroscience, University Hospital G.B. Rossi, AOUI Verona, P.le LA Scuro, 10 37134, Verona, VR, Italy.
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Dörner M, Schreiber F, Stephanik H, Tempelmann C, Winter N, Stahl JH, Wittlinger J, Willikens S, Kramer M, Heinze HJ, Vielhaber S, Schelle T, Grimm A, Schreiber S. Peripheral Nerve Imaging Aids in the Diagnosis of Immune-Mediated Neuropathies-A Case Series. Diagnostics (Basel) 2020; 10:E535. [PMID: 32751486 PMCID: PMC7459443 DOI: 10.3390/diagnostics10080535] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/24/2020] [Revised: 07/28/2020] [Accepted: 07/29/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Diagnosis of immune-mediated neuropathies and their differentiation from amyotrophic lateral sclerosis (ALS) can be challenging, especially at early disease stages. Accurate diagnosis is, however, important due to the different prognosis and available treatment options. We present one patient with a left-sided dorsal flexor paresis and initial suspicion of ALS and another with multifocal sensory deficits. In both, peripheral nerve imaging was the key for diagnosis. METHODS We performed high-resolution nerve ultrasound (HRUS) and 7T or 3T magnetic resonance neurography (MRN). RESULTS In both patients, HRUS revealed mild to severe, segmental or inhomogeneous, nerve enlargement at multiple sites, as well as an area increase of isolated fascicles. MRN depicted T2 hyperintense nerves with additional contrast-enhancement. DISCUSSION Peripheral nerve imaging was compatible with the respective diagnosis of an immune-mediated neuropathy, i.e., multifocal motor neuropathy (MMN) in patient 1 and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) in patient 2. Peripheral nerve imaging, especially HRUS, should play an important role in the diagnostic work-up for immune-mediated neuropathies and their differentiation from ALS.
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Affiliation(s)
- Marc Dörner
- Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany; (N.W.); (J.-H.S.); (J.W.); (S.W.); (M.K.); (A.G.)
| | - Frank Schreiber
- Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany; (F.S.); (H.S.); (C.T.); (H.-J.H.); (S.V.); (S.S.)
- German Center for Neurodegenerative Diseases (DZNE) within the Helmholtz Association, 39120 Magdeburg, Germany
| | - Heike Stephanik
- Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany; (F.S.); (H.S.); (C.T.); (H.-J.H.); (S.V.); (S.S.)
| | - Claus Tempelmann
- Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany; (F.S.); (H.S.); (C.T.); (H.-J.H.); (S.V.); (S.S.)
| | - Natalie Winter
- Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany; (N.W.); (J.-H.S.); (J.W.); (S.W.); (M.K.); (A.G.)
| | - Jan-Hendrik Stahl
- Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany; (N.W.); (J.-H.S.); (J.W.); (S.W.); (M.K.); (A.G.)
| | - Julia Wittlinger
- Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany; (N.W.); (J.-H.S.); (J.W.); (S.W.); (M.K.); (A.G.)
| | - Sophia Willikens
- Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany; (N.W.); (J.-H.S.); (J.W.); (S.W.); (M.K.); (A.G.)
| | - Magdalena Kramer
- Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany; (N.W.); (J.-H.S.); (J.W.); (S.W.); (M.K.); (A.G.)
| | - Hans-Jochen Heinze
- Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany; (F.S.); (H.S.); (C.T.); (H.-J.H.); (S.V.); (S.S.)
- German Center for Neurodegenerative Diseases (DZNE) within the Helmholtz Association, 39120 Magdeburg, Germany
- Center for Behavioural Brain Sciences (CBBS), 39120 Magdeburg, Germany
- Leibniz Institue for Neurobiology (LIN), 39120 Magdeburg, Germany
| | - Stefan Vielhaber
- Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany; (F.S.); (H.S.); (C.T.); (H.-J.H.); (S.V.); (S.S.)
- German Center for Neurodegenerative Diseases (DZNE) within the Helmholtz Association, 39120 Magdeburg, Germany
- Center for Behavioural Brain Sciences (CBBS), 39120 Magdeburg, Germany
| | - Thomas Schelle
- Department of Neurology, Städtisches Klinikum Dessau, 06847 Dessau, Germany;
| | - Alexander Grimm
- Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany; (N.W.); (J.-H.S.); (J.W.); (S.W.); (M.K.); (A.G.)
| | - Stefanie Schreiber
- Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany; (F.S.); (H.S.); (C.T.); (H.-J.H.); (S.V.); (S.S.)
- German Center for Neurodegenerative Diseases (DZNE) within the Helmholtz Association, 39120 Magdeburg, Germany
- Center for Behavioural Brain Sciences (CBBS), 39120 Magdeburg, Germany
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Herraets IJT, Goedee HS, Telleman JA, van Eijk RPA, Verhamme C, Saris CGJ, Eftimov F, van Alfen N, van Asseldonk JT, Visser LH, van den Berg LH, van der Pol LW. Nerve ultrasound for diagnosing chronic inflammatory neuropathy: A multicenter validation study. Neurology 2020; 95:e1745-e1753. [PMID: 32675082 DOI: 10.1212/wnl.0000000000010369] [Citation(s) in RCA: 30] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/23/2019] [Accepted: 04/06/2020] [Indexed: 01/06/2023] Open
Abstract
OBJECTIVE To validate the diagnostic accuracy of a previously described short sonographic protocol to identify chronic inflammatory neuropathy (CIN), including chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis Sumner syndrome, and multifocal motor neuropathy (MMN), and to determine the added value of nerve ultrasound to detect treatment-responsive patients compared to nerve conduction studies (NCS) in a prospective multicenter study. METHODS We included 100 consecutive patients clinically suspected of CIN in 3 centers. The study protocol consisted of neurologic examination, laboratory tests, NCS, and nerve ultrasound. We validated a short sonographic protocol (median nerve at forearm, upper arm, and C5 nerve root) and determined its diagnostic accuracy using the European Federation of Neurological Societies/Peripheral Nerve Society criteria of CIDP/MMN (reference standard). In addition, to determine the added value of nerve ultrasound in detecting treatment-responsive patients, we used previously published diagnostic criteria based on clinical, NCS, and sonographic findings and treatment response (alternative reference standard). RESULTS Sensitivity and specificity of the sonographic protocol for CIN according to the reference standard were 87.4% and 67.3%, respectively. Sensitivity and specificity of this protocol according to the alternative reference standard were 84.6% and 72.8%, respectively, and of NCS 76.1% and 93.4%. With addition of nerve ultrasound, 44 diagnoses of CIN were established compared to 33 diagnoses with NCS alone. CONCLUSIONS A short sonographic protocol shows high diagnostic accuracy for detecting CIN. Nerve ultrasound is able to detect up to 25% more patients who respond to treatment. CLASSIFICATION OF EVIDENCE This multicenter study provides Class IV evidence that nerve ultrasound improves diagnosis of CIN.
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Affiliation(s)
- Ingrid J T Herraets
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - H Stephan Goedee
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Johan A Telleman
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Ruben P A van Eijk
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Camiel Verhamme
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Christiaan G J Saris
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Filip Eftimov
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Nens van Alfen
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - J Thies van Asseldonk
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Leo H Visser
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Leonard H van den Berg
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Ludo W van der Pol
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., L.W.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.P.A.v.E.), University Medical Center Utrecht; Amsterdam Neuroscience (C.V., F.E.), Amsterdam University Medical Center, University of Amsterdam; and Department of Neurology (C.G.J.S., N.v.A.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.
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Lehmann HC, Wunderlich G, Fink GR, Sommer C. Diagnosis of peripheral neuropathy. Neurol Res Pract 2020; 2:20. [PMID: 33324924 PMCID: PMC7650053 DOI: 10.1186/s42466-020-00064-2] [Citation(s) in RCA: 71] [Impact Index Per Article: 14.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2020] [Accepted: 05/10/2020] [Indexed: 11/10/2022] Open
Abstract
Introduction Peripheral neuropathy represents a spectrum of diseases with different etiologies. The most common causes are diabetes, exposure to toxic substances including alcohol and chemotherapeutics, immune-mediated conditions, and gene mutations. A thorough workup including clinical history and examination, nerve conduction studies, and comprehensive laboratory tests is warranted to identify treatable causes. First steps The variability of symptoms allows distinguishing characteristic clinical phenotypes of peripheral neuropathy that should be recognized in order to stratify the diagnostic workup accordingly. Nerve conduction studies are essential to determine the phenotype (axonal versus demyelinating) and severity. Laboratory tests, including genetic testing, CSF examination, nerve imaging, and nerve biopsy, represent additional clinical tests that can be useful in specific clinical scenarios. Comments We propose a flow chart based on five common basic clinical patterns of peripheral neuropathy. Based on these five clinical phenotypes, we suggest differential diagnostic pathways in order to establish the underlying cause. Conclusions The recognition of characteristic clinical phenotypes combined with nerve conduction studies allows pursuing subsequent diagnostic pathways that incorporate nerve conduction studies and additional diagnostic tests. This two-tiered approach promises higher yield and better cost-effectiveness in the diagnostic workup in patients with peripheral neuropathy.
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Affiliation(s)
- Helmar C Lehmann
- Department of Neurology, Faculty of Medicine and University Hospital of Cologne, Kerpener Straße 62, D-50937 Köln, Germany
| | - Gilbert Wunderlich
- Department of Neurology, Faculty of Medicine and University Hospital of Cologne, Kerpener Straße 62, D-50937 Köln, Germany.,Center for Rare Diseases, Faculty of Medicine and University Hospital of Cologne, Köln, Germany
| | - Gereon R Fink
- Department of Neurology, Faculty of Medicine and University Hospital of Cologne, Kerpener Straße 62, D-50937 Köln, Germany.,Institute of Neuroscience and Medicine (INM-3), Research Centre Juelich, Jülich, Germany
| | - Claudia Sommer
- Department of Neurology, University of Würzburg, Würzburg, Germany
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Knoll A, Pal'a A, Pedro MT, Bäzner U, Schneider M, König RW, Wirtz CR, Friedrich S, Pauly M, Antoniadis G. Clinical outcome after decompression of intraneural peroneal ganglion cyst and its morphologic correlation to postoperative nerve ultrasound. J Neurosurg 2020; 133:233-239. [PMID: 31252391 DOI: 10.3171/2019.3.jns182699] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2018] [Accepted: 03/12/2019] [Indexed: 11/06/2022]
Abstract
OBJECTIVE Intraneural ganglion cysts are rare and benign mucinous lesions that affect peripheral nerves, most frequently the common peroneal nerve (CPN). The precise pathophysiological mechanisms of intraneural ganglion cyst development remain unclear. A well-established theory suggests the spread of mucinous fluid along the articular branch of the peroneal nerve as the underlying mechanism. Clinical outcome following decompression of intraneural ganglion cysts has been demonstrated to be excellent. The aim of this study was to evaluate the correlation between clinical outcome and ultrasound-detected morphological nerve features following decompression of intraneural ganglion cysts of the CPN. METHODS Data were retrospectively analyzed from 20 patients who underwent common peroneal nerve ganglion cyst decompression surgery at the Universität Ulm/Günzburg Neurosurgery Department between October 2003 and October 2017. Postoperative clinical outcome was evaluated by assessment of the muscular strength of the anterior tibial muscle, the extensor hallucis longus muscle, and the peroneus muscle according to the Medical Research Council grading system. Hypesthesia was measured by sensation testing. In all patients, postoperative morphological assessment of the peroneal nerve was conducted between October 2016 and October 2017 using the iU22 Philips Medical ultrasound system at the last routine follow-up appointment. Finally, the correlations between morphological changes in nerve ultrasound and postoperative clinical outcomes were evaluated. RESULTS During the postoperative ultrasound scan an intraneural hypoechogenic ring structure located at the medial side of the peroneal nerve was detected in 15 (75%) of 20 patients, 14 of whom demonstrated an improvement in motor function. A regular intraneural fasicular structure was identified in 3 patients (15%), who also reported recovery. In 1 patient, a recurrent cyst was detected, and 1 patient showed intraneural fibrosis for which recovery did not occur in the year following the procedure. Two patients (10%) developed neuropathic pain that could not be explained by nerve ultrasound findings. CONCLUSIONS The results of this study demonstrate significant recovery from preoperative weakness after decompression of intraneural ganglion cysts of the CPN. A favorable clinical outcome was highly correlated with an intraneural hypoechogenic ring-shaped structure on the medial side of the CPN identified during a follow-up postoperative ultrasound scan. These study results indicate the potential benefit of ultrasound scanning as a prognostic tool following decompression procedures for intraneural ganglion cysts of the CPN.
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Affiliation(s)
| | | | | | | | | | | | | | | | | | - Gregor Antoniadis
- 3Department of Neurosurgery-Section of Peripheral Nerve Surgery, University of Ulm, Günzburg, Germany
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47
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Carroll AS, Simon NG. Current and future applications of ultrasound imaging in peripheral nerve disorders. World J Radiol 2020; 12:101-129. [PMID: 32742576 PMCID: PMC7364285 DOI: 10.4329/wjr.v12.i6.101] [Citation(s) in RCA: 25] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2020] [Revised: 05/10/2020] [Accepted: 05/28/2020] [Indexed: 02/06/2023] Open
Abstract
Neuromuscular ultrasound (NMUS) is a rapidly evolving technique used in neuromuscular medicine to provide complimentary information to standard electrodiagnostic studies. NMUS provides a dynamic, real time assessment of anatomy which can alter both diagnostic and management pathways in peripheral nerve disorders. This review describes the current and future techniques used in NMUS and details the applications and developments in the diagnosis and monitoring of compressive, hereditary, immune-mediated and axonal peripheral nerve disorders, and motor neuron diseases. Technological advances have allowed the increased utilisation of ultrasound for management of peripheral nerve disorders; however, several practical considerations need to be taken into account to facilitate the widespread uptake of this technique.
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Affiliation(s)
- Antonia S Carroll
- Brain and Mind Research Centre, University of Sydney, Camperdown 2050, NSW, Australia
- Department of Neurology, Westmead Hospital, University of Sydney, Westmead 2145, NSW, Australia
- Department of Neurology, St Vincent’s Hospital, Sydney, Darlinghurst 2010, NSW, Australia
| | - Neil G Simon
- Northern Clinical School, University of Sydney, Frenchs Forest 2086, NSW, Australia
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48
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Sustained response to subcutaneous immunoglobulins in chronic ataxic neuropathy with anti-disialosyl IgM antibodies (CANDA): report of two cases and review of the literature. J Neurol 2020; 267:2353-2361. [PMID: 32347337 DOI: 10.1007/s00415-020-09843-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2019] [Revised: 04/14/2020] [Accepted: 04/16/2020] [Indexed: 10/24/2022]
Abstract
INTRODUCTION Chronic ataxic neuropathy with anti-disialosyl IgM antibodies (CANDA) is a rare disorder for which the pathological, neurophysiological, and therapeutic evidence remains anecdotal and controversial. METHODS This report on CANDA focuses on the neurophysiological patterns and treatment responses shared by two cases. One patient underwent nerve ultrasound follow-up. A comprehensive review of the literature highlighted the diverse experiences with different treatment options. RESULTS Response to different therapies was similar in both patients: intravenous immunoglobulins achieved a favorable response albeit with significant wearing-off fluctuations; treatment with subcutaneous immunoglobulins (SCIg) was an effective alternative leading to a clinical response for at least 2 years. Rituximab, which was trialed in both patients, was not continued long enough to determine its efficacy in modifying the disease course and/or modulating responsiveness to immunoglobulins. Steroids caused clinical worsening in both patients. CONCLUSIONS Immunoglobulin therapy appeared as the most effective in the treatment of these two patients. SCIg provided an effective treatment option for the long-term management of CANDA.
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49
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Shang P, Zhu M, Wang Y, Zheng X, Wu X, Zhu J, Feng J, Zhang HL. Axonal variants of Guillain-Barré syndrome: an update. J Neurol 2020; 268:2402-2419. [PMID: 32140865 DOI: 10.1007/s00415-020-09742-2] [Citation(s) in RCA: 36] [Impact Index Per Article: 7.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/16/2020] [Revised: 01/30/2020] [Accepted: 01/31/2020] [Indexed: 12/11/2022]
Abstract
Axonal variants of Guillain-Barré syndrome (GBS) mainly include acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and pharyngeal-cervical-brachial weakness. Molecular mimicry of human gangliosides by a pathogen's lipooligosaccharides is a well-established mechanism for Campylobacter jejuni-associated GBS. New triggers of the axonal variants of GBS (axonal GBS), such as Zika virus, hepatitis viruses, intravenous administration of ganglioside, vaccination, and surgery, are being identified. However, the pathogenetic mechanisms of axonal GBS related to antecedent bacterial or viral infections other than Campylobacter jejuni remain unknown. Currently, autoantibody classification and serial electrophysiology are cardinal approaches to differentiate axonal GBS from the prototype of GBS, acute inflammatory demyelinating polyneuropathy. Newly developed technologies, including metabolite analysis, peripheral nerve ultrasound, and feature selection via artificial intelligence are facilitating more accurate diagnosis of axonal GBS. Nevertheless, some key issues, such as genetic susceptibilities, remain unanswered and moreover, current therapies bear limitations. Although several therapies have shown considerable benefits to experimental animals, randomized controlled trials are still needed to validate their efficacy.
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Affiliation(s)
- Pei Shang
- Department of Neurology, First Hospital of Jilin University, Xinmin Street 71#, Changchun, 130021, China
| | - Mingqin Zhu
- Department of Neurology, First Hospital of Jilin University, Xinmin Street 71#, Changchun, 130021, China
| | - Ying Wang
- Department of Neurology, First Hospital of Jilin University, Xinmin Street 71#, Changchun, 130021, China
| | - Xiangyu Zheng
- Department of Neurology, First Hospital of Jilin University, Xinmin Street 71#, Changchun, 130021, China
| | - Xiujuan Wu
- Department of Neurology, First Hospital of Jilin University, Xinmin Street 71#, Changchun, 130021, China
| | - Jie Zhu
- Department of Neurology, First Hospital of Jilin University, Xinmin Street 71#, Changchun, 130021, China.,Department of Neurobiology, Care Sciences and Society, Karolinska Institute, Stockholm, Sweden
| | - Jiachun Feng
- Department of Neurology, First Hospital of Jilin University, Xinmin Street 71#, Changchun, 130021, China.
| | - Hong-Liang Zhang
- Department of Life Sciences, National Natural Science Foundation of China, Shuangqing Road 83#, Beijing, 100085, China.
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50
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Herraets IJT, Goedee HS, Telleman JA, van Eijk RPA, van Asseldonk JT, Visser LH, van den Berg LH, van der Pol WL. Nerve ultrasound improves detection of treatment-responsive chronic inflammatory neuropathies. Neurology 2020; 94:e1470-e1479. [PMID: 31959710 DOI: 10.1212/wnl.0000000000008978] [Citation(s) in RCA: 38] [Impact Index Per Article: 7.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/23/2019] [Accepted: 11/07/2019] [Indexed: 02/03/2023] Open
Abstract
OBJECTIVE To examine the diagnostic accuracy of nerve ultrasound in a prospective cohort of consecutive patients with a clinical suspicion of chronic inflammatory neuropathies, including chronic inflammatory demyelinating polyneuropathy, Lewis-Sumner syndrome, and multifocal motor neuropathy, and to determine the added value in the detection of treatment-responsive patients. METHODS Between February 2015 and July 2018, we included 100 consecutive incident patients with a clinical suspicion of chronic inflammatory neuropathy. All patients underwent nerve ultrasound, extensive standardized nerve conduction studies (NCS), and other relevant diagnostic investigations. We evaluated treatment response using predefined criteria. A diagnosis of chronic inflammatory neuropathy was established when NCS were abnormal (fulfilling criteria of demyelination of the European Federation of Neurological Societies/Peripheral Nerve Society) or when the degree of nerve enlargement detected by sonography was compatible with chronic inflammatory neuropathy and there was response to treatment. RESULTS A diagnosis of chronic inflammatory neuropathy was established in 38 patients. Sensitivity and specificity of nerve ultrasound and NCS were 97.4% and 69.4% and 78.9% and 93.5%, respectively. The added value of nerve ultrasound in detection of treatment-responsive chronic inflammatory neuropathy was 21.1% compared to NCS alone. CONCLUSIONS Nerve ultrasound and NCS are complementary techniques with superior sensitivity in the former and specificity in the latter. Addition of nerve ultrasound significantly improves the detection of chronic inflammatory neuropathies. Therefore, it deserves a prominent place in the diagnostic workup of chronic inflammatory neuropathies. CLASSIFICATION OF EVIDENCE This study provides Class IV evidence that nerve ultrasound is an accurate diagnostic tool to detect chronic inflammatory neuropathies.
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Affiliation(s)
- Ingrid J T Herraets
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; and Biostatistics & Research Support (R.P.A.v.E.), Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, the Netherlands
| | - H Stephan Goedee
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; and Biostatistics & Research Support (R.P.A.v.E.), Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, the Netherlands
| | - Johan A Telleman
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; and Biostatistics & Research Support (R.P.A.v.E.), Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, the Netherlands
| | - Ruben P A van Eijk
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; and Biostatistics & Research Support (R.P.A.v.E.), Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, the Netherlands
| | - J Thies van Asseldonk
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; and Biostatistics & Research Support (R.P.A.v.E.), Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, the Netherlands
| | - Leo H Visser
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; and Biostatistics & Research Support (R.P.A.v.E.), Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, the Netherlands
| | - Leonard H van den Berg
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; and Biostatistics & Research Support (R.P.A.v.E.), Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, the Netherlands
| | - W Ludo van der Pol
- From the Department of Neurology and Neurosurgery (I.J.T.H., H.S.G., J.A.T., R.P.A.v.E., L.H.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Department of Neurology and Clinical Neurophysiology (I.J.T.H., J.A.T., J.T.v.A., L.H.V.), Elisabeth-Tweesteden Hospital Tilburg; and Biostatistics & Research Support (R.P.A.v.E.), Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, the Netherlands.
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