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Roçi E, Mara E, Dodaj S, Vyshka G. Wernicke encephalopathy presenting as a stroke mimic: A case report. World J Clin Cases 2025; 13:103585. [DOI: 10.12998/wjcc.v13.i19.103585] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/23/2024] [Revised: 02/16/2025] [Accepted: 02/24/2025] [Indexed: 03/19/2025] Open
Abstract
BACKGROUND Several conditions may present with acute neurological symptoms, thus mimicking the presentation of stroke. Although the underlying disorder can be diagnosed after careful medical, neurological, and radiological examinations, a few conditions, such as Wernicke encephalopathy (WE), may present a particular diagnostic difficulty. WE is a neurological disorder caused by deficiency of thiamine (B1 vitamin), most often resulting from alcoholism, malnutrition, hyperemesis gravidarum or bariatric surgery. The diagnosis of WE in a certain historical, clinical setting is easily suggested, but in a few cases presenting with acute neurological deficits, it can be particularly challenging.
CASE SUMMARY We present the case of a 63-year-old man who was brought to the emergency department after developing weakness of the left extremities, dizziness and a confusional state, which had lasted for approximately 30 minutes. The patient had a similar episode of a confusional state approximately two months earlier; at that time, a transient ischemic attack was suspected and he was started on aspirin. The initial clinical evaluation and imaging findings were unremarkable for stroke, but the patient’s symptoms, history of chronic alcohol abuse and abnormal liver function tests prompted the consideration of WE. Magnetic resonance imaging findings in subthalamic areas and electroencephalogram data of diffuse delta activity supported this diagnosis.
CONCLUSION Through this case report, we aim to underscore the importance of considering WE as a differential diagnosis in patients presenting with symptoms suggestive of stroke, especially when the presentation is atypical or when risk factors for thiamine deficiency are present. Since intravenous thiamine significantly improves outcomes, delayed recognition and treatment in some cases might be deleterious.
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Affiliation(s)
- Ermir Roçi
- Stroke Unit, Service of Neurology, University Hospital Center Mother Theresa, Tirana 1005, Albania
| | - Emili Mara
- Stroke Unit, University Hospital Center Mother Teresa, Tirana 1005, Albania
| | - Stela Dodaj
- Stroke Unit, University Hospital Center Mother Teresa, Tirana 1005, Albania
| | - Gentian Vyshka
- Department of Biomedical and Experimental, Faculty of Medicine, University of Medicine, Tirana 1005, Albania
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Hong J, Cho D, Kim HJ, Jo J, Seong GM. Reversible Severe Acute Lactic Acidosis Caused by Thiamine Deficiency in Intensive Care Unit. Case Rep Crit Care 2025; 2025:3211626. [PMID: 40375943 PMCID: PMC12081139 DOI: 10.1155/crcc/3211626] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2025] [Accepted: 04/18/2025] [Indexed: 05/18/2025] Open
Abstract
Lactic acidosis is a common cause of metabolic acidosis in hospitalized patients. It is typically caused by hypoperfusion and anaerobic metabolism and is often associated with sepsis. However, it can also result from impaired lactate metabolism, independent of hypoxemia. We report the case of a 50-year-old woman with severe lactic acidosis who was admitted to the intensive care unit. Lactic acidosis was initially attributed to an uncontrolled infection. However, brain magnetic resonance imaging revealed Wernicke's encephalopathy due to thiamine deficiency. The administration of high-dose intravenous thiamine rapidly improved the mental status and normalized serum lactate levels. This case highlights the importance of identifying thiamine deficiency as a reversible cause of lactic acidosis in critically ill patients.
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Affiliation(s)
- Jisu Hong
- Department of Critical Care Nursing, Jeju National University Hospital, Jeju, Republic of Korea
| | - Daehong Cho
- Department of Internal Medicine, Jeju National University Hospital, Jeju National University College of Medicine, Jeju, Republic of Korea
| | - Hong Jun Kim
- Department of Neurology, Jeju National University Hospital, Jeju National University College of Medicine, Jeju, Republic of Korea
| | - Jaemin Jo
- Department of Internal Medicine, Jeju National University Hospital, Jeju National University College of Medicine, Jeju, Republic of Korea
| | - Gil Myeong Seong
- Department of Internal Medicine, Jeju National University Hospital, Jeju National University College of Medicine, Jeju, Republic of Korea
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Carson L, Veremu M, Jesuthasan A, Ham T. Wernicke's encephalopathy presenting with confusion and hearing loss. BMJ Case Rep 2025; 18:e263527. [PMID: 39828324 DOI: 10.1136/bcr-2024-263527] [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] [Indexed: 01/22/2025] Open
Abstract
We present a case of a male in his early 50s assessed in the emergency department with a seemingly clear alcohol history but with classic symptoms of Wernicke's encephalopathy (WE): disorientation, gait ataxia and vertical nystagmus. He also had significant bilateral hearing loss and profound anterograde amnesia. Neuroimaging revealed hallmark signs of WE, including symmetrical T2/fluid-attenuated inversion recovery hyperintensity in the medial thalami. Here, we consider important differentials beyond WE, discuss the importance of revisiting the patient's history and explore the significance of his imaging findings. Although the patient's cognitive function and ocular symptoms improved on standard treatment, his condition progressed to Korsakoff's syndrome accompanied by residual anterograde amnesia and ongoing confabulation that required cognitive rehabilitation.
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Affiliation(s)
- Lucy Carson
- Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK
| | - Munashe Veremu
- Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK
| | - Aaron Jesuthasan
- Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK
| | - Timothy Ham
- Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK
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Arendt CT, Uckermark C, Kovacheva L, Lieschke F, Golbach R, Edwin Thanarajah S, Hattingen E, Weidauer S. Wernicke Encephalopathy: Typical and Atypical Findings in Alcoholics and Non-Alcoholics and Correlation with Clinical Symptoms. Clin Neuroradiol 2024; 34:881-897. [PMID: 39014226 DOI: 10.1007/s00062-024-01434-y] [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: 01/17/2024] [Accepted: 06/14/2024] [Indexed: 07/18/2024]
Abstract
PURPOSE Clinical diagnosis of Wernicke encephalopathy (WE) can be challenging due to incomplete presentation of the classical triad. The aim was to provide an update on the relevance of standard MRI and to put typical and atypical imaging findings into context with clinical features. METHODS In this two-center retrospective observational study, the local radiology information system was searched for consecutive patients with clinical or imaging suspicion of WE. Two independent raters evaluated T2-weighted imaging (WI), fluid-attenuation inversion recovery (FLAIR), diffusion WI (DWI), T2*WI and/or susceptibility WI (SWI), and contrast-enhanced (CE)-T1WI, and noted the involvement of typical (i.e., mammillary bodies (MB), periaqueductal grey (PAG), thalamus, hypothalamus, tectal plate) and atypical (all others) lesion sites. Unusual signal patterns like hemorrhages were also documented. Reported clinical features together with the diagnostic criteria of the latest guidelines of the European Federation of Neurological Societies (EFNS) were used to test for relationships with MRI biomarkers. RESULTS 47 patients with clinically confirmed WE were included (Jan '99-Apr '23; mean age, 53 yrs; 70% males). Interrater reliability for imaging findings was substantial (κ = 0.71), with lowest agreements for T2WI (κ = 0.85) compared to all other sequences and for PAG (κ = 0.65) compared to all other typical regions. In consensus, 77% (n = 36/47) of WE cases were rated MRI positive, with FLAIR (n = 36/47, 77%) showing the strongest relation (χ2 = 47.0; P < 0.001) compared to all other sequences. Microbleeds in the MB were detected in four out of ten patients who received SWI, not visible on corresponding T2*WI. Atypical findings were observed in 23% (n = 11/47) of cases, always alongside typical findings, in both alcoholics (n = 9/44, 21%) and non-alcoholics (n = 2/3, 67%). Isolated involvement of structures, explicitly PAG (n = 4/36; 11%) or MB (n = 1/36; 3%), was present but observed less frequently than combined lesions (n = 31/36; 86%). A cut-off width of 2.5 mm for the PAG on 2D axial FLAIR was established between cases and age- and sex-matched controls. An independent association was demonstrated only between short-term memory loss and changes in the MB (OR = 2.2 [95% CI: 1.1-4.5]; P = 0.024). In retrospect, EFNS criteria were positive (≥ 2 out of 4) in every case, but its count (range, 2-4) showed no significant (P = 0.427) relationship with signal changes on standard MRI. CONCLUSION The proposed sequence protocol (FLAIR, DWI, SWI and T1WI + CE) yielded good detection rates for neuroradiological findings in WE, with SWI showing microbleeds in the MB with superior detectability. However, false negative results in about a quarter of cases underline the importance of neurological alertness for the diagnosis. Awareness of atypical MRI findings should be raised, not only in non-alcoholics. There is limited correlation between clinical signs and standard MRI biomarkers.
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Affiliation(s)
- C T Arendt
- Institute of Neuroradiology, University Hospital Frankfurt, Goethe University, Frankfurt/Main, Germany.
| | - C Uckermark
- Institute of Neuroradiology, University Hospital Frankfurt, Goethe University, Frankfurt/Main, Germany
| | - L Kovacheva
- Institute of Neuroradiology, University Hospital Frankfurt, Goethe University, Frankfurt/Main, Germany
| | - F Lieschke
- Department of Neurology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany
| | - R Golbach
- Institute of Biostatistics and Mathematical Modeling, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany
| | - S Edwin Thanarajah
- Department for Psychiatry, Psychosomatic Medicine and Psychotherapy, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany
| | - E Hattingen
- Institute of Neuroradiology, University Hospital Frankfurt, Goethe University, Frankfurt/Main, Germany
| | - S Weidauer
- Institute of Neuroradiology, University Hospital Frankfurt, Goethe University, Frankfurt/Main, Germany
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Xu H, Shao N, Zhu Z, Wang P, Sun L, Xu Y. Wernicke's encephalopathy with pinpoint pupils and diplopia. BMC Neurol 2024; 24:422. [PMID: 39478488 PMCID: PMC11523574 DOI: 10.1186/s12883-024-03935-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2024] [Accepted: 10/23/2024] [Indexed: 11/03/2024] Open
Abstract
This case report presents the clinical findings of a female patient diagnosed with Wernicke's encephalopathy, characterized by pinpoint pupils. While pupillary changes can occur in Wernicke's encephalopathy, the presence of pinpoint pupils is exceedingly rare. In this report, we aim to document and discuss this unusual presentation, as well as speculate on the potential mechanisms underlying this atypical manifestation of the disease.
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Affiliation(s)
- Hongjia Xu
- Department of Neurology, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Na Shao
- Department of Neurology, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Zhengyu Zhu
- Department of Neurology, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Pin Wang
- Department of Neurology, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Lin Sun
- Department of Neurology, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Yingying Xu
- Department of Neurology, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China.
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Uchôa LRA, Brunelli JM, Alves IS, Leite CDC, Martin MDGM, Takahashi JT. Imaging of Vertigo and Dizziness: A Site-based Approach Part 3 (Brainstem, Cerebellum, and Miscellaneous). Semin Ultrasound CT MR 2024; 45:383-394. [PMID: 39374862 DOI: 10.1053/j.sult.2024.09.005] [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: 10/09/2024]
Abstract
Dizziness and vertigo, frequently associated with cerebrovascular origins, pose a substantial challenge in emergency medical settings due to their potential to be associated with severe underlying conditions. Sudden-onset dizziness, in particular, may be an early indicator of critical events such as stroke or transient ischemic attack. This comprehensive review encompasses the differential diagnosis of central causes of dizziness and vertigo, emphasizing the crucial role of imaging modalities in the accurate detection and assessment, including cerebrovascular diseases, inflammatory disorders, infections, and other conditions such as vestibular migraine and cervical spondylosis. It highlights the significance of advanced imaging techniques, particularly magnetic resonance imaging and computed tomography in identifying and distinguishing these conditions.
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Affiliation(s)
- Luiz Ricardo Araújo Uchôa
- Head and Neck Radiology and Neuroradiology Section, Department of Radiology, Hospital Sírio-Libanês, Sao Paulo-SP, Brazil.
| | - Julia Martins Brunelli
- Head and Neck Radiology and Neuroradiology Section, Department of Radiology, Hospital Sírio-Libanês, Sao Paulo-SP, Brazil.
| | - Isabela S Alves
- Head and Neck Radiology and Neuroradiology Section, Department of Radiology, Hospital Sírio-Libanês, Sao Paulo-SP, Brazil.
| | - Claudia da Costa Leite
- Neuroradiology Section, Department of Radiology, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo-SP, Brazil.
| | - Maria da Graça Morais Martin
- Neuroradiology Section, Department of Radiology, Hospital Sírio-Libanês, Sao Paulo-SP, Brazil; Neuroradiology Section, Instituto de Radiologia, Hospital das Clinicas da Universidade de Sao Paulo, Sao Paulo-SP, Brazil.
| | - Jorge Tomio Takahashi
- Head and Neck Radiology and Neuroradiology Section, Department of Radiology, Hospital Sírio-Libanês, Sao Paulo-SP, Brazil.
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Nicolini G, Marasco G, Metelli F, Fuccio L. Short-term memory loss, ataxia, and diplopia in a middle-aged woman. Intern Emerg Med 2024; 19:2081-2082. [PMID: 38861112 DOI: 10.1007/s11739-024-03667-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/07/2024] [Accepted: 05/28/2024] [Indexed: 06/12/2024]
Affiliation(s)
- Giacomo Nicolini
- Department of Medical and Surgical Sciences, University of Bologna, Via Massarenti 9, 40128, Bologna, Italy
| | - Giovanni Marasco
- Department of Medical and Surgical Sciences, University of Bologna, Via Massarenti 9, 40128, Bologna, Italy.
- IRCCS Azienda Ospedaliero Universitaria Di Bologna, Bologna, Italy.
| | - Flavio Metelli
- Department of Medical and Surgical Sciences, University of Bologna, Via Massarenti 9, 40128, Bologna, Italy
| | - Lorenzo Fuccio
- Department of Medical and Surgical Sciences, University of Bologna, Via Massarenti 9, 40128, Bologna, Italy
- IRCCS Azienda Ospedaliero Universitaria Di Bologna, Bologna, Italy
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Lahnine G, Abdourabbih Y, El Bouardi N, Haloua M, Alami B, Lamrani MYA, Boubbou M, Maaroufi M. Bilateral thalamic infarcts: Percheron territory. Radiol Case Rep 2024; 19:1344-1350. [PMID: 38292802 PMCID: PMC10825914 DOI: 10.1016/j.radcr.2023.12.024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2023] [Revised: 12/11/2023] [Accepted: 12/12/2023] [Indexed: 02/01/2024] Open
Abstract
Blood supply to the human thalami is complex and multiple variants exist. The artery of Percheron is one of those variants and is characterized by a solitary arterial trunk that branches from one of the proximal segments of either posterior cerebral artery and sup- plies blood to the paramedian thalami. Its occlusion results in bilateral paramedian thalamic infarction sometimes extending to the midbrain. We report a case of bithalamic infarction secondary to occlusion of the artery of Percheron. We will illustrate the complex clinical symptomatology and underscore the role of imaging, especially MRI, for diagnosis.
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Vasantha Shekar Reddy H, S Nagabushana D, Kattamanchi D, Dakappa A. Nystagmus in a child with nephrotic syndrome. BMJ Case Rep 2024; 17:e259734. [PMID: 38417935 PMCID: PMC10900356 DOI: 10.1136/bcr-2024-259734] [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] [Indexed: 03/01/2024] Open
Abstract
We report a child with steroid-dependent nephrotic syndrome presenting with excessive irritability, double vision and inability to walk for 5 days. On examination, the child was irritable with Glasgow coma sccale (GCS of 12/15, had bilateral convergent squint (R>L), vertical nystagmus, ataxia without any focal neurological deficits and normal fundus. MRI brain with venogram showed bilateral symmetric FLAIR hyperintensity in the medial thalamus and periaqueductal grey matter showing diffuse restriction with normal venogram. A possibility of Wernicke encephalopathy (WE) was considered and the child was started on thiamine supplementation, following which he had significant improvement in his symptoms. His irritability reduced with significant improvement in the range of eye movements and vertical nystagmus. At 3-month follow-up, the child is asymptomatic with normal gait. Although WE is uncommon in children with nephrotic syndrome, the possibility has to be kept in mind when a child presents with atypical neurological symptoms.
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Affiliation(s)
| | | | | | - Ahish Dakappa
- Pediatrics, MS Ramaiah Medical College Hospital, Bangalore, India
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Mazzacane F, Ferrari F, Malvaso A, Mottese Y, Gastaldi M, Costa A, Pichiecchio A, Cavallini A. Acute amnestic syndrome in fornix lesions: a systematic review of reported cases with a focus on differential diagnosis. Front Neurol 2024; 15:1338291. [PMID: 38333604 PMCID: PMC10850356 DOI: 10.3389/fneur.2024.1338291] [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: 11/15/2023] [Accepted: 01/09/2024] [Indexed: 02/10/2024] Open
Abstract
Introduction Acute amnestic syndrome is an uncommon clinical presentation of neurological disease. Differential diagnosis encompasses several syndromes including Wernicke-Korsakoff and transient global amnesia (TGA). Structural lesions of the fornix account for a minority of cases of acute amnestic syndromes. Etiology varies from iatrogenic injury to ischemic, inflammatory, or neoplastic lesions. A prompt diagnosis of the underlying pathology is essential but challenging. The aim of this review is to systematically review the existing literature regarding cases of acute amnestic syndrome associated with non-iatrogenic lesions of the fornix. Methods We performed a systematic literature search on PubMed, Scopus, and Web of Science up to September 2023 to identify case reports and case series of patients with amnestic syndrome due to fornix lesions. The systematic review was conducted according to PRISMA guidelines. The research was limited to articles written in English. Cases of fornix damage directly ascribable to a surgical procedure were excluded. Results A total of 52 publications reporting 55 cases were included in the review. Focusing on acute/subacute onset, vascular etiology was highly prevalent, being responsible for 78% of cases, 40/55 (74%) of which were due to acute ischemic stroke. The amnestic syndrome was characterized by anterograde amnesia in all patients, associated with retrograde amnesia in 27% of cases. Amnesia was an isolated presentation in most cases. Up to two thirds of patients had persistent memory deficits of any severity at follow-up. Discussion Acute amnestic syndrome can be rarely caused by fornix lesions. In most cases of acute/subacute presentation, the etiology is ischemic stroke, mainly caused by strokes involving the subcallosal artery territory. The differential diagnosis is challenging and a distinction from common mimics is often difficult on a clinical basis. A high index of suspicion should be maintained to avoid misdiagnosis and provide adequate acute treatment to patients with time-dependent disease, also employing advanced neuroimaging. More research is needed to better understand the outcome and identify prognostic factors in patients with amnestic syndrome due to fornix lesions.
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Affiliation(s)
- F. Mazzacane
- Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy
- Department of Emergency Neurology and Stroke Unit, IRCCS Fondazione Mondino, Pavia, Italy
- Unit of Behavioral Neurology, Mondino Foundation, Pavia, Italy
| | - F. Ferrari
- Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy
- Department of Emergency Neurology and Stroke Unit, IRCCS Fondazione Mondino, Pavia, Italy
| | - A. Malvaso
- Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy
- Neuroimmunology Unit, IRCCS Mondino Foundation, Pavia, Italy
| | - Y. Mottese
- Department of Neuroradiology, IRCCS Mondino Foundation, Pavia, Italy
| | - M. Gastaldi
- Neuroimmunology Unit, IRCCS Mondino Foundation, Pavia, Italy
| | - A. Costa
- Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy
- Unit of Behavioral Neurology, Mondino Foundation, Pavia, Italy
| | - A. Pichiecchio
- Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy
- Department of Neuroradiology, IRCCS Mondino Foundation, Pavia, Italy
| | - A. Cavallini
- Department of Emergency Neurology and Stroke Unit, IRCCS Fondazione Mondino, Pavia, Italy
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García de Andoin Sojo C, Gómez Muga JJ, Aza Martínez I, Antón Méndez L, Fornell Pérez R. Inflammatory lesions of the brainstem: Keys for the diagnosis by MRI. RADIOLOGIA 2024; 66:32-46. [PMID: 38365353 DOI: 10.1016/j.rxeng.2023.01.010] [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: 09/20/2022] [Accepted: 01/15/2023] [Indexed: 02/18/2024]
Abstract
OBJECTIVE To describe the magnetic resonance imaging (MRI) findings for the most common inflammatory and immune-mediated diseases that involve the brainstem. CONCLUSION Inflammatory lesions involving the brainstem are associated with a wide range of autoimmune, infectious, and paraneoplastic syndromes, making the differential diagnosis complex. Being familiar with these entities, their clinical characteristics, and their manifestations on MRI, especially the number of lesions, their shape and extension, and their appearance in different sequences, is useful for orienting the radiological diagnosis.
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Affiliation(s)
| | - J J Gómez Muga
- Servicio de Radiodiagnóstico, Hospital Universitario Basurto, Bilbao, Spain
| | - I Aza Martínez
- Servicio de Radiodiagnóstico, Hospital Universitario Basurto, Bilbao, Spain
| | - L Antón Méndez
- Servicio de Radiodiagnóstico, Hospital Universitario Basurto, Bilbao, Spain
| | - R Fornell Pérez
- Servicio de Radiodiagnóstico, Hospital Universitario Basurto, Bilbao, Spain
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Eva L, Brehar FM, Florian IA, Covache-Busuioc RA, Costin HP, Dumitrascu DI, Bratu BG, Glavan LA, Ciurea AV. Neuropsychiatric and Neuropsychological Aspects of Alcohol-Related Cognitive Disorders: An In-Depth Review of Wernicke's Encephalopathy and Korsakoff's Syndrome. J Clin Med 2023; 12:6101. [PMID: 37763040 PMCID: PMC10532206 DOI: 10.3390/jcm12186101] [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: 08/14/2023] [Revised: 09/16/2023] [Accepted: 09/18/2023] [Indexed: 09/29/2023] Open
Abstract
Alcohol-related cognitive disorders have long been an area of study, yet they continue to pose challenges in the diagnosis, treatment, and understanding of underlying neuropsychiatric mechanisms. The present article offers a comprehensive review of Wernicke's Encephalopathy and Korsakoff's Syndrome, two conditions often seen on a continuum of alcohol-related brain damage. Drawing on current medical literature, neuroimaging studies, and clinical case reports, we explore the neuropsychiatric and neuropsychological profiles, symptomatology, and differential diagnoses of these disorders. We delve into the biochemical pathways implicated in the development of WE and KS, notably thiamine deficiency and its impact on neurotransmitter systems and neural networks. The article also addresses the challenges in early diagnosis, often complicated by non-specific symptoms and co-occurring psychiatric conditions. Furthermore, we review the current state of treatment protocols, including pharmacological and non-pharmacological interventions. Finally, the article highlights gaps in current knowledge and suggests directions for future research to improve diagnosis, treatment, and patient outcomes. Understanding the nuanced interplay between the neuropsychiatric and neuropsychological aspects of WE and KS is crucial for both clinicians and researchers alike, in order to provide effective treatment and to advance our understanding of these complex conditions.
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Affiliation(s)
- Lucian Eva
- Faculty of Medicine, Dunarea de Jos University, 800010 Galati, Romania;
- Department of Neurosurgery, Clinical Emergency Hospital “Prof. Dr. Nicolae Oblu”, 700309 Iasi, Romania
| | - Felix-Mircea Brehar
- Department of Neurosurgery, Clinical Emergency Hospital “Bagdasar-Arseni”, 041915 Bucharest, Romania
- Department of Neurosurgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania; (R.-A.C.-B.); (H.P.C.); (D.-I.D.); (B.-G.B.); (L.-A.G.); (A.V.C.)
| | - Ioan-Alexandru Florian
- Department of Neurosciences, “Iuliu Hatieganu” University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania
| | - Razvan-Adrian Covache-Busuioc
- Department of Neurosurgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania; (R.-A.C.-B.); (H.P.C.); (D.-I.D.); (B.-G.B.); (L.-A.G.); (A.V.C.)
| | - Horia Petre Costin
- Department of Neurosurgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania; (R.-A.C.-B.); (H.P.C.); (D.-I.D.); (B.-G.B.); (L.-A.G.); (A.V.C.)
| | - David-Ioan Dumitrascu
- Department of Neurosurgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania; (R.-A.C.-B.); (H.P.C.); (D.-I.D.); (B.-G.B.); (L.-A.G.); (A.V.C.)
| | - Bogdan-Gabriel Bratu
- Department of Neurosurgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania; (R.-A.C.-B.); (H.P.C.); (D.-I.D.); (B.-G.B.); (L.-A.G.); (A.V.C.)
| | - Luca-Andrei Glavan
- Department of Neurosurgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania; (R.-A.C.-B.); (H.P.C.); (D.-I.D.); (B.-G.B.); (L.-A.G.); (A.V.C.)
| | - Alexandru Vlad Ciurea
- Department of Neurosurgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania; (R.-A.C.-B.); (H.P.C.); (D.-I.D.); (B.-G.B.); (L.-A.G.); (A.V.C.)
- Neurosurgery Department, Sanador Clinical Hospital, 010991 Bucharest, Romania
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Bakirtzis C, Lima M, De Lorenzo SS, Artemiadis A, Theotokis P, Kesidou E, Konstantinidou N, Sintila SA, Boziki MK, Parissis D, Ioannidis P, Karapanayiotides T, Hadjigeorgiou G, Grigoriadis N. Secondary Central Nervous System Demyelinating Disorders in the Elderly: A Narrative Review. Healthcare (Basel) 2023; 11:2126. [PMID: 37570367 PMCID: PMC10418902 DOI: 10.3390/healthcare11152126] [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: 06/07/2023] [Revised: 07/14/2023] [Accepted: 07/24/2023] [Indexed: 08/13/2023] Open
Abstract
Secondary demyelinating diseases comprise a wide spectrum group of pathological conditions and may either be attributed to a disorder primarily affecting the neurons or axons, followed by demyelination, or to an underlying condition leading to secondary damage of the myelin sheath. In the elderly, primary demyelinating diseases of the central nervous system (CNS), such as multiple sclerosis, are relatively uncommon. However, secondary causes of CNS demyelination may often occur and in this case, extensive diagnostic workup is usually needed. Infectious, postinfectious, or postvaccinal demyelination may be observed, attributed to age-related alterations of the immune system in this population. Osmotic disturbances and nutritional deficiencies, more commonly observed in the elderly, may lead to conditions such as pontine/extrapontine myelinolysis, Wernicke encephalopathy, and demyelination of the posterior columns of the spinal cord. The prevalence of malignancies is higher in the elderly, sometimes leading to radiation-induced, immunotherapy-related, or paraneoplastic CNS demyelination. This review intends to aid clinical neurologists in broadening their diagnostic approach to secondary CNS demyelinating diseases in the elderly. Common clinical conditions leading to secondary demyelination and their clinical manifestations are summarized here, while the current knowledge of the underlying pathophysiological mechanisms is additionally presented.
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Affiliation(s)
- Christos Bakirtzis
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Maria Lima
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Sotiria Stavropoulou De Lorenzo
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Artemios Artemiadis
- Faculty of Medicine, University of Cyprus, Nicosia CY-2029, Cyprus; (A.A.); (G.H.)
| | - Paschalis Theotokis
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Evangelia Kesidou
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Natalia Konstantinidou
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Styliani-Aggeliki Sintila
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Marina-Kleopatra Boziki
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Dimitrios Parissis
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Panagiotis Ioannidis
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | - Theodoros Karapanayiotides
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
| | | | - Nikolaos Grigoriadis
- Second Department of Neurology, Aristotle University of Thessaloniki, GR-54124 Thessaloniki, Greece; (M.L.); (S.S.D.L.); (P.T.); (E.K.); (N.K.); (S.-A.S.); (M.-K.B.); (D.P.); (P.I.); (T.K.); (N.G.)
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Habas E, Farfar K, Errayes N, Rayani A, Elzouki AN. Wernicke Encephalopathy: An Updated Narrative Review. SAUDI JOURNAL OF MEDICINE & MEDICAL SCIENCES 2023; 11:193-200. [PMID: 37533659 PMCID: PMC10393093 DOI: 10.4103/sjmms.sjmms_416_22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 08/21/2022] [Revised: 12/27/2022] [Accepted: 06/07/2023] [Indexed: 08/04/2023]
Abstract
Wernicke's encephalopathy (WE) and Korsakoff Syndrome (KS) are distinct neurological disorders that may have overlapping clinical features. Due to the overlap, they are collectively known as Wernicke-Korsakoff syndrome. WE is related to diencephalic and mesencephalic dysfunction due to thiamine. WE typically manifests as confusion, ophthalmoplegia, nystagmus, and gait ataxia (Wernicke's triad), although they may not consistently occur together. Although WE mostly occurs in alcoholics, other etiologies, such as post-bariatric surgery, must be considered. Early diagnosis and therapy by intravenous thiamine are essential to prevent WE complications and to reduce morbidity and mortality. Therefore, physicians' and patients' awareness of WE is essential for early diagnosis and therapy. Accordingly, this narrative review aimed to provide an update on WE by reviewing articles published between April 2015 to April 2022 about the etiology, pathophysiology, diagnosis, and WE management updates. EMBASE, PubMed, Google Scholar, Google, and Scopus search engines were used to conduct the literature search.
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Affiliation(s)
- Elmukhtar Habas
- Department of Medicine, Hamad Medical Corporation, Doha, Qatar
| | - Kalifa Farfar
- Department of Medicine, Hamad Medical Corporation, Doha, Qatar
| | - Nada Errayes
- Department of Epidemiology, University of Lincoln, Lincoln, UK
| | | | - Abdel-Naser Elzouki
- Department of Medicine, Hamad Medical Corporation, Doha, Qatar
- Department of Medicine, Weill Cornell Medical College, Doha, Qatar
- Department of Medicine, College of Medicine, Qatar University, Doha, Qatar
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15
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García de Andoin Sojo C, Gómez Muga J, Aza Martínez I, Antón Méndez L, Fornell Pérez R. Lesiones inflamatorias del troncoencéfalo: claves diagnósticas en RM. RADIOLOGIA 2023. [DOI: 10.1016/j.rx.2023.01.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/24/2023]
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Rodriguez DN, Gera K, Paudel B, Pham A. Wernicke's Encephalopathy in Type 2 Achalasia: Case Report and Literature Review. J Investig Med High Impact Case Rep 2023; 11:23247096231190628. [PMID: 37539958 PMCID: PMC10403980 DOI: 10.1177/23247096231190628] [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: 02/20/2023] [Revised: 06/07/2023] [Accepted: 07/09/2023] [Indexed: 08/05/2023] Open
Abstract
Achalasia is primarily a smooth muscle motility disorder of the esophagus driven by aberrant peristalsis and failure of sphincter relaxation. Notably, achalasia is a heterogeneous disease with primarily 3 possible pattern subtypes. According to the review of current cases and literature regarding achalasia, patients primarily present with dysphagia, usually to solids and, if progressed, to solids and liquids. Rarely, untreated achalasia may result in thiamine deficiency and present as Wernicke-Korsakoff syndrome (WKS). This acute neurologic condition primarily affects the central and peripheral nervous system and is known by the triad of ataxia, ophthalmoplegia, and confusion. Individuals who present with WKS typically have a notable history of chronic alcohol abuse with decreased thiamine intake and metabolism. Although less common, individuals with WKS may have a pertinent history of starvation, anorexia nervosa, and malnutrition. This case highlights a unique presentation of Wernicke's encephalopathy (WE) in a 30-year-old woman with severe type II achalasia complicated by a 60-pound weight loss in a span of 2 months. According to our literature review, there have only been 2 previously reported cases of severe achalasia leading to the development of WE. Considering the limited number of case reports available, WE must be in the differentials in patients with underlying achalasia, and our case report highlights this unusual presentation with corresponding brain imaging and manometry testing.
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17
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Wijdicks EFM. Identifying encephalopathies from acute metabolic derangements. J Intern Med 2022; 292:846-857. [PMID: 35809045 DOI: 10.1111/joim.13538] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Abstract
Metabolic derangements, when acute and severe, affect brain function. This presents mostly with a marked decline in the level of consciousness, resulting in impaired responsiveness, abnormal receptivity, impaired content, and loss of memory retention. The term metabolic encephalopathy has been used but is conjecture that can be challenged in the age of modern neuroimaging. We now recognize that many metabolic encephalopathies may involve structural lesions and at an early stage. Common clinical conundrums are the evaluation of the degree of brain injury and its recoverability. This review discusses the appropriate terminology for these conditions, the diagnostic approach, therapy recommendations, and prediction of recovery potential. In evaluating a presumed metabolic cause for encephalopathy, we must (1) search for and rule out structural injury to the brain despite an obvious explanatory metabolic derangement, (2) recognize that several confounding conditions often co-exist, and (3) acknowledge that resolution of brain dysfunction may be protracted despite normalization of laboratory values.
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Affiliation(s)
- Eelco F M Wijdicks
- Division of Neurocritical Care and Hospital Neurology, Mayo Clinic, Rochester, Minnesota, USA
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18
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Kuzume D, Morimoto Y, Yamasaki M, Hosomi N. [Wernicke encephalopathy with lesions in the bilateral abducens nuclei: a case report]. Rinsho Shinkeigaku 2022; 62:869-872. [PMID: 36288967 DOI: 10.5692/clinicalneurol.cn-001783] [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] [Indexed: 06/16/2023]
Abstract
A 35-year-old Japanese man had been treated for alcoholism until 6 months before coming to our hospital, after which he discontinued treatment for alcoholism. He noticed dizziness from two weeks ago. He visited our hospital because his dizziness was worsened and he noticed diplopia from two days ago. Physical examination revealed bilateral abducens nerve palsy, decreased limb tendon reflex, and ataxia. His blood vitamin B1 level was 16 ng/ml (normal range 24-66 ng/ml). FLAIR images on brain MRI showed high signal intensity lesions in the bilateral abducens nuclei and mammillary body. We diagnosed him as Wernicke encephalopathy (WE) with lesions in the bilateral abducens nuclei. Treatment with thiamine rapidly resulted in improvement of his neurological symptoms and MRI findings. He was discharged from our department on the 10th hospitalization day. Previous reports have shown that abducens nerve palsy and horizontal gaze evoked nystagmus may occur in the early state of WE. This case report highlights the importance to comprehend the atypical MRI findings of WE to treat a patient at the early stage.
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Divya MB, Kubera NS, Jha N, Jha AK, Thabah MM. Atypical neurological manifestations in Wernicke's encephalopathy due to hyperemesis gravidarum. Nutr Neurosci 2022; 25:2051-2056. [PMID: 34042559 DOI: 10.1080/1028415x.2021.1931781] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
Abstract
OBJECTIVES Hyperemesis gravidarum is known to induce nutritional, water and electrolyte deficiencies which can be fatal if not treated urgently. Thiamine deficiency may lead to a constellation of neurological symptoms that include Wernicke encephalopathy. Moreover, Wernicke encephalopathy is typically manifested as ocular paresis, ataxia and confusion. METHODS Retrospective review of 6 women who developed neurological abnormalities following hyperemesis gravidarum and were treated with varying dosage of parenteral thiamine. RESULTS Five women developed atypical neurological symptoms, namely, slurred speech, visual loss, seizure and aggressive behaviour while one woman developed typical clinical triad of Wernicke encephalopathy after hyperemesis gravidarum. Magnetic Resonance Imaging (MRI) scans revealed abnormalities suggestive of Wernicke encephalopathy in three women only. All women improved after parenteral thiamine administration during hospital stay and had a complete neurological recovery during 2 months follow up. DISCUSSION Wernicke encephalopathy may not be necessarily associated with the typical neurological triad and may not have noticeable hyperintensity signal in dorsomedial thalami, mammillary bodies, hippocampus and periaqueductal region during magnetic resonance imaging. Atypical neurological signs and symptoms following hyperemesis gravidarum would invariably respond immediately to appropriate dosage of parenteral thiamine. A lower loading dosage of thiamine (100 mg thrice daily) appeared adequate for management in women with normal MRI scans.
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Affiliation(s)
- M B Divya
- Department of Obstetrics and Gynaecology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - N S Kubera
- Department of Obstetrics and Gynaecology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Nivedita Jha
- Department of Obstetrics and Gynaecology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Ajay Kumar Jha
- Department of Anaesthesiology and Critical Care, Jawaharlal Institute of Medical Education and Research, Pondicherry, India
| | - Molly Mary Thabah
- Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
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20
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Biswas S, Pendharkar HS, Murumkar VS. MRI Spectrum of Toxic Encephalopathy-An Institutional Experience. Neurol India 2022; 70:1525-1533. [PMID: 36076654 DOI: 10.4103/0028-3886.355127] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
Background There are numerous toxins that affect our nervous system, both central and peripheral. Innumerable differentials exist in patients of acute encephalopathy and the list can be narrowed down with appropriate imaging. Specific neuroradiological features point to a particular diagnosis in a substantial number of cases. Objective Through this study, we aimed to demonstrate the varied imaging findings of toxic encephalopathy on MRI encountered at our institute. Material and Methods A retrospective analysis of the patients clinically diagnosed as toxic encephalopathy and referred for imaging between March 2015 and December 2019 was done. A total of 25 patients were included. Patient records were reviewed for clinical details, laboratory investigations, and treatment; the institute Picture Archiving and Communication System provided the imaging findings. Results Patients presenting were aged between 22 and 55 years (mean-34.3 years). Four patients (16%) presented with imaging findings characteristic of Marchiafava-Bignami disease and six patients (24%) had MRI findings of Wernicke encephalopathy. Three patients (12%) had methanol poisoning sequelae while imaging findings of nitroimidazole drug toxicity were observed in another three patients (12%). Two patients (8%) each of carbon monoxide poisoning and lead toxicity were seen. We had one patient (4%) each of isoniazid, methyl iodide, dextropropoxyphene toxicity, chronic toluene abuse, and hyperglycemia-induced hemiballismus-hemichorea. Conclusion Our study illustrates the amalgamated spectrum of MRI appearances in various subgroups of toxic encephalopathies. Imaging substantiated by relevant history and clinical manifestations can accurately diagnose the possible causative agent in the majority of the cases.
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Affiliation(s)
- Shamick Biswas
- Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India
| | - Hima S Pendharkar
- Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India
| | - Vivek S Murumkar
- Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India
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21
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Zhang Y, Wang L, Jiang J, Chen WY. Non-alcoholic Wernicke encephalopathy in an esophageal cancer patient receiving radiotherapy: A case report. World J Clin Cases 2022; 10:5810-5815. [PMID: 35979132 PMCID: PMC9258394 DOI: 10.12998/wjcc.v10.i17.5810] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/01/2021] [Revised: 01/13/2022] [Accepted: 04/23/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Wernicke encephalopathy is a rare but potentially fatal adverse event caused by thiamine deficiency. Reports of non-alcoholic Wernicke encephalopathy due to malignancy are scarce in the literature, with those reported mainly being on haematological cancer, followed by gastrointestinal cancer. As a result, there is considerable under-recognition and delay in the diagnosis and treatment of Wernicke encephalopathy in oncology departments. To our knowledge, there has been no report of Wernicke encephalopathy in a patient with esophageal cancer while receiving radiotherapy.
CASE SUMMARY A 64-year-old man presented to the oncology outpatient clinic with a history of dysphagia for 2 mo, and was diagnosed with locally advanced esophageal squamous cell carcinoma (stage IIIB). Radiotherapy was initiated to alleviate dysphagia due to malignant esophageal stenosis; however, the patient exhibited consciousness disturbances starting on day 10 of radiotherapy. Brain magnetic resonance imaging indicated the development of Wernicke encephalopathy. Subsequent treatment with thiamine led to rapid improvement in the patient’s neurological symptoms.
CONCLUSION Wernicke encephalopathy may develop in non-alcoholic patients undergoing radiotherapy for esophageal cancer. Early diagnosis and sufficient thiamine supplementation during radiotherapy are essential.
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Affiliation(s)
- Ye Zhang
- Department of General Practice, The Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
| | - Lei Wang
- Department of General Practice, The Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
| | - Jin Jiang
- Department of Oncology, The Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
| | - Wen-Yu Chen
- Department of Respiration, The Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
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Meys KME, de Vries LS, Groenendaal F, Vann SD, Lequin MH. The Mammillary Bodies: A Review of Causes of Injury in Infants and Children. AJNR Am J Neuroradiol 2022; 43:802-812. [PMID: 35487586 PMCID: PMC9172959 DOI: 10.3174/ajnr.a7463] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/18/2021] [Accepted: 11/22/2021] [Indexed: 12/20/2022]
Abstract
Despite their small size, the mammillary bodies play an important role in supporting recollective memory. However, they have typically been overlooked when assessing neurologic conditions that present with memory impairment. While there is increasing evidence of mammillary body involvement in a wide range of neurologic disorders in adults, very little attention has been given to infants and children. Literature searches of PubMed and EMBASE were performed to identify articles that describe mammillary body pathology on brain MR imaging in children. Mammillary body pathology is present in the pediatric population in several conditions, indicated by signal change and/or atrophy on MR imaging. The main causes of mammillary body pathology are thiamine deficiency, hypoxia-ischemia, direct damage due to masses or hydrocephalus, or deafferentation resulting from pathology within the wider Papez circuit. Optimizing scanning protocols and assessing mammillary body status as a standard procedure are critical, given their role in memory processes.
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Affiliation(s)
- K M E Meys
- From the Department of Radiology (K.M.E.M., F.G., M.H.L.), Wilhelmina Children's Hospital, University Medical Center Utrecht and Utrecht University, Utrecht, Netherlands
| | - L S de Vries
- Department of Neonatology (L.S.D.V.), Wilhelmina Children's Hospital, University Medical Center Utrecht and Utrecht University, Utrecht, Netherlands
| | - F Groenendaal
- From the Department of Radiology (K.M.E.M., F.G., M.H.L.), Wilhelmina Children's Hospital, University Medical Center Utrecht and Utrecht University, Utrecht, Netherlands
| | - S D Vann
- School of Psychology (S.D.V.), Cardiff University, Cardiff, UK
| | - M H Lequin
- From the Department of Radiology (K.M.E.M., F.G., M.H.L.), Wilhelmina Children's Hospital, University Medical Center Utrecht and Utrecht University, Utrecht, Netherlands
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Bagash H, Marwat A, Marwat A, Kraus B. A Case of Chronic Wernicke Encephalopathy (WE): An Underdiagnosed Phenomena. Cureus 2021; 13:e19100. [PMID: 34868752 PMCID: PMC8627220 DOI: 10.7759/cureus.19100] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/28/2021] [Indexed: 12/15/2022] Open
Abstract
Wernicke encephalopathy (WE) is the most common neurological complication of thiamine deficiency in patients who have a background of chronic alcohol use disorder. WE is characterized by acute onset of confusion, gait ataxia, and oculomotor dysfunction. Prompt treatment with parenteral thiamine leads to improvement. Untreated WE has mortality rates of up to 20% and many cases progress to the more chronic Korsakoff syndrome. Cases of untreated WE in which symptoms last beyond the acute phase and become chronic are rarely found in the literature. Here, we present a case of a 64-year-old female having a background of chronic alcohol use disorder presenting with symptoms of gait ataxia, recurrent falls, and decreased concentration. These symptoms had progressed over a period of nine months. The patient was seen by her family physician and several specialists undergoing many diagnostic studies with inconclusive results. Ultimately, with a high index of suspicion for thiamine deficiency, she was admitted for IV thiamine treatment. Upon follow-up in the clinic, the patient reported improvement in her balance and concentration further confirming the initial suspicion of WE with thiamine deficiency as the cause of her symptoms. This case corroborates the existence of WE as a chronic phenomenon in addition to the more commonly reported acute WE. Furthermore, this case highlights the importance of recognizing WE as a potential cause of chronic neurological symptoms in people with alcohol-related disorders and the role of IV thiamine in treatment.
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Affiliation(s)
- Hina Bagash
- Internal Medicine, Rawalpindi Medical University, Rawalpindi, PAK
| | - Assad Marwat
- Internal Medicine, Shifa Tameer-E-Millat University, Shifa College of Medicine, Islamabad, PAK
| | - Asghar Marwat
- Internal Medicine, Prairie Ridge Health, Sun Prairie, USA.,Internal Medicine, Conemaugh Memorial Medical Center, Johnstown, USA
| | - Bruce Kraus
- Internal Medicine, Prairie Ridge Health, Columbus, USA
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24
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Zuccoli G. The importance of knowing the typical and atypical imaging findings of Wernicke encephalopathy. Acta Neurol Belg 2021; 121:1399-1400. [PMID: 32965649 DOI: 10.1007/s13760-020-01482-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/12/2020] [Accepted: 08/17/2020] [Indexed: 11/26/2022]
Affiliation(s)
- Giulio Zuccoli
- Program for the Study of Neurodevelopment in Rare Disorders (NDRD), University of Pittsburgh, Children Hospital of Pittsburgh, 4401 Penn Ave, Pittsburgh, PA, 15224, USA.
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25
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Petechial Hemorrhage in Wernicke Encephalopathy : Imaging and Clinical Significance. Clin Neuroradiol 2021; 32:309-312. [PMID: 34324006 DOI: 10.1007/s00062-021-01064-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2021] [Accepted: 06/29/2021] [Indexed: 10/20/2022]
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26
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Furtmann JK, Sichtermann T, Oros-Peusquens AM, Dekeyzer S, Shah NJ, Wiesmann M, Nikoubashman O. MRI Analysis Of the Water Content Change In the Brain During Acute Ethanol Consumption Via Quantitative Water Mapping. Alcohol Alcohol 2021; 57:429-436. [PMID: 34002208 DOI: 10.1093/alcalc/agab026] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2020] [Revised: 03/15/2021] [Accepted: 03/16/2021] [Indexed: 11/13/2022] Open
Abstract
AIMS Alcohol consumption influences the water balance in the brain. While the impact of chronic alcohol misuse on cerebral water content has been the subject of several studies, less is known about the effects of acute alcohol misuse, with contradictory results in the literature. Therefore, we investigated the effects of acute alcohol intoxication on cerebral water content using a precise quantitative magnetic resonance imaging (MRI) sequence. METHODS In a prospective study, we measured cerebral water content in 20 healthy volunteers before alcohol consumption and after reaching a breath alcohol concentration of 1 ‰. A quantitative MRI water mapping sequence was conducted on a clinical 3 T system. Non-alcoholic fluid input and output were documented and accounted for. Water content was assessed for whole brain, grey and white matter and more specifically for regions known to be affected by acute or chronic alcohol misuse (occipital and frontal lobes, thalamus and pons). Changes in the volume of grey and white matter as well as the whole brain were examined. RESULTS Quantitative cerebral water content before and after acute alcohol consumption did not differ significantly (P ≥ 0.07), with changes often being within the range of measurement accuracy. Whole brain, white and grey matter volume did not change significantly (P ≥ 0.12). CONCLUSION The results of our study show no significant water content or volume change in the brain after recent alcohol intake in healthy volunteers. This accounts for the whole brain, grey and white matter, occipital and frontal lobes, thalamus and pons.
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Affiliation(s)
- Johanna K Furtmann
- Department of Diagnostic and Interventional Neuroradiology, University Hospital RWTH Aachen, Pauwelsstraße 30, 52074 Aachen, Germany
| | - Thorsten Sichtermann
- Department of Diagnostic and Interventional Neuroradiology, University Hospital RWTH Aachen, Pauwelsstraße 30, 52074 Aachen, Germany
| | - Ana-Maria Oros-Peusquens
- Institute of Neurosciences and Medicine 4 (INM-4), Forschungszentrum Jülich, 52425 Jülich, Germany
| | - Sven Dekeyzer
- Department of Radiology, Antwerp University Hospital, Drie Eikenstraat 655, 2650 Edegem, Belgium
| | - Nadim J Shah
- Institute of Neurosciences and Medicine 4 (INM-4), Forschungszentrum Jülich, 52425 Jülich, Germany
| | - Martin Wiesmann
- Department of Diagnostic and Interventional Neuroradiology, University Hospital RWTH Aachen, Pauwelsstraße 30, 52074 Aachen, Germany
| | - Omid Nikoubashman
- Department of Diagnostic and Interventional Neuroradiology, University Hospital RWTH Aachen, Pauwelsstraße 30, 52074 Aachen, Germany
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27
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Landolfo M, Bragagni A, Borghi C. Not all that trembles…: a rare case of extrapyramidal impairment. Intern Emerg Med 2021; 16:729-733. [PMID: 31848996 DOI: 10.1007/s11739-019-02255-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/11/2019] [Accepted: 12/04/2019] [Indexed: 10/25/2022]
Affiliation(s)
- Matteo Landolfo
- Internal Medicine, Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy.
| | - Alessio Bragagni
- Internal Medicine, Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
| | - Claudio Borghi
- Internal Medicine, Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
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28
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Nasir S, Abou Areda M, Ma EL, Chow RD, Verceles A, Peng CCH. Non-alcoholic Wernicke's encephalopathy: toxic ingestion or an honest mis-steak? J Community Hosp Intern Med Perspect 2021; 11:147-151. [PMID: 33552440 PMCID: PMC7850391 DOI: 10.1080/20009666.2020.1843236] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/04/2022] Open
Abstract
A 21-year-old male presented with a 2-week history of nausea and non-bloody, non-bilious vomiting, accompanied by diffuse chronic myalgia. The patient endorsed headaches, dizziness, and diplopia that had started one day prior to admission. The patient had consumed a meat-only diet for the prior year. The patient was found to have a high anion gap metabolic acidosis with a superimposed normal anion gap metabolic acidosis in the setting of a several-month history of ingesting multiple naturopathic substances as well as recent use of disulfiram for management of his chronic myalgia. Magnetic resonance imaging (MRI) of the brain demonstrated symmetric hyperintensity involving bilateral thalami, periventricular regions, putamina, pons and medulla, with sparing of the mammillary bodies, consistent with Wernicke’s encephalopathy (WE). The patient was treated with intravenous thiamine, a balanced nutritional diet, and hydration. Over the ensuing four days, his metabolic derangements resolved and a repeat MRI demonstrated significantly decreased FLAIR signal abnormality.
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Affiliation(s)
- Salahuddin Nasir
- American University of Antigua, College of Medicine, Osbourn, Antigua and Barbuda
| | - Moustafa Abou Areda
- Department of Medicine, University of Maryland Medical CenterMidtown Campus, Baltimore, Maryland, USA
| | - Elise L Ma
- Department of Medicine, University of Maryland Medical CenterMidtown Campus, Baltimore, Maryland, USA
| | - Robert D Chow
- Department of Medicine, University of Maryland Medical CenterMidtown Campus, Baltimore, Maryland, USA
| | - Avelino Verceles
- Division of Pulmonary, Critical Care, and Sleep Medicine, University of Maryland School of Medicine, Baltimore, Maryland, USA
| | - Carol Chiung-Hui Peng
- Department of Medicine, University of Maryland Medical CenterMidtown Campus, Baltimore, Maryland, USA
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29
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Eren OE, Schöberl F, Campana M, Habs M, Conrad J. A unique MRI-pattern in alcohol-associated Wernicke encephalopathy. Acta Neurol Belg 2020; 120:1439-1441. [PMID: 32779121 PMCID: PMC7641934 DOI: 10.1007/s13760-020-01463-7] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2020] [Accepted: 07/31/2020] [Indexed: 11/24/2022]
Affiliation(s)
- Ozan E Eren
- Department of Neurology, University Hospital, LMU, Marchioninistr. 15, Munich, 81377, Germany.
| | - Florian Schöberl
- Department of Neurology, University Hospital, LMU, Marchioninistr. 15, Munich, 81377, Germany
| | - Mattia Campana
- Department of Psychiatry, University Hospital, LMU, Nußbaumstraße 7, Munich, 80336, Germany
| | - Maximilian Habs
- Department of Neurology, University Hospital, LMU, Marchioninistr. 15, Munich, 81377, Germany
| | - Julian Conrad
- Department of Neurology, University Hospital, LMU, Marchioninistr. 15, Munich, 81377, Germany
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30
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Bouchal S, Bougtoub N, Alami B, Chtaou N, Maaroufi M, Belahsen F. [Gayet-Wernicke encephalopathy: clinical features and radiological anomalies]. Pan Afr Med J 2020; 36:259. [PMID: 33014255 PMCID: PMC7519788 DOI: 10.11604/pamj.2020.36.259.14410] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2017] [Accepted: 11/14/2019] [Indexed: 11/11/2022] Open
Abstract
Gayet Wernicke encephalopathy (EGW) is a neurological emergency secondary to thiamine deficiency (vitamin B1). This is more often secondary to chronic alcoholism. The purpose of this study is to remind clinicians of some clinical signs different from ethylism, suggesting EGW as well as to report four cases characterized by different types of anomalies shown by MRI. The average age of patients was 40 years (2 females 2 males). The neurological picture was characterized by disorders of vigilance in all patients, oculomotor disorders in 2 cases, and cerebellar ataxia in one patient. Chronic vomiting was reported in two cases, prolonged fasting in the first case and alcoholism in the second case. MRI of the brain showed anomalies suggesting EGW in all patients with contrast enhancement in one case. Thiamin deficiency was confirmed in two patients. In our context EGW seems to be more frequent in pathological circumstances other than chronic alcoholism (chronic vomiting, severe malnutrition, severe starvation, and chemotherapy...). The clinical signs can suggest other pathologies such as cerebral venous thrombosis, stroke, or other metabolic disorders but MRI excluded them and allowed the diagnosis of EGW. MRI of the brain has an essential role in the diagnosis of EGW. Absence or delay in treatment may influence the prognosis.
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Affiliation(s)
- Siham Bouchal
- Service de Neurologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Naoual Bougtoub
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Badr Alami
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Naima Chtaou
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Mustafa Maaroufi
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Faouzi Belahsen
- Service de Neurologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
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31
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Ota Y, Capizzano AA, Moritani T, Naganawa S, Kurokawa R, Srinivasan A. Comprehensive review of Wernicke encephalopathy: pathophysiology, clinical symptoms and imaging findings. Jpn J Radiol 2020; 38:809-820. [PMID: 32390125 DOI: 10.1007/s11604-020-00989-3] [Citation(s) in RCA: 68] [Impact Index Per Article: 13.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2020] [Accepted: 04/29/2020] [Indexed: 12/14/2022]
Abstract
Wernicke's encephalopathy (WE) is a severe and life-threatening illness resulting from vitamin B1 (thiamine) deficiency. The prevalence of WE has been estimated from 0.4 to 2.8%. If not treated properly, severe neurologic disorders such as Korsakoff psychosis and even death may occur. The classical triad of clinical symptoms (abnormal mental state, ataxia, and ophthalmoplegia) is found in only 16-33% of patients on initial examination. The originally described underlying condition of WE is alcoholism, but it accounts for about 50% of causes of WE. Nonalcoholic patients are also affected by WE and likely to present symptoms and radiological imaging findings different from patients with alcoholism, which further complicates the diagnosis of WE. Being familiar with predisposing causes, symptoms and radiological imaging findings of WE is important for radiologists and clinicians when making the diagnosis to start immediate treatment. This review discusses pathophysiologies, underlying causes, clinical symptoms, imaging findings and their mimics.
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Affiliation(s)
- Yoshiaki Ota
- The Division of Neuroradiology, Department of Radiology, University of Michigan, 1500 E Medical Center Dr, UH B2, Ann Arbor, MI, 48109, USA. .,Department of Radiology, Kyoto Prefectural University of Medicine, Kawaramachi-Hirokoji, 465 Kajii-cho, Kyoto, Kyoto, 602-8566, Japan.
| | - Aristides A Capizzano
- The Division of Neuroradiology, Department of Radiology, University of Michigan, 1500 E Medical Center Dr, UH B2, Ann Arbor, MI, 48109, USA
| | - Toshio Moritani
- The Division of Neuroradiology, Department of Radiology, University of Michigan, 1500 E Medical Center Dr, UH B2, Ann Arbor, MI, 48109, USA
| | - Shotaro Naganawa
- The Division of Neuroradiology, Department of Radiology, University of Michigan, 1500 E Medical Center Dr, UH B2, Ann Arbor, MI, 48109, USA
| | - Ryo Kurokawa
- Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan
| | - Ashok Srinivasan
- The Division of Neuroradiology, Department of Radiology, University of Michigan, 1500 E Medical Center Dr, UH B2, Ann Arbor, MI, 48109, USA
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Sciacca S, Lynch J, Davagnanam I, Barker R. Midbrain, Pons, and Medulla: Anatomy and Syndromes. Radiographics 2020; 39:1110-1125. [PMID: 31283463 DOI: 10.1148/rg.2019180126] [Citation(s) in RCA: 41] [Impact Index Per Article: 8.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
The anatomy of the brainstem is complex. It contains numerous cranial nerve nuclei and is traversed by multiple tracts between the brain and spinal cord. Improved MRI resolution now allows the radiologist to identify a higher level of anatomic detail, but an understanding of functional anatomy is crucial for correct interpretation of disease. Brainstem syndromes are most commonly due to occlusion of the posterior circulation or mass effect from intrinsic space-occupying lesions. These syndromes can have subtle imaging findings that may be missed by a radiologist unfamiliar with the anatomy or typical manifesting features. This article presents the developmental anatomy of the brainstem and discusses associated pathologic syndromes. Congenital and acquired syndromes are described and correlated with anatomic locations at imaging, with diagrams to provide a reference to aid in radiologic interpretation. ©RSNA, 2019.
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Affiliation(s)
- Sara Sciacca
- From the Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, London WC1N 3BG, England (S.S., J.L., I.D.); and Department of Radiology, Frimley Health NHS Foundation Trust, Frimley, England (R.B.)
| | - Jeremy Lynch
- From the Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, London WC1N 3BG, England (S.S., J.L., I.D.); and Department of Radiology, Frimley Health NHS Foundation Trust, Frimley, England (R.B.)
| | - Indran Davagnanam
- From the Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, London WC1N 3BG, England (S.S., J.L., I.D.); and Department of Radiology, Frimley Health NHS Foundation Trust, Frimley, England (R.B.)
| | - Robert Barker
- From the Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, London WC1N 3BG, England (S.S., J.L., I.D.); and Department of Radiology, Frimley Health NHS Foundation Trust, Frimley, England (R.B.)
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Hou W, Yiin RSZ, Goh CK. Metronidazole induced encephalopathy: case report and discussion on the differential diagnoses, in particular, Wernicke's encephalopathy. J Radiol Case Rep 2020; 13:1-7. [PMID: 32184926 DOI: 10.3941/jrcr.v13i9.3739] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
Metronidazole induced encephalopathy is a rare central nervous system toxicity, which may be completely reversible with prompt cessation of metronidazole usage. We present a case of metronidazole induced encephalopathy in a 59-year-old man with a history of Whipple's procedure for pancreatic neuroendocrine tumour. The characteristic magnetic resonance imaging features of metronidazole induced encephalopathy and its main differential diagnosis, Wernicke's encephalopathy, are discussed.
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Affiliation(s)
- Wenlu Hou
- Department of Radiology, Changi General Hospital, Singapore
| | | | - Chin Kong Goh
- Department of Radiology, Changi General Hospital, Singapore
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34
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Villacieros-Álvarez J, Chicharro P, Trillo S, Barbosa A. Encefalopatía de Wernicke-escorbuto, ¿una asociación infradiagnosticada? Neurologia 2020; 35:47-49. [DOI: 10.1016/j.nrl.2017.05.011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/30/2017] [Revised: 05/04/2017] [Accepted: 05/11/2017] [Indexed: 10/19/2022] Open
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35
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Villacieros-Álvarez J, Chicharro P, Trillo S, Barbosa A. Scurvy and Wernicke's encephalopathy: An underdiagnosed association? NEUROLOGÍA (ENGLISH EDITION) 2020. [DOI: 10.1016/j.nrleng.2019.02.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022] Open
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36
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Recent Advances in the Treatment of Cerebellar Disorders. Brain Sci 2019; 10:brainsci10010011. [PMID: 31878024 PMCID: PMC7017280 DOI: 10.3390/brainsci10010011] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2019] [Revised: 12/13/2019] [Accepted: 12/20/2019] [Indexed: 12/19/2022] Open
Abstract
Various etiopathologies affect the cerebellum, resulting in the development of cerebellar ataxias (CAs), a heterogeneous group of disorders characterized clinically by movement incoordination, affective dysregulation, and cognitive dysmetria. Recent progress in clinical and basic research has opened the door of the ‘‘era of therapy” of CAs. The therapeutic rationale of cerebellar diseases takes into account the capacity of the cerebellum to compensate for pathology and restoration, which is collectively termed cerebellar reserve. In general, treatments of CAs are classified into two categories: cause-cure treatments, aimed at arresting disease progression, and neuromodulation therapies, aimed at potentiating cerebellar reserve. Both forms of therapies should be introduced as soon as possible, at a time where cerebellar reserve is still preserved. Clinical studies have established evidence-based cause-cure treatments for metabolic and immune-mediated CAs. Elaborate protocols of rehabilitation and non-invasive cerebellar stimulation facilitate cerebellar reserve, leading to recovery in the case of controllable pathologies (metabolic and immune-mediated CAs) and delay of disease progression in the case of uncontrollable pathologies (degenerative CAs). Furthermore, recent advances in molecular biology have encouraged the development of new forms of therapies: the molecular targeting therapy, which manipulates impaired RNA or proteins, and the neurotransplantation therapy, which delays cell degeneration and facilitates compensatory functions. The present review focuses on the therapeutic rationales of these recently developed therapeutic modalities, highlighting the underlying pathogenesis.
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37
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Zhu S, Qiang J, Xia Q, Wang Y, Zhang J, Liu X. Hypothalamic sydrome as an initial presentation of Wernicke encephalopathy: A case report. Medicine (Baltimore) 2019; 98:e16181. [PMID: 31261554 PMCID: PMC6617480 DOI: 10.1097/md.0000000000016181] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/11/2019] [Revised: 04/28/2019] [Accepted: 06/03/2019] [Indexed: 01/18/2023] Open
Abstract
RATIONALE Wernicke encephalopathy (WE) is a syndrome characterized by an acute or subacute onset of ataxia, ophthalmoplegia, and mental status changes. To our knowledge, hypothalamic syndrome is rare in WE. PATIENT CONCERNS A 73-year-old female patient with acute cerebral infarct, who showed initial symptoms of vomiting, nausea, ataxia, and subsequent anorexia, was treated with parenteral nutritional supplement for 20 days. Nevertheless, the patient still developed refractory hyponatremia despite the appropriate sodium supplement given for a week following parenteral nutritional supplement. In fact, after 14 days of parenteral nutritional supplement, the patient gradually showed hypotension and apathy. Hyponatremia, hypotension, anorexia and apathy were signs of hypothalamic syndrome. DIAGNOSES Finally, the patient was diagnosed as WE by head magnetic resonance imaging, which showed symmetrical lesions in T2-weighted imaging images and FLAIR high signal intensity in the periaqueduct, hypothalamus, thalamus, mammiliary bodies, medulla oblongata, and vermis cerebelli. INTERVENTIONS The patient was given thiamine supplementation. OUTCOMES The patient regained consciousness within 3 days. The sings of hyponatremia, hypotension, and apathy were relieved subsequently. LESSONS When patients develop unexplained hypothalamic syndrome, we should think of the possibility of WE. The concomitant presence of hyponatremia, hypotension, anorexia, and apathy in WE is rare. Therefore, this case is reported here for discussion.
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Affiliation(s)
- Sha Zhu
- Department of Neurology, Peking University International Hospital
| | - Jun Qiang
- Department of Neurology, Peking University International Hospital
| | - Qing Xia
- Department of Neurology, Peking University International Hospital
| | - Yanshu Wang
- Department of Neurology, Peking University International Hospital
| | - Jun Zhang
- Department of Neurology, Peking University People's Hospital, Beijing, China
| | - Xianzeng Liu
- Department of Neurology, Peking University International Hospital
- Department of Neurology, Peking University People's Hospital, Beijing, China
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38
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Eraslan C, Güler A, Kurt E, Çallı C, Kitiş Ö. Non-alkolik nedenlere bağlı gelişen Wernicke ensefalopatisinde manyetik rezonans görüntüleme bulguları. EGE TIP DERGISI 2019. [DOI: 10.19161/etd.418147] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
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39
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Tan TXZ, Lim KC, Chan Chung C, Aung T. Starvation-induced diplopia and weakness: a case of beriberi and Wernicke's encephalopathy. BMJ Case Rep 2019; 12:12/1/e227412. [PMID: 30610031 DOI: 10.1136/bcr-2018-227412] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
A 56-year-old teetotaller man with hypertension and gout presented with a week duration of painless worsening diplopia on a background of loss of weight and appetite, generalised lethargy and weakness for 1 year. On examination, he was noted to be hypothermic and tachycardic with generalised muscle wasting. Proximal myopathy, lower limb fasciculations and areflexia, restricted bilateral eye abduction and nystagmus were observed. Blood investigations demonstrated compensated lactic acidosis, acute kidney injury and leucocytosis. A nerve conduction study showed severe length-dependent axonal sensorimotor polyneuropathy. This was a diagnostic dilemma until an MRI brain revealed symmetrical signal abnormality and enhancement in the periaqueductal area indicative of Wernicke's encephalopathy, caused by thiamine deficiency from poor nutrition. Beriberi, also caused by thiamine deficiency, accounted for his tachycardia, polyneuropathy, areflexia, hypothermia and biochemical abnormalities. Both beriberi and Wernicke's encephalopathy are medical emergencies, which were treated with intravenous thiamine to good effect.
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Affiliation(s)
| | - Kheng Choon Lim
- Department of Radiology, Singapore General Hospital, Singapore
| | | | - Than Aung
- Department of Internal Medicine, Singapore General Hospital, Singapore
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40
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Hsu CCT, Krings T. Acquired Metabolic Diseases. Clin Neuroradiol 2019. [DOI: 10.1007/978-3-319-61423-6_69-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
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41
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Acquired Metabolic Diseases. Clin Neuroradiol 2019. [DOI: 10.1007/978-3-319-68536-6_69] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
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42
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Paul AB. Wernicke Encephalopathy. Neuroradiology 2019. [DOI: 10.1016/b978-0-323-44549-8.00005-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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43
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Ramineni KK, Marupaka SK, Jakkani R, Ingle A. Wernicke Encephalopathy with Atypical Findings on Magnetic Resonance Imaging. Ann Indian Acad Neurol 2018; 21:328-330. [PMID: 30532369 PMCID: PMC6238578 DOI: 10.4103/aian.aian_65_18] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Affiliation(s)
- Kiran Kumar Ramineni
- Department of Neurology, Yashoda Superspeciality Hospitals, Hyderabad, Telangana, India
| | - Sravan Kumar Marupaka
- Department of Radiology, Yashoda Superspeciality Hospitals, Hyderabad, Telangana, India
| | - Ravikanth Jakkani
- Department of Radiology, Yashoda Superspeciality Hospitals, Hyderabad, Telangana, India
| | - Abhijeet Ingle
- Department of Pathology, Yashoda Superspeciality Hospitals, Hyderabad, Telangana, India
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44
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Isolated Mammillary Bodies Damage-An Atypical Presentation of Wernicke Syndrome. Behav Sci (Basel) 2018; 8:bs8110104. [PMID: 30424509 PMCID: PMC6262580 DOI: 10.3390/bs8110104] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/10/2018] [Revised: 11/02/2018] [Accepted: 11/09/2018] [Indexed: 12/27/2022] Open
Abstract
We report atypical magnetic resonance imaging (MRI) lesions in a case of Wernicke encephalopathy. The patient presented with isolated anterograde amnesia following a partial colectomy complicated by peritonitis. Fluid-attenuated inversion recovery and T2 MRI sequences were normal. However, bilateral contrast enhancement of mammillary bodies was shown on T1 gadolinium-enhanced sequences. Blood tests revealed thiamine deficiency. The diagnosis of Wernicke encephalopathy was made and thiamine supplementation was given, resulting in complete recovery of the memory functions.
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45
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Abstract
The nervous system is vulnerable to intrinsic and extrinsic metabolic perturbations. In particular, the cerebellum, with its large Purkinje cells and its high density of neurons and glial cells, has high metabolic demand and is highly vulnerable to metabolic derangements. As a result, many disorders of intermediary metabolism will preferentially and sometimes selectively target the cerebellum. However, many of these disorders present in a multisystem fashion with ataxia being a part of the neurologic symptom complex. The presentation of these disorders depends on the time of onset and type of metabolic derangement. Early infantile or intrauterine-onset diseases will present in a young child typically with global hypotonia and both nystagmus and ataxia become more apparent later in life, while later-onset diseases usually present primarily with ataxia. It is important to note that the majority of these disorders are progressive if they are untreated. This chapter provides a review of acquired and genetic metabolic disorders that target the cerebellum, and discusses their diagnostic evaluation and therapy.
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Affiliation(s)
- Fatima Y Ismail
- Department of Neurology and Developmental Medicine, Kennedy Krieger Institute, Johns Hopkins University, Baltimore, MD, United States; College of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United Arab Emirates
| | - Hiroshi Mitoma
- Department of Medical Education, Tokyo Medical University, Tokyo, Japan
| | - Ali Fatemi
- Department of Neurology and Developmental Medicine, Kennedy Krieger Institute, Johns Hopkins University, Baltimore, MD, United States.
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46
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Mackay CJ, Tran VT, Chen Y. Optic Chiasm Involvement With Concurrent Typical Wernicke Encephalopathy Magnetic Resonance Findings: A Case Report. Curr Probl Diagn Radiol 2017; 47:452-455. [PMID: 29029866 DOI: 10.1067/j.cpradiol.2017.09.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2017] [Accepted: 09/05/2017] [Indexed: 11/22/2022]
Abstract
The variable clinical presentation of Wernicke encephalopathy often complicates interpretation. Prompt and accurate diagnosis relies on a constellation of typical and atypical magnetic resonance imaging (MRI) findings, which are not always simultaneously present. Our case demonstrates concurrent presentation of all typical Wernicke encephalopathy MRI findings with additional signal abnormalities involving the optic chiasm and optic tract. We suggest that optic pathway involvement may be considered among several atypical MRI manifestations, reinforcing the prompt diagnosis of the potentially life-threatening encephalopathy.
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Affiliation(s)
- Chad J Mackay
- Department of Radiology, St. Joseph's Hospital, Medical College of Wisconsin Affiliated Hospitals, Milwaukee, WI.
| | - Vu T Tran
- Department of Radiology, St. Joseph's Hospital, Medical College of Wisconsin Affiliated Hospitals, Milwaukee, WI
| | - Yao Chen
- Department of Radiology, St. Joseph's Hospital, Medical College of Wisconsin Affiliated Hospitals, Milwaukee, WI
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47
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Bülbül M, Kaplanoğlu M, Arslan Yıldırım E, Yılmaz B. Hiperemezis Gravidarum. ARŞIV KAYNAK TARAMA DERGISI 2017. [DOI: 10.17827/aktd.303579] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/03/2022]
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Verma R, Garg V. Wernicke’s encephalopathy associated with liver abscess. BMJ Case Rep 2017; 2017:bcr-2017-219905. [PMID: 28765488 DOI: 10.1136/bcr-2017-219905] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Wernicke's encephalopathy is a rare neurological disorder caused by thiamine deficiency, characterised by ocular motor dysfunction, ataxia and impairment in consciousness. It predominantly affects brain regions with a high metabolic rate such as mammillary bodies, medial thalamic nuclei, the tectal region and the cerebellum. Although chronic alcoholism is the most common cause of Wernicke's encephalopathy, various other conditions not related to alcohol consumption such as bariatric surgery, acute pancreatitis, hyperemesis gravidarum, prolonged fasting and gastrointestinal surgery have been implicated in its aetiology. We report the case of a patient who underwent surgery for liver abscess and subsequently developed Wernicke's encephalopathy; he showed a positive response to thiamine supplementation. This is the first report describing liver abscess as the cause of Wernicke's encephalopathy.
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Affiliation(s)
- Rajesh Verma
- Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India
| | - Vipul Garg
- King George's Medical University, Lucknow, Uttar Pradesh, India
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Planas-Ballvé A, Grau-López L, Morillas RM, Planas R. Neurological manifestations of excessive alcohol consumption. GASTROENTEROLOGIA Y HEPATOLOGIA 2017; 40:709-717. [PMID: 28651796 DOI: 10.1016/j.gastrohep.2017.05.011] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/05/2017] [Revised: 05/10/2017] [Accepted: 05/19/2017] [Indexed: 12/28/2022]
Abstract
This article reviews the different acute and chronic neurological manifestations of excessive alcohol consumption that affect the central or peripheral nervous system. Several mechanisms can be implicated depending on the disorder, ranging from nutritional factors, alcohol-related toxicity, metabolic changes and immune-mediated mechanisms. Recognition and early treatment of these manifestations is essential given their association with high morbidity and significantly increased mortality.
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Affiliation(s)
- Anna Planas-Ballvé
- Unidad de Neurociencias, Servicio de Neurología, Hospital Germans Trias i Pujol, Badalona (Barcelona), España.
| | - Laia Grau-López
- Unidad de Neurociencias, Servicio de Neurología, Hospital Germans Trias i Pujol, Badalona (Barcelona), España
| | - Rosa María Morillas
- Unidad de Hepatología, CIBERehd, Servicio de Aparato Digestivo, Hospital Germans Trias i Pujol, Badalona (Barcelona), España
| | - Ramón Planas
- Unidad de Hepatología, CIBERehd, Servicio de Aparato Digestivo, Hospital Germans Trias i Pujol, Badalona (Barcelona), España
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Nishimoto A, Usery J, Winton JC, Twilla J. High-dose Parenteral Thiamine in Treatment of Wernicke's Encephalopathy: Case Series and Review of the Literature. ACTA ACUST UNITED AC 2017; 31:121-124. [PMID: 28064230 DOI: 10.21873/invivo.11034] [Citation(s) in RCA: 45] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/19/2016] [Revised: 11/15/2016] [Accepted: 12/13/2016] [Indexed: 12/14/2022]
Abstract
BACKGROUND Thiamine deficiency can lead to Wernicke's encephalopathy (WE), an acute and potentially life-threatening neurological disorder. Even though the main treatment modality for WE consists of thiamine replacement, evidence supporting an optimal dosing strategy and duration is unclear. PATIENTS AND METHODS We present a single-center case series of eleven patients that were admitted with possible WE and treated with high-dose parenteral thiamine. RESULTS Patients with suspected WE were treated with ≥500 mg intravenous thiamine for a median of 3 days with 73% of patients (eight out of eleven) displaying symptom resolution or improvement after treatment. No significant correlation between symptom resolution and timing of high-dose thiamine initiation (median=92 h) was identified. In patients whose symptoms resolved compared to those whose symptoms did not, there were no differences in patient variables nor adverse effects related to thiamine treatment. CONCLUSION High-dose thiamine (≥500 mg) appears safe and efficacious for use in patients with suspected WE.
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Affiliation(s)
- Andrew Nishimoto
- Department of Pharmacy, Methodist University Hospital, Memphis, TN, U.S.A.,Department of Clinical Pharmacy, University of Tennessee, Memphis, TN, U.S.A
| | - Justin Usery
- Department of Pharmacy, Methodist University Hospital, Memphis, TN, U.S.A.,Department of Clinical Pharmacy, University of Tennessee, Memphis, TN, U.S.A
| | - John C Winton
- Inpatient Physicians, Methodist Germantown Hospital, Memphis, TN, U.S.A
| | - Jennifer Twilla
- Department of Pharmacy, Methodist University Hospital, Memphis, TN, U.S.A. .,Department of Clinical Pharmacy, University of Tennessee, Memphis, TN, U.S.A
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