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Hu N, Nie Y, Dong X, Niu J, Cui L, Liu M. Ultrasonographic characteristics of peripheral neuropathy in systemic lupus erythematosus. Clin Neurophysiol 2024; 170:41-48. [PMID: 39662334 DOI: 10.1016/j.clinph.2024.12.002] [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: 01/04/2024] [Accepted: 12/02/2024] [Indexed: 12/13/2024]
Abstract
OBJECTIVE To explore the ultrasonographic characteristics of SLE-related peripheral neuropathy (PN) using nerve ultrasound. METHODS Patients with SLE were recruited consecutively between December 2022 and June 2023. Detailed clinical assessment and nerve ultrasound were conducted on each SLE patient and healthy control (HC) at predetermined sites of peripheral nerves. The upper limit of the CSA for each nerve location was defined as 95th percentile in healthy controls for the identification of nerve enlargement. Nerve conduction studies (NCS) and relevant ancillary examinations were conducted on all SLE patients for comparisons. RESULTS A total of 32 SLE female patients and age- and gender-marched 52 HC were finally recruited. At M6 (P = 0.041) point of median nerve, U1 (P < 0.001) and U6-10 (P < 0.001, =0.008, <0.001, <0.001, <0.001, respectively) points of ulnar nerve, the CSA in SLE patients were remarkably higher than that in HC. Mild and moderate nerve enlargement were detected in 12 and 20 SLE patients, respectively. Only 12 tested nerves showed both axonal damage in electrophysiological studies and nerve enlargement under ultrasound. CONCLUSION Nerve enlargement was extremely common in SLE patients, mainly in the forms of mild to moderate and focal thickening in upper limbs. There was significant inconsistency between NCS and ultrasound in detecting peripheral nerve involvement and a combined examination using NCS and nerve ultrasound might be more effective in detecting SLE-related PN.
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Affiliation(s)
- Nan Hu
- Department of Neurology, Peking Union Medical College Hospital, Beijing 100730, China
| | - Yuxue Nie
- Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Beijing 100730, China
| | - Xingbei Dong
- Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Beijing 100730, China
| | - Jingwen Niu
- Department of Neurology, Peking Union Medical College Hospital, Beijing 100730, China
| | - Liying Cui
- Department of Neurology, Peking Union Medical College Hospital, Beijing 100730, China
| | - Mingsheng Liu
- Department of Neurology, Peking Union Medical College Hospital, Beijing 100730, China.
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Roll SC, Takata SC, Yao B, Kysh L, Mack WJ. Sonographic reference values for median nerve cross-sectional area: A meta-analysis of data from healthy individuals. JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY 2023; 39:492-506. [PMID: 37654772 PMCID: PMC10468154 DOI: 10.1177/87564793231176009] [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] [Indexed: 09/02/2023]
Abstract
Objective Establish median nerve CSA reference values and identify patient-level factors impacting diagnostic thresholds. Methods Studies were identified through a robust search of multiple databases, and quality assessment was conducted using a modified version of the National Institute of Health Study Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies. A meta-analysis was performed to identify normative values stratified by anatomic location. A meta-regression was conducted to examine heterogeneity effects of age, sex, and laterality. Results The meta-analysis included 73 studies; 41 (56.2%) were high quality. The median nerve CSA [95% CI] was 6.46mm2 [6.09-6.84], 8.68mm2 [8.22-9.13], and 8.60mm2 [8.23-8.97] at the proximal forearm, the carpal tunnel inlet, and the proximal carpal tunnel, respectively. Age was positively associated with CSA at the level of proximal carpal tunnel (β=0.03mm2, p=0.047). Men (9.42mm2, [8.06-10.78]) had statistically larger proximal tunnel CSA (p = 0.03) as compared to women (7.71mm2, [7.01-8.42]). No difference was noted in laterality. Conclusion A reference value for median nerve CSA in the carpal tunnel is 8.60mm2. Adjustments may be required in pediatrics or older adults. The diagnostic threshold of 10.0mm2 for male patients should be cautiously applied as the upper limit of normative averages surpasses this threshold.
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Affiliation(s)
- Shawn C. Roll
- Chan Division of Occupational Science and Occupational Therapy, University of Southern California, Los Angeles, CA, USA
| | - Sandy C. Takata
- Chan Division of Occupational Science and Occupational Therapy, University of Southern California, Los Angeles, CA, USA
| | - Buwen Yao
- Chan Division of Occupational Science and Occupational Therapy, University of Southern California, Los Angeles, CA, USA
| | - Lynn Kysh
- Institute for Nursing and Interprofessional Research, Children’s Hospital Los Angeles, Los Angeles, CA, USA
| | - Wendy J. Mack
- Division of Population and Public Health, University of Southern California, Los Angeles, CA, USA
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Yun CJ, Crump N, Puckett M, Cartwright MS. Focused Neuromuscular Ultrasound Approach for the Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy. J Clin Neurophysiol 2023; 40:378-381. [PMID: 37143208 DOI: 10.1097/wnp.0000000000000905] [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] Open
Abstract
PURPOSE Previous ultrasonographic studies of individuals with chronic inflammatory demyelinating polyneuropathy (CIDP) have shown nerve enlargement at several sites. This prospective study compares only the bilateral median and ulnar nerves of individuals with CIDP with reference values to determine the clinical usefulness of this focused approach as a diagnostic tool. METHODS The cross-sectional area, echogenicity, and vascularity of the bilateral median and ulnar nerves of 25 subjects with CIDP were measured using ultrasound. Nineteen had typical CIDP based on the European Federation of Neurological Societies and the Peripheral Nerve Society guidelines, whereas six had atypical CIDP and were diagnosed based on clinical impression. RESULTS Focal nerve enlargement was found in at least one segment in all subjects. Subjects with typical CIDP had larger cross-sectional areas compared with subjects with atypical CIDP. CONCLUSION A focused ultrasound study, involving only the median and ulnar nerves, is sensitive for the detection of nerve enlargement in CIDP. Measuring the cross-sectional area of the median and ulnar nerves is clinically feasible and may help establish the diagnosis of CIDP.
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Affiliation(s)
- Chelsea J Yun
- Department of Neurology, Wake Forest School of Medicine, Winston-Salem, North Carolina, U.S.A
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Castoro R, Caress JB, Li J, Cartwright MS. Arg953* mutation in Periaxin causes CMT4F without nerve hypertrophy on ultrasound imaging: A case report and review of the literature. Clin Neurophysiol 2023; 147:14-16. [PMID: 36623372 DOI: 10.1016/j.clinph.2022.12.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/27/2022] [Revised: 12/14/2022] [Accepted: 12/18/2022] [Indexed: 12/27/2022]
Affiliation(s)
- Ryan Castoro
- Department of Neurology, Wake Forest School of Medicine, NC, USA; Department of Neurology, Wayne State University, Detroit, MI, USA.
| | - James B Caress
- Department of Neurology, Wake Forest School of Medicine, NC, USA
| | - Jun Li
- Houston Methodist Neurological Institute, Houston, TX, USA
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刘 小, 张 朔, 马 妍, 孙 阿, 张 英, 樊 东. [Diagnostic value of F wave changes in patients with Charcot-Marie-Tooth1A and chronic inflammatory demyelinating polyneuropathy]. BEIJING DA XUE XUE BAO. YI XUE BAN = JOURNAL OF PEKING UNIVERSITY. HEALTH SCIENCES 2023; 55:160-166. [PMID: 36718706 PMCID: PMC9894793] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Subscribe] [Scholar Register] [Received: 06/01/2020] [Indexed: 02/01/2023]
Abstract
OBJECTIVE To analyze and compare the characteristics and causes of F wave changes in patients with Charcot-Marie-Tooth1A (CMT1A) and chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS Thirty patients with CMT1A and 30 patients with CIDP were enrolled in Peking University Third Hospital from January 2012 to December 2018. Their clinical data, electrophysiological data(nerve conduction velocity, F wave and H reflex) and neurological function scores were recorded. Some patients underwent magnetic resonance imaging of brachial plexus and lumbar plexus, and the results were analyzed and compared. RESULTS The average motor conduction velocity (MCV) of median nerve was (21.10±10.60) m/s in CMT1A and (31.52±12.46) m/s in CIDP. There was a significant difference between the two groups (t=-6.75, P < 0.001). About 43.3% (13/30) of the patients with CMT1A did not elicit F wave in ulnar nerve, which was significantly higher than that of the patients with CIDP (4/30, 13.3%), χ2=6.65, P=0.010. Among the patients who could elicit F wave, the latency of F wave in CMT1A group was (52.40±17.56) ms and that in CIDP group was (42.20±12.73) ms. There was a significant difference between the two groups (t=2.96, P=0.006). The occurrence rate of F wave in CMT1A group was 34.6%±39%, and that in CIDP group was 70.7%±15.2%. There was a significant difference between the two groups (t=-5.13, P < 0.001). The MCV of median nerve in a patient with anti neurofascin 155 (NF155) was 23.22 m/s, the latency of F wave was 62.9-70.7 ms, and the occurrence rate was 85%-95%. The proportion of brachial plexus and lumbar plexus thickening in CMT1A was 83.3% (5/6) and 85.7% (6/7), respectively. The proportion of brachial plexus and lumbar plexus thickening in the CIDP patients was only 25.0% (1/4, 2/8). The nerve roots of brachial plexus and lumbar plexus were significantly thickened in a patient with anti NF155 antibody. CONCLUSION The prolonged latency of F wave in patients with CMT1A reflects the homogenous changes in both proximal and distal peripheral nerves, which can be used as a method to differentiate the CIDP patients characterized by focal demyelinating pathology. Moreover, attention should be paid to differentiate it from the peripheral neuropathy caused by anti NF155 CIDP. Although F wave is often used as an indicator of proximal nerve injury, motor neuron excitability, anterior horn cells, and motor nerve myelin sheath lesions can affect its latency and occurrence rate. F wave abnormalities need to be comprehensively analyzed in combination with the etiology, other electrophysiological results, and MRI imaging.
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Affiliation(s)
- 小璇 刘
- />北京大学第三医院神经内科，北京 100191Department of Neurology, Peking University Third Hospital, Beijing 100191, China
| | - 朔 张
- />北京大学第三医院神经内科，北京 100191Department of Neurology, Peking University Third Hospital, Beijing 100191, China
| | - 妍 马
- />北京大学第三医院神经内科，北京 100191Department of Neurology, Peking University Third Hospital, Beijing 100191, China
| | - 阿萍 孙
- />北京大学第三医院神经内科，北京 100191Department of Neurology, Peking University Third Hospital, Beijing 100191, China
| | - 英爽 张
- />北京大学第三医院神经内科，北京 100191Department of Neurology, Peking University Third Hospital, Beijing 100191, China
| | - 东升 樊
- />北京大学第三医院神经内科，北京 100191Department of Neurology, Peking University Third Hospital, Beijing 100191, China
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Abdelnaby R, Elgenidy A, Sonbol YT, Dardeer KT, Ebrahim MA, Maallem I, Youssef MW, Moawad MHED, Hassan YG, Rabie SA, Mohammed MK, Abdelrhem HAH, Mohamed KA, Walter U. Nerve Sonography in Charcot-Marie-Tooth Disease: A Systematic Review and Meta-analysis of 6061 Measured Nerves. ULTRASOUND IN MEDICINE & BIOLOGY 2022; 48:1397-1409. [PMID: 35667977 DOI: 10.1016/j.ultrasmedbio.2022.04.220] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/29/2022] [Revised: 04/15/2022] [Accepted: 04/29/2022] [Indexed: 06/15/2023]
Abstract
Because of the insidious character and variations in presenting symptoms, Charcot-Marie-Tooth (CMT) disease is challenging to diagnose in children. Diagnosis is based on clinical and nerve conduction studies, as well as genetic examination. Therefore, competent nerve imaging techniques and non-invasive alternatives to nerve conduction studies are a necessity, especially in children. We performed a systematic review and meta-analysis to evaluate the current evidence and effectiveness of ultrasound in investigating nerve cross-sectional area (CSA) in those with CMT compared with healthy controls and to pool the CSA measurements. We included studies published in international peer-reviewed journals that measured nerve CSA by ultrasound in patients with CMT. We implemented double-arm meta-analyses to compare the mean CSA of nerves between patients with CMT and healthy controls by calculating the pooled mean difference in CSA. Moreover, we performed subgroup analyses by stratifying the studies according to the site of CSA measurement and examined the difference in nerve CSA between CMT1A and other CMT types. The included studies provide measurements of 12 nerve roots and nerves (vagus, C3, C4, C5, C6, greater auricular, phrenic, median, ulnar, fibular, tibial and sural nerves) in 628 patients with CMT and 586 healthy controls with a total of 6061 measured nerves. Meta-analyses of sonographic nerve CSA are provided to express nerve ultrasonography in the diagnosis of CMT patient.
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Affiliation(s)
- Ramy Abdelnaby
- Department of Neurology, RWTH Aachen University, Aachen, Germany.
| | | | | | | | | | - Imene Maallem
- Faculty of Medicine, University Badji Mokhtar Annaba, Annaba, Algeria
| | | | | | | | | | | | | | | | - Uwe Walter
- Department of Neurology, Rostock University, Rostock, Germany
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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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Niu J, Ding Q, Fan J, Zhang L, Liu J, Guan Y, Wu S, Cui L, Liu M. Nerve Ultrasound Performances in Differentiating POEMS Syndrome from CIDP. Neurotherapeutics 2022; 19:455-463. [PMID: 35257327 PMCID: PMC9226218 DOI: 10.1007/s13311-022-01209-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/19/2022] [Indexed: 10/18/2022] Open
Abstract
Chronic inflammatory demyelinating polyneuropathy (CIDP) and polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome are both acquired demyelinating polyneuropathies. We aim to explore the different features of ultrasonographic changes between CIDP and POEMS syndrome. Nerve ultrasonographic studies were performed in 120 patients with CIDP and 34 patients with POEMS syndrome. Cross-sectional areas (CSAs) were measured on the bilateral median nerve, ulnar nerve, and brachial plexus. Nerve conduction studies were performed on median and ulnar nerves to detect motor conduction blocks (CBs). CSAs at all sites were larger in patients with CIDP and POEMS syndrome than in healthy controls. Maximal CSA (median (min to max)) was 14 (6-194) mm2 for median nerve, 9 (4-92) mm2 for ulnar nerve, and 14 (7-199) mm2 for brachial plexus in CIDP and 11 (8-16) mm2 for median nerve, 8.5 (6-13) mm2 for ulnar nerve, and 14 (10-20) mm2 for brachial plexus in POEMS syndrome. The ratio of maximum/minimum CSA of the median nerve was significantly larger in CIDP (2.8 ± 2.8) than in POEMS syndrome (1.7 ± 0.3). CBs or probable CBs were detected in 60 out of 120 CIDP patients but in none of the POEMS syndromes. For distinguishing CIDP and POEMS syndrome, a two-step protocol using CB and maximum/minimum CSA of the median nerve yields a sensitivity of 93% and a specificity of 79%. In conclusion, compared with CIDP, nerve CSA enlargement was more homogeneous along the same nerve in individual POEMS patients, as well as among different POEMS patients. The addition of nerve ultrasound to nerve conduction studies significantly improves the differential diagnosis between the two diseases.
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Affiliation(s)
- Jingwen Niu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Qingyun Ding
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Jing Fan
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Lei Zhang
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Jingwen Liu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Yuzhou Guan
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Shuang Wu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Liying Cui
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Mingsheng Liu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
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Liu L, Ye Y, Wang L, Song X, Cao J, Qi Y, Xing Y. Nerve ultrasound evaluation of Guillain-Barré syndrome subtypes in northern China. Muscle Nerve 2021; 64:560-566. [PMID: 34355400 DOI: 10.1002/mus.27386] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2020] [Revised: 08/01/2021] [Accepted: 08/02/2021] [Indexed: 01/23/2023]
Abstract
INTRODUCTION/AIMS Ultrasound (US) studies have demonstrated patchy enlargement of spinal and peripheral nerves in Guillain-Barré syndrome (GBS). However, whether ultrasound yields useful information for early classification of GBS has not been established. We aimed to evaluate nerve ultrasound in patients with GBS in northern China and compare the sonographic characteristics between demyelinating and axonal subtypes. METHODS Between November 2018 and October 2019, 38 hospitalized GBS patients within 3 wk of disease onset and 40 healthy controls were enrolled. Ultrasonographic cross-sectional areas (CSA) of the peripheral nerves, vagus nerve, and cervical nerve roots were prospectively recorded in GBS subtypes and controls. RESULTS Ultrasonographic CSA exhibited significant enlargement in most patients' nerves compared with healthy controls, most prominent in cervical nerves. The CSA tended to be larger in acute inflammatory demyelinating polyneuropathy (AIDP) than in acute motor axonal neuropathy (AMAN)/acute motor and sensory axonal neuropathy (AMSAN), especially in cervical nerves (C5: 5.9 ± 1.6 mm2 vs. 7.0 ± 1.7 mm2 , p = .042; C6: 10.5 ± 1.8 mm2 vs. 12.0 ± 2.1 mm2 , p = .033). The chi-squared test revealed significant differences in nerve enlargement in C5 (p < .001), C6 (p < .001), the proximal median nerve (p < .001), and the vagus nerve (p = .003) between GBS and controls. The vagus nerve was larger in patients with autonomic dysfunction than in patients without it (2.3 ± 1.0 mm2 vs. 1.4 ± 0.5 mm2 , p = .003). DISCUSSION The demyelinating subtype presented with more significant cervical nerve enlargement in GBS. Vagus nerve enlargement may be a useful marker for autonomic dysfunction.
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Affiliation(s)
- Li Liu
- Department of Vascular Ultrasonography, Xuanwu Hospital, Capital Medical University, Beijing, China.,Center of Vascular Ultrasonography, Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, Beijing, China.,Department of Neurology, Changchun City People's hospital, Changchun, China
| | - Yuqin Ye
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Lijuan Wang
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Xiaonan Song
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Jie Cao
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Yajie Qi
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Yingqi Xing
- Department of Vascular Ultrasonography, Xuanwu Hospital, Capital Medical University, Beijing, China.,Center of Vascular Ultrasonography, Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, Beijing, China.,Department of Neurology, The First Hospital of Jilin University, Changchun, China
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Nathani D, Spies J, Barnett MH, Pollard J, Wang M, Sommer C, Kiernan MC. Nerve biopsy: Current indications and decision tools. Muscle Nerve 2021; 64:125-139. [PMID: 33629393 PMCID: PMC8359441 DOI: 10.1002/mus.27201] [Citation(s) in RCA: 18] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/15/2019] [Revised: 02/04/2021] [Accepted: 02/06/2021] [Indexed: 02/06/2023]
Abstract
After initial investigation of patients presenting with symptoms suggestive of neuropathy, a clinical decision is made for a minority of patients to undergo further assessment with nerve biopsy. Many nerve biopsies do not demonstrate a definitive pathological diagnosis and there is considerable cost and morbidity associated with the procedure. This highlights the need for appropriate selection of patients, nerves and neuropathology techniques. Additionally, concomitant muscle and skin biopsies may improve the diagnostic yield in some cases. Several advances have been made in diagnostics in recent years, particularly in genomics. The indications for nerve biopsy have consequently changed over time. This review explores the current indications for nerve biopsies and some of the issues surrounding its use. Also included are comments on alternative diagnostic modalities that may help to supplant or reduce the use of nerve biopsy as a diagnostic test. These primarily include extraneural biopsy and neuroimaging techniques such as magnetic resonance neurography and nerve ultrasound. Finally, we propose an algorithm to assist in deciding when to perform nerve biopsies.
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Affiliation(s)
- Dev Nathani
- Brain and Mind CentreUniversity of SydneySydneyNew South WalesAustralia
- Institute of Clinical Neuroscience, Royal Prince Alfred HospitalSydneyNew South WalesAustralia
| | - Judith Spies
- Brain and Mind CentreUniversity of SydneySydneyNew South WalesAustralia
- Institute of Clinical Neuroscience, Royal Prince Alfred HospitalSydneyNew South WalesAustralia
| | - Michael H. Barnett
- Brain and Mind CentreUniversity of SydneySydneyNew South WalesAustralia
- Institute of Clinical Neuroscience, Royal Prince Alfred HospitalSydneyNew South WalesAustralia
| | - John Pollard
- Brain and Mind CentreUniversity of SydneySydneyNew South WalesAustralia
- Institute of Clinical Neuroscience, Royal Prince Alfred HospitalSydneyNew South WalesAustralia
| | - Min‐Xia Wang
- Brain and Mind CentreUniversity of SydneySydneyNew South WalesAustralia
- Institute of Clinical Neuroscience, Royal Prince Alfred HospitalSydneyNew South WalesAustralia
| | - Claudia Sommer
- Neurologische KlinikUniversitätsklinikum WürzburgWürzburgGermany
| | - Matthew C. Kiernan
- Brain and Mind CentreUniversity of SydneySydneyNew South WalesAustralia
- Institute of Clinical Neuroscience, Royal Prince Alfred HospitalSydneyNew South WalesAustralia
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Du K, Xu K, Cheng S, Lv H, Zhang W, Wang Z, Yuan Y, Meng L. Nerve Ultrasound Comparison Between Transthyretin Familial Amyloid Polyneuropathy and Chronic Inflammatory Demyelinating Polyneuropathy. Front Neurol 2021; 12:632096. [PMID: 33716932 PMCID: PMC7953716 DOI: 10.3389/fneur.2021.632096] [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: 11/22/2020] [Accepted: 02/08/2021] [Indexed: 11/13/2022] Open
Abstract
Backgrounds: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is frequently misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP) because of similar phenotypes in the two diseases. This study was intended to identify the role of nerve ultrasonography in evaluating TTR-FAP and CIDP. Methods: Eighteen patients with TTR-FAP, 13 patients with CIDP, and 14 healthy controls (HC) were enrolled in this study. Consecutive ultrasonography scanning was performed in six pairs of nerves of bilateral limbs with 30 sites. The cross-sectional areas (CSAs) and CSA variability data of different groups were calculated and compared. Results: Both TTR-FAP and CIDP showed larger CSAs at most sites of both upper and lower limbs than in HC groups. CIDP patients had larger CSAs than TTR-FAP patients at 8/15 of these sites, especially at U1-3, Sci2 sites (p < 0.01). However, the CSAs at above sites were not a credible index to differentiate TTR-FAP from CIDP with a low area under the curve (<0.8). The CSA variability of median nerves was significantly higher in CIDP than in TTR-FAP and HC groups, with high sensitivity (0.692) and specificity (0.833) to differentiate CIDP from TTR-FAP. The CSA variability of ulnar nerves was not significantly different between the three groups. For the TTR-FAP group, mean CSAs at each site were not correlated with different Coutinho stages, modified polyneuropathy disability, course of sensory motor peripheral neuropathy, Neuropathy Impairment Score, or Norfolk Quality of life-diabetic neuropathy score. The mean compound muscle action potential of ulnar nerves was negatively correlated with the mean CSAs of ulnar nerves. Interpretation: TTR-FAP patients had milder nerve enlargement with less variability in CSAs of median nerves than those with CIDP, suggesting that nerve ultrasound can be a potential useful auxiliary tool to help differentiate the two neuropathies.
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Affiliation(s)
- Kang Du
- Department of Neurology, Peking University First Hospital, Beijing, China
| | - Ke Xu
- Department of Neurology, Peking University First Hospital, Beijing, China
| | - Si Cheng
- Department of Neurology, Peking University First Hospital, Beijing, China.,Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - He Lv
- Department of Neurology, Peking University First Hospital, Beijing, China
| | - Wei Zhang
- Department of Neurology, Peking University First Hospital, Beijing, China
| | - Zhaoxia Wang
- Department of Neurology, Peking University First Hospital, Beijing, China
| | - Yun Yuan
- Department of Neurology, Peking University First Hospital, Beijing, China
| | - Lingchao Meng
- Department of Neurology, Peking University First Hospital, Beijing, China
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Ding Q, Li J, Guan Y, Wu S, Niu J, Shen D, Hu Y, Wu Y, Liu M, Cui L. Nerve ultrasound studies in POEMS syndrome. Muscle Nerve 2021; 63:758-764. [PMID: 33604886 DOI: 10.1002/mus.27209] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2020] [Revised: 02/12/2021] [Accepted: 02/16/2021] [Indexed: 11/12/2022]
Abstract
INTRODUCTION The aim of our study was to assess the ultrasonographic features of peripheral nerves in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome. METHOD 34 POEMS syndrome patients and 26 healthy control (HC) participants were recruited prospectively. Cross-sectional area (CSA) was measured in nerves of limbs, trunks of brachial plexus, and cervical nerve roots RESULTS: The CSAs were mildly enlarged at the arm segment of median nerve, elbow segment of ulnar nerve and upper trunk, moderately enlarged at the forearm segment of both median and ulnar nerve, upper trunk of brachial plexus, and C6, C7 cervical nerve roots, and markedly enlarged at the arm segment of ulnar nerve, middle and lower trunk of brachial plexus, as well as C5 cervical root. DISCUSSION The CSAs of upper limb nerves were larger in POEMS syndrome patients than in HCs, and the enlargements were most prominent proximally.
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Affiliation(s)
- Qingyun Ding
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Jian Li
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Yuzhou Guan
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Shuang Wu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Jingwen Niu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Dongchao Shen
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Youfang Hu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Yinmin Wu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Mingsheng Liu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Liying Cui
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
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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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Li Y, Niu J, Liu T, Ding Q, Wu S, Guan Y, Cui L, Liu M. Conduction Block and Nerve Cross-Sectional Area in Multifocal Motor Neuropathy. Front Neurol 2019; 10:1055. [PMID: 31649606 PMCID: PMC6794440 DOI: 10.3389/fneur.2019.01055] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2019] [Accepted: 09/18/2019] [Indexed: 12/13/2022] Open
Abstract
Introduction: Motor nerve conduction block (CB) is the main electrophysiological feature of multifocal motor neuropathy (MMN). Increased cross-sectional area (CSA) can be detected by nerve ultrasound in MMN. In this study, we aim to analyze the correlation between CB and CSA in MMN. Methods: Twelve patients with MMN were recruited. Ultrasonography tests and motor nerve conduction studies (NCSs) were performed on median and ulnar nerves simultaneously. CSA was measured at 10 consecutive sites on those nerves, meanwhile nerves were traced continuously and recorded thoroughly under ultrasound. Results: In motor NCSs, 12 definite CB and 12 probable CB areas were detected across standard segments of median and ulnar nerves. With ultrasound studies, increased CSA was detected at 36 sites. There were 9 standard segments with CB and increased CSA, 15 segments with CB but normal CSA, and 27 segments with increased CSA but no CB. Discussion: In MMN, motor nerve CB was not always consistent with increased CSA.
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Affiliation(s)
- Yi Li
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Jingwen Niu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Tanxin Liu
- Department of Medical English, Peking University Health Science Center, Beijing, China
| | - Qingyun Ding
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Shuang Wu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Yuzhou Guan
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Liying Cui
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Mingsheng Liu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
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Niu J, Li Y, Liu T, Ding Q, Cui L, Guan Y, Zhang L, Liu M. Serial nerve ultrasound and motor nerve conduction studies in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 2019; 60:254-262. [PMID: 31206194 DOI: 10.1002/mus.26611] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2018] [Revised: 06/09/2019] [Accepted: 06/09/2019] [Indexed: 12/12/2022]
Abstract
INTRODUCTION The objective of this study was to evaluate the correlation between cross-sectional area (CSA) and nerve conduction studies (NCS) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and to determine how CSA changes over time after standard treatment. METHODS Fifty-four patients with CIDP were recruited prospectively, and 21 patients were followed for more than 6 months. Ultrasonography and motor NCS were performed in the median and ulnar nerves. RESULTS No or weak correlation was observed between the maximum CSA and motor conduction velocity. There were segmental nerve enlargements at 61% of sites with conduction block or temporal dispersion. Among 19 patients with clinical improvement after immunotherapy, CSA decreased to normal in 5, increased in 10, and were unchanged in 4. DISCUSSION Different patterns of CSA and motor NCS changes after immune treatment may indicate different CIDP pathologic mechanisms. Exploration of these pathologic mechanisms could guide treatment choices in the future. Muscle Nerve, 2019.
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Affiliation(s)
- Jingwen Niu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, 100730, Beijing, China
| | - Yi Li
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, 100730, Beijing, China
| | - Tanxin Liu
- Department of Medical English, Peking University Health Science Center, Beijing, 100191, China
| | - Qingyun Ding
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, 100730, Beijing, China
| | - Liying Cui
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, 100730, Beijing, China
| | - Yuzhou Guan
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, 100730, Beijing, China
| | - Lei Zhang
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, 100730, Beijing, China
| | - Mingsheng Liu
- Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, 100730, Beijing, China
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Nerve ultrasound findings differentiate Charcot-Marie-Tooth disease (CMT) 1A from other demyelinating CMTs. Clin Neurophysiol 2018; 129:2259-2267. [PMID: 30216910 DOI: 10.1016/j.clinph.2018.08.016] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/19/2018] [Revised: 08/04/2018] [Accepted: 08/21/2018] [Indexed: 12/11/2022]
Abstract
OBJECTIVE Ulnar/median motor nerve conduction velocity (MNCV) is ≤38 m/s in demyelinating Charcot-Marie-Tooth disease (CMT). Previous nerve high resolution ultrasound (HRUS) studies explored demyelinating CMT assuming it as a homogeneous genetic/pathological entity or focused on CMT1A. METHODS To explore the spectrum of nerve HRUS findings in demyelinating CMTs, we recruited patients with CMT1A (N = 44), CMT1B (N = 9), CMTX (N = 8) and CMT4C (N = 4). They underwent nerve conduction study (NCS) and HRUS of the median, ulnar, peroneal nerve, and the brachial plexus. RESULTS Median, ulnar and peroneal MNCV significantly differed across CMT subtypes. Cross sectional area (CSA) was markedly and diffusely enlarged at all sites, except entrapment ones, in CMT1A, while it was slightly enlarged or within normal range in the other CMTs. No significant right-to-left difference was found. Age had limited effect on CSA. CSAs of some CMT1A patients largely overlapped with those of other demyelinating CMTs. A combination of three median CSA measures could separate CMT1A from other demyelinating CMTs. CONCLUSIONS Nerve HRUS findings are heterogeneous in demyelinating CMTs. SIGNIFICANCE Nerve HRUS may separate CMT1A from other demyelinating CMTs. The large demyelinating CMTs HRUS spectrum may be related to its pathophysiological variability.
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Décard BF, Pham M, Grimm A. Ultrasound and MRI of nerves for monitoring disease activity and treatment effects in chronic dysimmune neuropathies – Current concepts and future directions. Clin Neurophysiol 2018; 129:155-167. [DOI: 10.1016/j.clinph.2017.10.028] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/12/2017] [Revised: 10/03/2017] [Accepted: 10/07/2017] [Indexed: 02/07/2023]
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