|
1
|
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.
Collapse
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
| |
Collapse
|
|
2
|
Shi Z, Sun Y, Han X. Unmasking the hidden culprit: neurosyphilis mimicking parkinsonism in a middle-aged male. BMC Neurol 2025; 25:96. [PMID: 40055622 PMCID: PMC11887257 DOI: 10.1186/s12883-025-04101-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2024] [Accepted: 02/21/2025] [Indexed: 05/13/2025] Open
Abstract
BACKGROUND General paresis, a tertiary manifestation of neurosyphilis affecting the brain, is characterized by mental and behavioral disorders, such as attention disorder, cognitive impairment, and personality changes. But parkinsonism is rarely reported in patients with neurosyphilis, let alone general paresis. This study reports a case suffering from both general paresis and parkinsonism. CASE PRESENTATION A 50-year-old man was initially misdiagnosed with "alcohol-related psychiatric and behavioral disorders" due to the onset of psychiatric symptoms following alcohol abstinence. The excessive administration of psychotropic medications, attributed to their limited efficacy, was identified as the primary cause of his subsequent extrapyramidal symptoms, including tremor and bradykinesia. However, treatment with levodopa yielded only marginal effectiveness. Following a comprehensive diagnostic evaluation, which encompassed brain magnetic resonance imaging, syphilis screening, and cerebrospinal fluid analysis, neurosyphilis was ultimately identified as the underlying etiology. Subsequent treatment with aqueous penicillin resulted in a marked improvement in his symptoms. CONCLUSION This case illustrates a rare manifestation of neurosyphilis, specifically parkinsonism. The diagnostic process was complicated by several confounding factors. As neurosyphilis is known as the "great imitator," capable of mimicking various neuropsychiatric disorders, routine syphilis screening is imperative for patients presenting with mental disorders and parkinsonian symptoms to facilitate early diagnosis and enhance prognosis.
Collapse
Affiliation(s)
- Zhaobo Shi
- Department of Neurology, Kaifeng Central Hospital, Kaifeng, 475000, Henan Province, China
| | - Yong Sun
- Department of Neurology, Kaifeng Central Hospital, Kaifeng, 475000, Henan Province, China
| | - Xinsheng Han
- Department of Neurology, Kaifeng Central Hospital, Kaifeng, 475000, Henan Province, China.
| |
Collapse
|
|
3
|
Abouelbaqua K, Rebahi H, Louhab N, Kissani N, El Adib AR. Wernicke Encephalopathy Related to Hyperemesis Gravidarum: A Retrospective Study of 12 Cases. Case Rep Crit Care 2025; 2025:7607058. [PMID: 39949614 PMCID: PMC11824303 DOI: 10.1155/crcc/7607058] [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: 03/21/2023] [Revised: 10/10/2024] [Accepted: 10/21/2024] [Indexed: 02/16/2025] Open
Abstract
Objective: Wernicke encephalopathy (WE) related to hyperemesis gravidarum (HG) is a devastating neuropsychiatric syndrome that remains frequently undiagnosed in pregnant women. Although many cases have been published, more studies are required to establish guidelines for the early detection and treatment of this condition. Material and Methods: We conducted a retrospective study to analyze the available data concerning 12 cases of WE complicating HG in the Mother and Child Hospital's Obstetric Intensive Care Unit, belonging to Mohamed VI University Hospital of Marrakesh. Results: Twelve out of 76 HG admitted cases developed WE. Pregnant WE patients became depleted after 11 weeks of vomiting at median gestational weeks of 16.2. They had a severe weight loss of more than 5% of their body and had all presented prodromal signs of WE before the actual onset of the clinical triad. WE diagnosis was clinically made based on Caine's operational criteria as they allow early identification of the disease. A good tool that could also aid diagnosis is an encephalic MRI; however, it should not delay treatment with prompt administration of high doses of thiamin of > 500 mg/day. Chronic sequelae in this study occurred in 45.5% and death in one case. Conclusion: In HG, thiamin rapidly depletes which can lead to WE with adverse outcomes for the mother and fetus. Therefore, physicians must be vigilant in detecting early signs of WE to promptly provide a high dose of thiamin with targeted multimodal therapies as this could be lifesaving.
Collapse
Affiliation(s)
- Kaouthar Abouelbaqua
- Anesthesia and Intensive Care Medicine Department, Mohammed VI University Hospital of Marrakech, Marrakech, Morocco
- Laboratory of Childhood, Health & Development, Faculty of Medicine and Pharmacy of Marrakech, Cadi Ayyad University, Marrakech, Morocco
| | - Houssam Rebahi
- Anesthesia and Intensive Care Medicine Department, Mohammed VI University Hospital of Marrakech, Marrakech, Morocco
- Laboratory of Childhood, Health & Development, Faculty of Medicine and Pharmacy of Marrakech, Cadi Ayyad University, Marrakech, Morocco
| | - Nisrine Louhab
- Neurology Department, Mohammed VI University Hospital of Marrakech, Marrakech, Morocco
| | - Najib Kissani
- Neurology Department, Mohammed VI University Hospital of Marrakech, Marrakech, Morocco
| | - Ahmed Rhassane El Adib
- Anesthesia and Intensive Care Medicine Department, Mohammed VI University Hospital of Marrakech, Marrakech, Morocco
- Laboratory of Childhood, Health & Development, Faculty of Medicine and Pharmacy of Marrakech, Cadi Ayyad University, Marrakech, Morocco
| |
Collapse
|
|
4
|
Taguchi S, Nakura T, Doyu M, Saiki H. Cortical Ribboning as a Key MRI Finding in Wernicke's Encephalopathy With Altered Mental Status. Cureus 2025; 17:e79279. [PMID: 40125225 PMCID: PMC11926919 DOI: 10.7759/cureus.79279] [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] [Accepted: 02/19/2025] [Indexed: 03/25/2025] Open
Abstract
Wernicke's encephalopathy (WE) is associated with thiamine (vitamin B1) deficiency and may lead to mental status changes, ophthalmoplegia, and ataxia. While treatment is simple, delayed diagnosis can have serious consequences, making early detection essential. However, the complete triad of symptoms is rarely seen. When mental status changes occur, physical examination may be limited, making WE harder to identify without other characteristic signs. Furthermore, measuring blood thiamine levels is not always immediately possible, adding to the challenge of diagnosis. We encountered a case of WE presenting with mental status changes, where cortical ribboning appeared as a significant MRI finding. A 77-year-old male had experienced slowness of movement for approximately one year and was diagnosed with multiple system atrophy (MSA). He had difficulty eating; thus, he preferred soft rice porridge. He developed acute mental status changes, and a brain MRI revealed prominent cortical ribboning in both frontal cortices along with subtle medial thalami. The patient received intravenous thiamine, leading to dramatic recovery of mental status, and subsequent MRI follow-up showed near-complete resolution of both cortical and thalamic lesions. Cortical ribboning, which played a key role in this patient, is recognized as an atypical MRI finding of thiamine deficiency and is suggested to be associated with altered mental status in WE. However, to the best of our knowledge, no literature clearly lists thiamine deficiency as a differential diagnosis for cortical ribboning in patients with mental status changes. Recognizing this key imaging finding is crucial in differentiating WE in patients with mental status changes. Preventing the potentially fatal outcome of WE is of paramount importance. Patients with dysphagia tend to prefer soft foods, which may result in reduced thiamine intake. Our experience reaffirms the importance of a well-balanced diet in MSA patients with dysphagia.
Collapse
Affiliation(s)
- Soutarou Taguchi
- Parkinson's Disease Advanced Therapy Center, Aichi Medical University Hospital, Nagakute, JPN
- Neurology, Aichi Medical University, Nagakute, JPN
| | - Takahiro Nakura
- Parkinson's Disease Advanced Therapy Center, Aichi Medical University Hospital, Nagakute, JPN
| | - Manabu Doyu
- Neurology, Aichi Medical University, Nagakute, JPN
| | - Hidemoto Saiki
- Parkinson's Disease Advanced Therapy Center, Aichi Medical University Hospital, Nagakute, JPN
| |
Collapse
|
|
5
|
Takai Y, Yamagami A, Iwasa M, Inoue K, Yasumoto R, Ishikawa H, Wakakura M. A Case of Leber's Hereditary Optic Neuropathy With Reversible Symmetric Lesions in the Substantia Nigra. Cureus 2025; 17:e76883. [PMID: 39902000 PMCID: PMC11788512 DOI: 10.7759/cureus.76883] [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] [Accepted: 01/03/2025] [Indexed: 02/05/2025] Open
Abstract
A 34-year-old man with a history of alcoholism experienced progressive vision loss in both eyes over two months. His best corrected visual acuity was 0.1 OD and 0.2 OS, with visual field tests showing central scotoma bilaterally. Fundus examination revealed reddish optic discs with peripapillary telangiectasia in both eyes. Brain MRI showed bilateral high-intensity lesions in the substantia nigra on T2-weighted/Fluid-Attenuated Inversion Recovery (FLAIR) and diffusion-weighted images. Mitochondrial genetic analysis confirmed the m.11778G>A variant. After the patient stopped consuming alcohol and improved his nutrition, the substantia nigra lesions resolved 18 months after initial symptoms. The improvement of lesions following alcohol abstinence implies a possible link between nutritional status and substantia nigra abnormalities, suggesting concurrent alcohol encephalopathy. While substantia nigra lesions can complicate the diagnosis of Leber's hereditary optic neuropathy (LHON), careful assessment of alcohol consumption history and improvement following abstinence is essential for differential diagnosis.
Collapse
Affiliation(s)
| | | | | | - Kenji Inoue
- Opthalmology, Inouye Eye Hospital, Tokyo, JPN
| | - Ryoma Yasumoto
- Clinical Laboratory, Kitazato University Hospital, Kanagawa, JPN
| | | | | |
Collapse
|
|
6
|
Kimura S, Mori K, Mizoguchi T, Kuwashiro T, Sugimori H, Okada Y. A Case of Wernicke's Encephalopathy With Objective Evaluation of Pupil Findings Using a Pupillometer. Cureus 2025; 17:e77034. [PMID: 39917145 PMCID: PMC11798755 DOI: 10.7759/cureus.77034] [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] [Accepted: 01/06/2025] [Indexed: 02/09/2025] Open
Abstract
Wernicke encephalopathy is a nutritionally disabling encephalopathy resulting from thiamine deficiency. The typical triad of symptoms in Wernicke's encephalopathy includes impaired consciousness, ocular motility disturbances, and ataxic gait. Although constricted pupils and diminished light reflexes are recognized features of Wernicke's encephalopathy, few reports have specifically focused on these findings. Here, we highlight the pupil diameter and light reflex abnormalities observed in this case. A 73-year-old man was admitted to our hospital because of dizziness and then referred to our department four days later because of impaired consciousness. He presented with constricted pupils, diminished light reflexes, restricted eye movements, nystagmus, muscle weakness, and ataxia in the trunk and limbs. Magnetic resonance imaging revealed lesions in the cerebellar vermis, brain stem, and bilateral medial thalamus, leading to a diagnosis of Wernicke's encephalopathy. Following thiamine supplementation, his symptoms and magnetic resonance imaging findings improved. His pupil diameter and light reflexes, as assessed with a pupillometer, also improved over time. Tracking pupillary findings using the pupillometer may be useful in determining treatment efficacy in Wernicke's encephalopathy and is reported here.
Collapse
Affiliation(s)
- Shunsuke Kimura
- Department of Cerebrovascular Medicine and Neurology, Clinical Research Institute, National Hospital Organization Kyushu Medical Center, Fukuoka, JPN
| | - Kota Mori
- Department of Cerebrovascular Medicine and Neurology, National Hospital Organization Kyushu Medical Center, Fukuoka, JPN
| | - Tadataka Mizoguchi
- Department of Cerebrovascular Medicine and Neurology, National Hospital Organization Kyushu Medical Center, Fukuoka, JPN
| | - Takahiro Kuwashiro
- Department of Cerebrovascular Medicine and Neurology, National Hospital Organization Kyushu Medical Center, Fukuoka, JPN
| | - Hiroshi Sugimori
- Department of Cerebrovascular Medicine and Neurology, National Hospital Organization Kyushu Medical Center, Fukuoka, JPN
| | - Yasushi Okada
- Department of Cerebrovascular Medicine and Neurology, National Hospital Organization Kyushu Medical Center, Fukuoka, JPN
| |
Collapse
|
|
7
|
Butnariu I, Anghel DN, Ciobanu AM, Cojocaru F, Antonescu-Ghelmez D, Manea MM, Antonescu F. An atypical presentation of Wernicke-Korsakoff encephalopathy mimicking a left hemispheric stroke: case report. Front Psychiatry 2024; 15:1499803. [PMID: 39758439 PMCID: PMC11696281 DOI: 10.3389/fpsyt.2024.1499803] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/21/2024] [Accepted: 11/21/2024] [Indexed: 01/07/2025] Open
Abstract
Background Wernicke-Korsakoff encephalopathy is a metabolic disease caused by vitamin B1 deficiency that predominantly affects alcoholic patients. Its clinical picture is characterized mainly by altered mental status with memory deficits, ophthalmoparesis, and ataxia, although other clinical manifestations may also be present. The current case presents certain clinical difficulties regarding the diagnosis when confronting an atypical presentation of a classical disease in an acute setting when a decision to administer an intravenous thrombolytic agent needs to be made. Case presentation This case involves a young male patient, with a history of chronic alcohol abuse, malnourished, and in poor general health, who presented with right-sided hemiparesis and language disturbance of acute onset, suggesting a left hemispheric stroke. The psychological examination was difficult due to a mix of confusion and aphasia, the latter being challenging to assess as the patient had a dismissive and highly deflective attitude toward the examiner. The initial cerebral computed tomography scan was unremarkable, in line with early imaging in ischemic stroke patients. On subsequent magnetic resonance imaging, lesions were observed in the medullo-pontine tegmentum, around the aqueduct of Sylvius, in the mamillary bodies, in the medial thalami, but also extensive bilateral cortical involvement in the frontal lobes. After receiving intravenous vitamin B1 treatment, the patient made a slow, but full, recovery, after eight weeks of hospitalization, and was subsequently transferred to a psychiatry clinic for treatment of his addiction. Conclusion Atypical Wernicke-Korsakoff encephalopathy can closely mimic stroke, usually akin to a vertebro-basilar lesion. Our case is the first report we are aware of Wernicke-Korsakoff encephalopathy feigning a left hemispheric stroke with aphasia and right hemiparesis. This has implications for the emergency medicine doctor, neurologist and the stroke specialist when considering an emergency differential diagnosis for a patient with an initial normal computed tomography scan, especially in regard to deciding acute therapy.
Collapse
Affiliation(s)
- Ioana Butnariu
- Department of Neurology, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
- Department of Neurology, National Institute of Neurology and Neurovascular Diseases, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
| | - Daniela Nicoleta Anghel
- Department of Neurology, National Institute of Neurology and Neurovascular Diseases, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
| | - Adela Magdalena Ciobanu
- Department of Neurology, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
- Department of Psychiatry, Prof. Dr. Alexandru Obregia Clinical Psychiatry Hospital, Bucharest, Romania
| | - Florentina Cojocaru
- Department of Neurology, National Institute of Neurology and Neurovascular Diseases, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
| | - Dana Antonescu-Ghelmez
- Department of Neurology, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
- Department of Neurology, National Institute of Neurology and Neurovascular Diseases, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
| | - Maria Mirabela Manea
- Department of Neurology, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
- Department of Neurology, National Institute of Neurology and Neurovascular Diseases, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
| | - Florian Antonescu
- Department of Neurology, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
- Department of Neurology, National Institute of Neurology and Neurovascular Diseases, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
| |
Collapse
|
|
8
|
Pohodich AE, Falardeau J. Optic Nerve Enhancement in the Setting of Wernicke Encephalopathy. J Neuroophthalmol 2024; 44:e384-e386. [PMID: 37318884 DOI: 10.1097/wno.0000000000001910] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/17/2023]
Affiliation(s)
- Amy E Pohodich
- Department of Ophthalmology (AEP, JF), Oregon Health and Science University, Portland, Oregon
| | | |
Collapse
|
|
9
|
Ma YY, He XC, Gao Y, Ma TT, Cheng G, Yue CW. Treatment of nasopharyngeal carcinoma and prevention of non-alcoholic Wernicke's disease: A case report and review of literature. World J Clin Cases 2024; 12:5628-5635. [PMID: 39188614 PMCID: PMC11269984 DOI: 10.12998/wjcc.v12.i24.5628] [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: 05/09/2024] [Revised: 06/20/2024] [Accepted: 07/02/2024] [Indexed: 07/11/2024] Open
Abstract
BACKGROUND Wernicke encephalopathy is a neurological disorder caused by thiamine deficiency, commonly seen in alcoholic populations but also involving other circumstances that may lead to thiamine deficiency. The recognition of Wernicke encephalopathy often depends on clinicians' keen ability to detect its typical triad of features; however, most cases do not present with the full constellation of signs, which complicates the timely identification of Wernicke encephalopathy. CASE SUMMARY This case report describes a patient with nasopharyngeal carcinoma who developed abnormal ocular function and ataxia following concurrent chemoradiotherapy, without a history of alcohol abuse. With the aid of radiological examinations, he received a timely diagnosis and treatment; however, his symptoms did not fully resolve during follow-up. CONCLUSION For patients with malignant tumors exhibiting neurological symptoms, clinicians should consider the possibility of Wernicke encephalopathy and provide prophylactic thiamine therapy.
Collapse
Affiliation(s)
- Yu-Yang Ma
- Yan’an Key Laboratory of Microbial Drug Innovation and Transformation, School of Basic Medicine, Yan’an University, Yan’an 716000, Shaanxi Province, China
- Department of Cardiology, Second Division, Shaanxi Provincial People’s Hospital, Xi’an 710000, Shaanxi Province, China
| | - Xiao-Chan He
- Xi’an Honghui Hospital, Xi’an Jiaotong University, Xi’an 710000, Shaanxi Province, China
| | - Yu Gao
- Yan’an Key Laboratory of Microbial Drug Innovation and Transformation, School of Basic Medicine, Yan’an University, Yan’an 716000, Shaanxi Province, China
| | - Tian-Tian Ma
- Department of Cardiology, Second Division, Shaanxi Provincial People’s Hospital, Xi’an 710000, Shaanxi Province, China
| | - Gong Cheng
- Department of Cardiology, Second Division, Shaanxi Provincial People’s Hospital, Xi’an 710000, Shaanxi Province, China
| | - Chang-Wu Yue
- Yan’an Key Laboratory of Microbial Drug Innovation and Transformation, School of Basic Medicine, Yan’an University, Yan’an 716000, Shaanxi Province, China
| |
Collapse
|
|
10
|
Failla G, Tiralongo F, Crimi P, Lo Fermo S, Foti PV, David E, Ini C, Palermo M, Falsaperla D, Palmucci S, Basile A. Beyond the bowel: Wernicke's encephalopathy as a neurological complication of Crohn's disease: A case report. Radiol Case Rep 2024; 19:3013-3018. [PMID: 38741688 PMCID: PMC11089286 DOI: 10.1016/j.radcr.2024.03.088] [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: 03/10/2024] [Revised: 03/27/2024] [Accepted: 03/29/2024] [Indexed: 05/16/2024] Open
Abstract
Wernicke encephalopathy (WE) is a rare but severe neurological syndrome characterized, in its classic form, by the acute onset of ocular disturbances, ataxia, and cognitive impairment. It is caused by a deficiency of thiamine (vitamin B1) and mainly affects chronic alcoholics, although it can also affect patients with pathologies that lead to malnutrition. We present a case of a 58-year-old woman, who presented with significant weight loss over the past 6 months and who came to the emergency department for episodes of repetitive vomiting and a sleepy state. The patient underwent blood chemistry tests and a brain CT scan, which revealed symmetrical and bilateral hypodensity of the medial portion of the thalamus, tectal plate, and periaqueductal gray matte, suggestive of WE. She was subsequently referred to the Department of Neurology and underwent a brain MRI, which confirmed the clinical suspicion. She also had an abdominal CT scan and ileo-colonoscopy and was diagnosed with Crohn's disease. Immediately after the clinical diagnosis of WE, a replacement therapy based on intravenous thiamine at high doses was promptly set up, and the patient improved from a clinical point of view. Wernicke encephalopathy can be difficult to diagnose when it occurs in non-alcoholic patients; WE associated with IBD is a rare condition, and it can present with atypical and more subtle symptoms. Radiologists and physicians must be aware of this condition and imaging findings for rapid diagnosis and treatment.
Collapse
Affiliation(s)
- Giovanni Failla
- Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, University of Catania, 95123 Catania, Italy
| | - Francesco Tiralongo
- Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, University of Catania, 95123 Catania, Italy
| | - Pina Crimi
- Neurologic Unit, AOU “Policlinico-San Marco”, Department of Medical, Surgical Sciences and Advanced Technologies, GF Ingrassia, University of Catania, Via Santa Sofia n.78, 95100, Catania, Sicily, Italy
| | - Salvatore Lo Fermo
- Department “GF Ingrassia”, Section of Neurosciences, Neurology Clinic, University of Catania, Operative Unit of Multiple Sclerosis, University-Hospital G. Rodolico - San Marco, Catania, Italy, Catania 9126, Italy
| | - Pietro Valerio Foti
- Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, University of Catania, 95123 Catania, Italy
| | - Emanuele David
- Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, University of Catania, 95123 Catania, Italy
| | - Corrado Ini
- Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, University of Catania, 95123 Catania, Italy
| | - Monica Palermo
- Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, University of Catania, 95123 Catania, Italy
| | - Daniele Falsaperla
- Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, University of Catania, 95123 Catania, Italy
| | - Stefano Palmucci
- UOSD I.P.T.R.A., Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University of Catania, University Hospital Policlinico “G. Rodolico-San Marco”, Catania, Italy
| | - Antonio Basile
- Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”, University of Catania, 95123 Catania, Italy
| |
Collapse
|
|
11
|
Namboodiri S, Rao S. A Case of Prolonged Wernicke's Encephalopathy After Treatment With IV Thiamine Due to the Subsequent Development of Refeeding Syndrome. Cureus 2024; 16:e65178. [PMID: 39176337 PMCID: PMC11341067 DOI: 10.7759/cureus.65178] [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] [Accepted: 07/23/2024] [Indexed: 08/24/2024] Open
Abstract
Wernicke's encephalopathy (WE) is a rare, life-threatening condition in which thiamine deficiency causes dysfunction of the Kreb's cycle, accumulation of lactic acid in the brain tissues, and irreversible cognitive impairment. Prompt treatment with IV thiamine can reverse the process. The classic Wernicke's triad of ataxia, memory issues, and ocular abnormalities is not often present. Caine's criteria, which requires two of the following: dietary deficiencies, ocular abnormalities, altered cognition or mental status, and cerebellar dysfunction, is highly sensitive and specific for Wernicke's diagnosis, especially in patients with alcohol use disorder. Refeeding syndrome (RS) has similar risk factors to WE, including disease states that lead to malnutrition. Patients with RS develop WE due to thiamine depletion that occurs when oral nutrition is reinitiated after a period of poor oral intake. We present a patient with initially undetected WE who developed RS after the initiation of treatment with IV thiamine. RS prolonged the neurologic symptoms of WE and led to an extended hospital stay and significant physical debility. In our patient, WE preceded RS instead of occurring as a consequence of it. The case highlights that if one of these disorders is present, the other may not be far behind. When WE precedes RS, prolonged treatment with IV thiamine may be warranted until the symptoms of both disorders resolve.
Collapse
Affiliation(s)
- Sally Namboodiri
- Medicine, Louis Stokes Cleveland VA Medical Center, Cleveland, USA
| | - Sanjay Rao
- Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, USA
| |
Collapse
|
|
12
|
Tao X, Qiao R, Liu C, Guo L, Li J, Kang Y, Wei Y. Wernicke encephalopathy induced by glucose infusion: A case report and literature review. Heliyon 2024; 10:e29419. [PMID: 38681648 PMCID: PMC11053267 DOI: 10.1016/j.heliyon.2024.e29419] [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: 10/28/2023] [Revised: 03/31/2024] [Accepted: 04/08/2024] [Indexed: 05/01/2024] Open
Abstract
Introduction Wernicke encephalopathy (WE) is a potentially fatal condition caused by thiamine (vitamin B1) deficiency. Chronic alcoholism is the most common cause of WE; however, other conditions responsible for thiamine deficiency should also be considered. Case Report We report the case of a 64-year-old woman with a history of diabetes who presented with confusion and apathy. Magnetic resonance imaging of the brain showed T2 hyperintensities involving dorsolateral medulla oblongata, tegmentum of the pons, vermis of the cerebellum, periaqueductal region, and the bilateral mammillary bodies. She had a history of intravenous glucose administration before her mental symptoms developed. On suspicion of WE, she was treated with a high dose of thiamine empirically. Her clinical condition improved rapidly in 2 weeks. Conclusion Endogenous thiamine stores can be rapidly depleted in the case of enhanced glucose oxidation. Patients who receive glucose should also be prescribed thiamine to avoid inducing or exacerbating WE.
Collapse
Affiliation(s)
- Xiangkun Tao
- Psychologic Medicine Science, Chongqing Medical University, Chongqing, China
| | - Renjie Qiao
- Clinical Medicine Science, Chongqing Medical University, Chongqing, China
| | - Can Liu
- Department of Radiology, Daping Hospital, Army Medical University, Chongqing, 400042, China
| | - Lu Guo
- Department of Neurology, Daping Hospital, Army Medical University, Chongqing, 400042, China
| | - Jingcheng Li
- Department of Neurology, Daping Hospital, Army Medical University, Chongqing, 400042, China
| | - Yulai Kang
- Department of Neurology, Daping Hospital, Army Medical University, Chongqing, 400042, China
| | - Youdong Wei
- Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing Key Laboratory of Neurobiology, Chongqing, China
| |
Collapse
|
|
13
|
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.
Collapse
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
| |
Collapse
|
|
14
|
Gafoor S, Robertson R. Silent Echoes: A Case Report of Wernicke Encephalopathy's Unheard Voice. Cureus 2024; 16:e52151. [PMID: 38347991 PMCID: PMC10859680 DOI: 10.7759/cureus.52151] [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] [Accepted: 01/12/2024] [Indexed: 02/15/2024] Open
Abstract
Wernicke encephalopathy (WE) is an acute neuropsychiatric emergency that is caused by a deficiency in vitamin B1 (thiamine). This condition is most commonly seen in patients with alcohol use disorder; however, patients with other disorders of severe malnourishment are also at increased risk. In severe cases, this disease may be followed by Korsakoff's psychosis and even death. We present a case of a 64-year-old African American female with a history of alcohol use disorder who presented to the emergency department on account of an acute confusional state. Neurological examination revealed right beating nystagmus on the left gaze and a wide-based gait. Initial laboratory work-up was unrevealing; however, magnetic resonance imaging (MRI) of the brain demonstrated an abnormal T2 fluid-attenuated inversion recovery (FLAIR) signal involving the bilateral mammillary bodies and surrounding lateral ventricles that extended into the periaqueductal parenchyma. The patient was admitted to the neurology unit, and high-dose intravenous thiamine was commenced. During hospitalization, the patient's confusion improved and they were subsequently discharged with oral thiamine. The spectrum of severity of WE is wide, ranging from fatal disease and can lead to permanent brain damage or even Korsakoff syndrome, characterized by severe memory loss and confabulation. The diagnosis is mainly clinical and based on the presence of symptoms in the classic triad of mental status change, oculomotor abnormality, and ataxia. This triad is only present in about 10% of cases, making the diagnosis very challenging. Laboratory testing can assist in making the diagnosis, but it is not always reliable or available. In situations of clinical uncertainty, imaging may also be used to support diagnosis or rule out other differentials. The mainstay of treatment is with high-dose parenteral thiamine.
Collapse
Affiliation(s)
- Stefan Gafoor
- Graduate Medical Education, Piedmont Athens Regional, Athens, USA
| | - Raheem Robertson
- Graduate Medical Education, Piedmont Athens Regional, Athens, USA
| |
Collapse
|
|
15
|
Ono K, Hayano S, Kashima M. Wernicke encephalopathy: limitations in a laboratory and radiological diagnosis. BMJ Case Rep 2023; 16:e254786. [PMID: 38081738 PMCID: PMC10729136 DOI: 10.1136/bcr-2023-254786] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2023] Open
Abstract
Wernicke encephalopathy is an emergent neurological disorder caused by vitamin B1 (thiamine) deficiency. Here, we present a case of Wernicke encephalopathy in a male patient in his 70s with normal serum thiamine levels and MRI findings on admission. He had a history of heavy alcohol consumption and a gradual decrease in food intake. On arrival at the hospital, his consciousness was impaired which persisted even after glucose replacement. Moreover, horizontal nystagmus and cerebellar ataxia were observed. Head CT scan and MRI revealed no abnormal findings. Further, his serum thiamine level was within the normal range. The patient was clinically diagnosed with Wernicke encephalopathy, and high-dose thiamine therapy was started. Then, his symptoms improved immediately. Thus, in case of clinical suspicion, treatment for Wernicke encephalopathy must be initiated promptly even in patients with normal serum thiamine levels.
Collapse
Affiliation(s)
- Kohei Ono
- Department of Surgery, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan
| | - Satoshi Hayano
- Department of Internal Medicine, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan
| | - Masayuki Kashima
- Department of Internal Medicine, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan
| |
Collapse
|
|
16
|
Hamid M, Benmoh Y, Bourazza A. Hypoglycemic encephalopathy with extensive brain injuries: A case report. Radiol Case Rep 2023; 18:4495-4498. [PMID: 37868008 PMCID: PMC10589743 DOI: 10.1016/j.radcr.2023.09.035] [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: 06/21/2023] [Revised: 09/11/2023] [Accepted: 09/12/2023] [Indexed: 10/24/2023] Open
Abstract
Hypoglycemia is known as a sudden diminution in blood glucose level <50 mg/dL. Hypoglycemic encephalopathy is a metabolic encephalopathy that is usually observed in patients treated for diabetes or chronic alcoholism. Neurological manifestations may range from transient deficits to prolonged coma, depending on the duration and severity of hypoglycemia. Neuroradiological features of hypoglycemia are variable involving the cerebral white and gray matter regions. Acquired metabolic or toxic conditions can cause hypoglycemia-like damage to the cerebral white matter and basal ganglia. Widespread lesions in the brain parenchyma or basal ganglia have a poor prognosis. In this report, we present a patient with widespread brain damage secondary to profound and prolonged hypoglycemia.
Collapse
Affiliation(s)
- Mohamed Hamid
- Department of Neurology, Mohammed V Military Instruction Hospital, Rabat, Morocco
| | - Youssouf Benmoh
- Department of Neurology, Mohammed V Military Instruction Hospital, Rabat, Morocco
| | - Ahmed Bourazza
- Department of Neurology, Mohammed V Military Instruction Hospital, Rabat, Morocco
| |
Collapse
|
|
17
|
Kitaguchi T, Ota Y, Liao E, Moritani T, Shah G, Yamada K, Srinivasan A. The role of MRI in the prognosis of Wernicke's encephalopathy. J Neuroimaging 2023; 33:917-925. [PMID: 37355834 DOI: 10.1111/jon.13143] [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: 01/29/2023] [Revised: 06/14/2023] [Accepted: 06/16/2023] [Indexed: 06/26/2023] Open
Abstract
BACKGROUND AND PURPOSE Wernicke's encephalopathy (WE) is a severe acute disorder related to thiamine deficiency. This study was aimed at revealing the relationship between clinical and imaging findings and WE recovery. METHODS We retrospectively reviewed 34 cases of WE diagnosed between 2003 and 2020 (median age: 57 years, 14 females) at two academic institutions. WE cases were divided into two groups with symptomatic recovery within 4 weeks (group 1) or later (group 2). The lesion sites were divided into typical and atypical sites (total sites defined as when either typical or atypical sites were involved). Clinical and MRI features were compared between them as appropriate. RESULTS WE patients were divided into group 1 (19 cases, median age: 57 years, 10 females) and group 2 (15 cases, median age: 57 years, four females). Regarding clinical features, only cerebellar ataxia was more often observed in group 1 than in group 2. Regarding MRI features, signal abnormality on T2-weighted image (WI)/fluid-attenuated inversion recovery (FLAIR) was more often observed in atypical sites between groups 1 and 2 (1/19 vs. 7/15; p = .01). There were significant differences between groups 1 and 2 regarding the presence of both vasogenic edema and cytotoxic edema in total sites (4/11 vs. 11/15, p = .005; 1/19 vs. 6/15, p = .03), with a significant difference in the presence of vasogenic edema in typical sites (4/19 vs. 10/15, p = .01). CONCLUSION The early recovered group showed a lower incidence of T2WI/FLAIR abnormality in atypical sites and diffusion signal abnormality in total or typical sites with a lower incidence of cerebellar ataxia.
Collapse
Affiliation(s)
- Tomoaki Kitaguchi
- Department of Radiology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Yoshiaki Ota
- Division of Neuroradiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan, USA
| | - Eric Liao
- Division of Neuroradiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan, USA
| | - Toshio Moritani
- Division of Neuroradiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan, USA
| | - Gaurang Shah
- Division of Neuroradiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan, USA
| | - Kei Yamada
- Department of Radiology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Ashok Srinivasan
- Division of Neuroradiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan, USA
| |
Collapse
|
|
18
|
Yu AT, Gross A, Park K, Harvey EJ. Wernicke Encephalopathy After Bariatric Surgery: a Literature Review. Obes Surg 2023; 33:3621-3627. [PMID: 37798508 DOI: 10.1007/s11695-023-06840-8] [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: 11/02/2022] [Revised: 09/14/2023] [Accepted: 09/24/2023] [Indexed: 10/07/2023]
Abstract
Wernicke encephalopathy (WE) is an acute neurological disorder classically characterized by ataxia, ophthalmoplegia, and altered mental status. This is caused by thiamine deficiency and is usually seen in malnourished populations. However, with the advent and rise of bariatric surgery in the last 50 years, WE has become an increasingly recognized and potentially deadly complication. Here, we review the populations at risk, clinical presentation, and the incidence of WE in the bariatric surgery population from 1985 to 2023. While the predominant procedure shifts throughout the years, the overall incidence of WE per 100,000 cases for the following procedures are sleeve gastrectomy (1.06), gastric band (1.16), RYGB (4.29), and biliopancreatic diversion with duodenal switch (8.92). Thus, early intervention and post-operative supplementation is recommended to prevent WE.
Collapse
Affiliation(s)
- Allen T Yu
- Department of Surgery, Mount Sinai Health System, New York, NY, 10029, USA.
| | - Aliza Gross
- Department of Surgery, Mount Sinai Health System, New York, NY, 10029, USA
| | - Koji Park
- Department of Surgery, Mount Sinai Health System, New York, NY, 10029, USA
| | - Eugenius J Harvey
- Department of Surgery, Mount Sinai Health System, New York, NY, 10029, USA
| |
Collapse
|
|
19
|
Adamou F, Darkaoui D, Koulali H, Ismaili Z, Kharrasse G. Wernicke's Encephalopathy in a Patient With Crohn's Disease: A Case Report. Cureus 2023; 15:e45810. [PMID: 37876382 PMCID: PMC10591271 DOI: 10.7759/cureus.45810] [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] [Accepted: 09/22/2023] [Indexed: 10/26/2023] Open
Abstract
The inflammatory bowel diseases (IBD), including Crohn's disease (CD) and ulcerative colitis (UC), are chronic inflammatory disorders of the intestine. Both are associated with intestinal and extra-intestinal manifestations (EIM). EIM are usually related to intestinal disease activity and may precede or develop concurrently with intestinal symptoms. Although they are well documented, the association of CD with neurological and neuromuscular involvement is rare and controversial, with sporadic and contradictory data regarding its prevalence and spectrum. Neurological involvement can affect the central or peripheral nervous system, with thrombotic events being the most frequent complication. Wernicke's encephalopathy (WE) is one of the neurological complications that occurs in the general population with a clinical prevalence ranging from 0.04% to 0.13%. Although no specific data exists for IBD patients, it is imperative for clinicians to be vigilant and consider the possibility of this condition even with mild neurological symptoms and to administer vitamin B1 promptly before attempting any biological assessment. Timely treatment is essential to avoid irreversible damage or even the death of the patient. We report herein a challenging case of WE in CD and we discuss the literature.
Collapse
Affiliation(s)
- Fatima Adamou
- Hepato-Gastroenterology, Mohammed VI University Hospital, Oujda, MAR
- Digestive Diseases Research Laboratory (DSRL), Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR
| | - Doua Darkaoui
- Hepato-Gastroenterology, Mohammed VI University Hospital, Oujda, MAR
- Digestive Diseases Research Laboratory (DSRL), Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR
| | - Hajar Koulali
- Hepato-Gastroenterology, Mohammed VI University Hospital, Oujda, MAR
- Digestive Diseases Research Laboratory (DSRL), Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR
| | - Zahi Ismaili
- Gastroenterology and Hepatology, Mohammed VI University Hospital Center/Mohammed First University, Oujda, MAR
| | - Ghizlane Kharrasse
- Gastroenterology and Hepatology, Mohammed VI University Hospital Center/Mohammed First University, Oujda, MAR
| |
Collapse
|
|
20
|
Suzuki K, Miyamoto K, Kanai T, Kurihara M, Kikuchi K, Harano K, Kato A, Yagi M, Ohgiya Y, Dohi K. Single-photon emission computed tomography (SPECT) predicted neurological prognosis in heat stroke: A case report. Heliyon 2023; 9:e18285. [PMID: 37539227 PMCID: PMC10393623 DOI: 10.1016/j.heliyon.2023.e18285] [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: 02/27/2023] [Revised: 06/29/2023] [Accepted: 07/13/2023] [Indexed: 08/05/2023] Open
Abstract
Heat stroke may cause multi-organ dysfunction and death. Some patients with neurological abnormalities in the acute phase have neurological sequelae, particularly cerebellar ataxia, in the recovery phase. However, there is no method to predict the neurological prognosis, and the usefulness of imaging has not yet been established. We report the case of an 86-year-old woman with dementia brought to our emergency department in a coma and hyperthermia. The patient was diagnosed with heat stroke and promptly treated in the ICU but remained unconscious. The patient gained consciousness on day 19, but difficulty with stillness associated with cerebellar ataxia in her right upper extremity became apparent. On day 1, head magnetic resonance imaging (MRI) showed no obvious abnormality. However, on day 6, high-signal areas, suggestive of edema, were seen in the bilateral cerebellar hemispheres. Single-photon emission computed tomography (SPECT) on day 9 revealed significant hypoperfusion in the right cerebellum. These changes improved at the time of hospital discharge. This was a case of persistent cerebellar ataxia due to heat stroke, in which imaging findings improved over time. In most cases, MRI findings do not match clinical symptoms. However, the low cerebral blood flow in the early SPECT images was consistent with the clinical symptoms. MRI may not be a prognostic indicator; however, SPECT images may be useful for predicting sequelae.
Collapse
Affiliation(s)
- Keisuke Suzuki
- Department of Emergency and Disaster Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan
- Department of Radiology, Division of Radiology, Showa University School of Medicine, Shinagawa-ku, Tokyo 142-8666, Japan
| | - Kazuyuki Miyamoto
- Department of Emergency and Disaster Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan
| | - Takahiro Kanai
- Department of Radiology, Division of Radiology, Showa University School of Medicine, Shinagawa-ku, Tokyo 142-8666, Japan
| | - Mariko Kurihara
- Department of Emergency and Disaster Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan
| | - Kazuki Kikuchi
- Department of Emergency and Disaster Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan
| | - Kohei Harano
- Department of Emergency and Disaster Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan
| | - Akihito Kato
- Department of Emergency and Disaster Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan
- Department of Emergency and Disaster Medicine, Showa University, Yokohama Northern Hospital, 35-1 Chigasaki Chuo Tsuzuki-ku, Yokohama 224-8503, Japan
| | - Masaharu Yagi
- Department of Emergency and Disaster Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan
| | - Yoshimitsu Ohgiya
- Department of Radiology, Division of Radiology, Showa University School of Medicine, Shinagawa-ku, Tokyo 142-8666, Japan
| | - Kenji Dohi
- Department of Emergency and Disaster Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan
| |
Collapse
|
|
21
|
Bouladi M, Lajmi H, Ben Othmen A, El Fekih L. Uncommon bilateral optic neuropathy in Wernicke's encephalopathy complicating gravidarum hyperemesis. LA TUNISIE MEDICALE 2023; 101:530-532. [PMID: 38372521 PMCID: PMC11361296] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Subscribe] [Scholar Register] [Received: 05/24/2023] [Accepted: 08/15/2023] [Indexed: 02/20/2024]
Abstract
Wernicke encephalopathy (WE) is a rare neurological disorder that results from vitamin B1 (Thiamin) deficiency, classically characterized by the triad of ophtalmoplagia, altered consciousness, and ataxia. WE is often associated with alcoholism, malnutrition, or gastrointestinal diseases with malabsorption. The association of «gravidarum hyperemesis» and WE seems to be underestimated. We report a 24-year-old pregnant woman with hyperemesis gravidarum, who presented with decreased visual acuity of both eyes. Fundus examination showed a bilateral stage 2 papillary edema. brain magnetic resonance imaging (MRI) showed bilateral and symmetrical hyper intense lesions on T2-weighted and FLAIR sequences in periaqueductal gray matter, thalamus, and mammillary bodies, which confirmed WE complicated by bilateral optic neuropathy. Her symptoms resolved after thiamine treatment. This case raises of the possibility of optic neuropathy in WE, which is a diagnostic emergency requiring early treatment to prevent complications.
Collapse
Affiliation(s)
- Mejda Bouladi
- Ophthalmology Department, Mongi Slim University Hospital, La Marsa, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunisia
| | - Houda Lajmi
- Ophtalmology Department, Security Forces Hospital, La Marsa, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunisia
| | - Amal Ben Othmen
- Ophthalmology Department, Mongi Slim University Hospital, La Marsa, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunisia
| | - Lamia El Fekih
- Ophthalmology Department, Mongi Slim University Hospital, La Marsa, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunisia
| |
Collapse
|
|
22
|
Silva AR, Almeida-Xavier S, Lopes M, Soares-Fernandes JP, Sousa F, Varanda S. Is there a time window for MRI in Wernicke encephalopathy - a decade of experience from a tertiary hospital. Neurol Sci 2023; 44:703-708. [PMID: 36335281 DOI: 10.1007/s10072-022-06477-y] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/10/2022] [Accepted: 10/22/2022] [Indexed: 11/06/2022]
Abstract
OBJECTIVE Wernicke encephalopathy (WE) is a neuropsychiatric syndrome caused by thiamine deficiency. Despite its low sensitivity, brain magnetic resonance imaging (MRI) is the most useful diagnostic technique. Our aim was to investigate whether the timing of the imaging study, and thiamine replacement can influence brain MRI findings in these patients. METHODS Retrospective observational study of hospitalized patients between January/2008 and December/2020 with a clinical diagnosis of WE. Data from clinical presentation, diagnostic features, therapeutic approach, and outcomes were collected. RESULTS We identified 41 patients (55 ± 13.3 years) with WE. Brain MRI was performed in 36 patients, and one third had T2/FLAIR hyperintensities suggestive of WE. We found an association between a history of poor diet and periventricular hyperintensities (p = 0.023), especially on the ventral surface of the thalamus and the periaqueductal region. It was found that the odds of having a typical imaging of WE decreased by 5.3% for each additional unit (100 mg) of thiamine administered (p = 0.046) (95% CI [0.89, 0.99]). On the other hand, the number of days from clinical presentation was not found to be a viable predictor (p = 0.254) (95% CI [0.88, 1.03]) Recovery was positively correlated with the total dose of thiamine received until discharge (p = 0.020). CONCLUSIONS MRI hyperintensities seem to be dependent on the timing of thiamine correction and, particularly, on the thiamine dosage prescribed at admission. Nevertheless, thiamine replacement should not be delayed, as its timely prescription is associated with a better prognosis at discharge.
Collapse
Affiliation(s)
- Ana Rita Silva
- Department of Neurology, Hospital de Braga, Braga, Portugal.
| | | | | | | | - Filipa Sousa
- Department of Neurology, Hospital de Braga, Braga, Portugal
| | - Sara Varanda
- Department of Neurology, Hospital de Braga, Braga, Portugal
| |
Collapse
|
|
23
|
Lin Q, Li G, Wang Z, Zhang Y. Case Report: Wernicke's encephalopathy after gastric surgery presenting as lactic acidosis and refractory thrombocytopenia. Front Surg 2023; 10:1016347. [PMID: 36896260 PMCID: PMC9989168 DOI: 10.3389/fsurg.2023.1016347] [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: 08/10/2022] [Accepted: 01/16/2023] [Indexed: 02/23/2023] Open
Abstract
Wernicke's encephalopathy (WE) is a severe neuropsychiatric disorder, mainly resulting from a nutritional deficiency of thiamine. WE is hard to detect at an early stage. Less than 20% of WE can be diagnosed during a patient's lifetime, and WE tends to occur in patients with chronic alcoholism. Therefore, a large proportion of non-alcoholic WE patients are misdiagnosed. Lactate is an important by-product of anaerobic metabolism when the aerobic metabolism is blocked without thiamine, which can potentially serve as an alerting index for WE. Here, we report a case of a patient with WE who suffered gastric outlet obstruction following postoperative fasting, accompanied by lactic acidosis and refractory thrombocytopenia. A 67-year-old non-alcoholic woman who suffered hyperemesis for 2 months was diagnosed with gastric outlet obstruction (GOO). Gastric biopsies with endoscopy revealed gastric cancer, and total gastrectomy, together with D2 nodal dissection, was performed. She developed a coma with refractory thrombocytopenia rapidly after the surgical procedures were performed. The above conditions were treated not by the administration of antibiotics but by that of thiamine. We also found before the start of the procedures that she had a high level of blood lactate for a long period of time. Early diagnosis of WE is important because permanent injury can be caused to the central nervous system. Even today, the diagnosis of WE mainly depends on clinical symptoms, but occasionally, a typical triad occurs among WE patients. Therefore, a sensitive index for early diagnosis is critical for WE. Rising levels of blood lactate as a result of thiamine deficiency can serve as a warning for WE. In addition, we noted that this patient had a non-typical thiamine-sensitive refractory thrombocytopenia.
Collapse
Affiliation(s)
- Qi Lin
- Department of Gastrointestinal Surgery, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
| | - Guanghua Li
- Department of Gastrointestinal Surgery, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
| | - Zhixiong Wang
- Department of Gastrointestinal Surgery, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
| | - Yu Zhang
- Department of Neurology, The First Affiliated Hospital of Jinan University, Guangzhou, China
| |
Collapse
|
|
24
|
Kareem O, Nisar S, Tanvir M, Muzaffer U, Bader GN. Thiamine deficiency in pregnancy and lactation: implications and present perspectives. Front Nutr 2023; 10:1080611. [PMID: 37153911 PMCID: PMC10158844 DOI: 10.3389/fnut.2023.1080611] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2022] [Accepted: 04/03/2023] [Indexed: 05/10/2023] Open
Abstract
During pregnancy, many physiologic changes occur in order to accommodate fetal growth. These changes require an increase in many of the nutritional needs to prevent long-term consequences for both mother and the offspring. One of the main vitamins that are needed throughout the pregnancy is thiamine (vitamin B1) which is a water-soluble vitamin that plays an important role in many metabolic and physiologic processes in the human body. Thiamine deficiency during pregnancy can cause can have many cardiac, neurologic, and psychological effects on the mother. It can also dispose the fetus to gastrointestinal, pulmonological, cardiac, and neurologic conditions. This paper reviews the recently published literature about thiamine and its physiologic roles, thiamine deficiency in pregnancy, its prevalence, its impact on infants and subsequent consequences in them. This review also highlights the knowledge gaps within these topics.
Collapse
Affiliation(s)
- Ozaifa Kareem
- Department of Pharmaceutical Sciences, University of Kashmir, Srinagar, India
- *Correspondence: Ozaifa Kareem, ,
| | - Sobia Nisar
- Department of Medicine, Government Medical College, Srinagar, India
| | - Masood Tanvir
- Department of Medicine, Government Medical College, Srinagar, India
| | - Umar Muzaffer
- Department of Medicine, Government Medical College, Srinagar, India
| | - G. N. Bader
- Department of Pharmaceutical Sciences, University of Kashmir, Srinagar, India
- G. N. Bader,
| |
Collapse
|
|
25
|
Duve KV, Shkrobot SI. THE NEUROLOGICAL MANIFESTATIONS AND FUNCTIONAL INDEPENDENCE IN PATIENTS WITH ENCEPHALOPATHIES OF DIFFERENT TYPES. POLSKI MERKURIUSZ LEKARSKI : ORGAN POLSKIEGO TOWARZYSTWA LEKARSKIEGO 2023; 51:489-495. [PMID: 38069849 DOI: 10.36740/merkur202305107] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/18/2023]
Abstract
OBJECTIVE Aim: To access the neurological manifestations and activities of daily living in patients with encephalopathy of one of the following types: post-infectious, chronic traumatic encephalopathy, alcohol-induced, and microvascular ischemic disease of the brain. PATIENTS AND METHODS Materials and Methods: In the period of 2021-2022 we examined 520 patients, who signed the informed consent, taking into account their age, sex, occupation, the cause, and the disease duration. Such parameters were evaluated, as the data of neurological manifestations, the activities of daily living (Barthel index), cognitive functioning (MoCA-test), and statistical methods (Statistica 13.0). RESULTS Results: A probable influence of the age factor on the frequency of occurrence of different types of encephalopathies was established (χ2=235.05; p<0.001). The cognitive impairment was diagnosed in 53.79 % of patients with CTE, 66.21% with SVD, and 58.82% with AE. 40% of patients with CTE are dependent on their activities of daily living, among patients with SVD - 31,72 %, among patients with AE - 44.12%, among patients with PIE - 53.91%. 17.97% of patients with PIE had moderate dependence by the Barthel index. Thus, the severity of disability doesn't depend on the age or sex of patients but is correlating with the duration of the disease. CONCLUSION Conclusions: The neurological manifestations in patients with encephalopathies and their activities of daily living were studied profoundly and the data obtained opened new directions in the following research.
Collapse
Affiliation(s)
- Khrystyna V Duve
- I. HORBACHEVSKY TERNOPIL NATIONAL MEDICAL UNIVERSITY OF THE MINISTRY OF HEALTH OF UKRAINE, TERNOPIL, UKRAINE
| | - Svitlana I Shkrobot
- I. HORBACHEVSKY TERNOPIL NATIONAL MEDICAL UNIVERSITY OF THE MINISTRY OF HEALTH OF UKRAINE, TERNOPIL, UKRAINE
| |
Collapse
|
|
26
|
Cetera GE, D'Ambrosi F, Di Martino DD, Landi L, Criscuolo S, Caschera L, Cesano N, Ossola MW. An uncommon presentation of Wernicke-Korsakoff's syndrome in pregnancy: Case report. Radiol Case Rep 2022; 18:921-925. [PMID: 36593922 PMCID: PMC9803690 DOI: 10.1016/j.radcr.2022.11.061] [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: 10/23/2022] [Revised: 11/18/2022] [Accepted: 11/23/2022] [Indexed: 12/25/2022] Open
Abstract
Wernicke's encephalopathy is an acute neuropsychiatric syndrome resulting from severe thiamine (vitamin B1) deficiency. Symptoms occur with an acute onset and may vary according to the brain area involved. Altered consciousness is the most common clinical feature, together with ocular abnormalities and ataxia. We report the case of a pregnant women affected by pre-gestational hyperthyroidism that caused an uncommon presentation of Wernicke's encephalopathy. Symptoms differed from the classic triad and diagnosis was made possible by a thorough analysis of anamnestic factors and brain MRI. Alongside thiamine supplementation, a multidisciplinary approach which included physiokinesis and a phoniatric support was fundamental for the patient's recovery.
Collapse
Affiliation(s)
- Giulia Emily Cetera
- Department of Woman, Child and Neonate, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Francesco Sforza, 35, Milan 20122 , Italy
| | - Francesco D'Ambrosi
- Department of Woman, Child and Neonate, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Francesco Sforza, 35, Milan 20122 , Italy,Corresponding author.
| | - Daniela Denis Di Martino
- Department of Woman, Child and Neonate, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Francesco Sforza, 35, Milan 20122 , Italy
| | - Laura Landi
- Department of Pediatric anesthesia and resuscitation, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Stefania Criscuolo
- Postgraduational School of Radiodiagnostic, Università degli Studi di Milano, Milan, Italy
| | - Luca Caschera
- Department of Neuroradiology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Nicola Cesano
- Department of Woman, Child and Neonate, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Francesco Sforza, 35, Milan 20122 , Italy
| | - Manuela Wally Ossola
- Department of Woman, Child and Neonate, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Francesco Sforza, 35, Milan 20122 , Italy
| |
Collapse
|
|
27
|
Muacevic A, Adler JR, Chang JS, Lui F. Wernicke's Encephalopathy Presenting With Confusion in a Patient With Schizophrenia. Cureus 2022; 14:e32320. [PMID: 36628041 PMCID: PMC9825115 DOI: 10.7759/cureus.32320] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/08/2022] [Indexed: 12/13/2022] Open
Abstract
Wernicke's encephalopathy (WE) is a neuropsychiatric condition caused by thiamine deficiency often associated with alcoholism. Other less common causes include prolonged gastroenterology problems or dietary insufficiencies associated with hyperemesis gravidarum, bariatric surgery, and eating disorders. Prolonged WE without proper treatment can lead to the chronic and irreversible condition, Wernicke-Korsakoff syndrome. Despite being known for its classic triad of clinical symptoms (nystagmus/ophthalmoplegia, gait ataxia, and confusion), WE patients more commonly present with non-specific symptoms of altered mental status. Obscure clinical presentations often led to delays in the appropriate of patients with WE. We are presenting a case of WE that is unusual because the underlying cause is schizophrenia and the lack of alcohol use. For a punctual diagnosis, a high index of suspicion is essential to prevent further exacerbation of neuronal death seen in WE. IV thiamine should be administered to any patient with acute encephalopathy or altered mental status, given its low cost and lack of side effects.
Collapse
|
|
28
|
Idris S, Ahmed AA, Ali R, Alsharari H, Ahmed EN, Deeba F. Wernicke’s Encephalopathy: A Precipitation of Glycogen Storage Disease. Cureus 2022; 14:e31471. [DOI: 10.7759/cureus.31471] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/13/2022] [Indexed: 11/16/2022] Open
|
|
29
|
Moya M, Escudero B, Gómez-Blázquez E, Rebolledo-Poves AB, López-Gallardo M, Guerrero C, Marco EM, Orio L. Upregulation of TLR4/MyD88 pathway in alcohol-induced Wernicke’s encephalopathy: Findings in preclinical models and in a postmortem human case. Front Pharmacol 2022; 13:866574. [PMID: 36225571 PMCID: PMC9549119 DOI: 10.3389/fphar.2022.866574] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/31/2022] [Accepted: 07/29/2022] [Indexed: 11/13/2022] Open
Abstract
Wernicke’s encephalopathy (WE) is a neurologic disease caused by vitamin B1 or thiamine deficiency (TD), being the alcohol use disorder its main risk factor. WE patients present limiting motor, cognitive, and emotional alterations related to a selective cerebral vulnerability. Neuroinflammation has been proposed to be one of the phenomena that contribute to brain damage. Our previous studies provide evidence for the involvement of the innate immune receptor Toll-like (TLR)4 in the inflammatory response induced in the frontal cortex and cerebellum in TD animal models (animals fed with TD diet [TDD] and receiving pyrithiamine). Nevertheless, the effects of the combination of chronic alcohol consumption and TD on TLR4 and their specific contribution to the pathogenesis of WE are currently unknown. In addition, no studies on TLR4 have been conducted on WE patients since brains from these patients are difficult to achieve. Here, we used rat models of chronic alcohol (CA; 9 months of forced consumption of 20% (w/v) alcohol), TD hit (TDD + daily 0.25 mg/kg i.p. pyrithiamine during 12 days), or combined treatment (CA + TDD) to check the activation of the proinflammatory TLR4/MyD88 pathway and related markers in the frontal cortex and the cerebellum. In addition, we characterized for the first time the TLR4 and its coreceptor MyD88 signature, along with other markers of this proinflammatory signaling such as phospo-NFκB p65 and IκBα, in the postmortem human frontal cortex and cerebellum (gray and white matter) of an alcohol-induced WE patient, comparing it with negative (no disease) and positive (aged brain with Alzheimer’s disease) control subjects for neuroinflammation. We found an increase in the cortical TLR4 and its adaptor molecule MyD88, together with an upregulation of the proinflammatory signaling molecules p-NF-ĸB and IĸBα in the CA + TDD animal model. In the patient diagnosed with alcohol-induced WE, we observed cortical and cerebellar upregulation of the TLR4/MyD88 pathway. Hence, our findings provide evidence, both in the animal model and the human postmortem brain, of the upregulation of the TLR4/MyD88 proinflammatory pathway in alcohol consumption–related WE.
Collapse
Affiliation(s)
- Marta Moya
- Department of Psychobiology and Methods in Behavioral Science, Faculty of Psychology, Complutense University of Madrid, Madrid, Spain
| | - Berta Escudero
- Department of Psychobiology and Methods in Behavioral Science, Faculty of Psychology, Complutense University of Madrid, Madrid, Spain
| | | | | | | | - Carmen Guerrero
- Biobanco of Hospital Universitario Fundación Alcorcón, Alcorcón, Spain
| | - Eva M. Marco
- Department of Genetics, Physiology and Microbiology, Faculty of Biology, Complutense University of Madrid, Madrid, Spain
| | - Laura Orio
- Department of Psychobiology and Methods in Behavioral Science, Faculty of Psychology, Complutense University of Madrid, Madrid, Spain
- Research Network in Primary Care in Addictions (Red de Investigación en Atención Primaria en Adicciones), Riapad, Spain
- *Correspondence: Laura Orio,
| |
Collapse
|
|
30
|
Abu-Abaa M. Seizure as the Main Manifestation of Nonalcoholic Wernicke’s Encephalopathy but Without Cortical Involvement: A Case Report. Cureus 2022; 14:e28866. [PMID: 36225406 PMCID: PMC9541440 DOI: 10.7759/cureus.28866] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/06/2022] [Indexed: 11/05/2022] Open
|
|
31
|
Ding LM, Deng LS, Qian JJ, Liu G, Zhou L, Zheng SS. Clinical analysis of Wernicke encephalopathy after liver transplantation. Hepatobiliary Pancreat Dis Int 2022:S1499-3872(22)00181-3. [PMID: 35909062 DOI: 10.1016/j.hbpd.2022.07.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2022] [Accepted: 07/08/2022] [Indexed: 02/05/2023]
Abstract
BACKGROUND Wernicke encephalopathy (WE) is an acute neurological disease resulting from vitamin B1 deficiency, and there are only very few case reports of WE after liver transplantation. The present study aimed to investigate the clinical characteristics, etiology, magnetic resonance imaging (MRI) features, treatment and prognosis of patients with WE after liver transplantation. METHODS Twenty-three patients with WE after liver transplantation from the First Affiliated Hospital, Zhejiang University School of Medicine and Jiangxi Provincial People's Hospital between January 2011 and December 2021 were retrospectively analyzed. RESULTS Among the 23 patients diagnosed with WE after liver transplantation, 6 (26%) had a classic triad of impaired consciousness, oculomotor palsy and ataxia, 17 (74%) had two features. The misdiagnosis rate was 65%. After treatment with high-dose vitamin B1, 19 (83%) patients showed improvement, whereas 4 showed no improvement, including 3 with residual short-term memory impairments and 1 with residual spatial and temporal disorientation and ataxia. CONCLUSIONS The misdiagnosis rate is high in the early stage of WE, and the prognosis is closely associated with whether WE is diagnosed early and treated timely. High-dose glucose or glucocorticoids can trigger WE and cannot be administered before vitamin B1 treatment. Vitamin B1 is suggested to be used as a prophylactic treatment for patients with WE after liver transplantation.
Collapse
Affiliation(s)
- Li-Min Ding
- Division of Hepatobiliary Pancreatic Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China; NHFPC Key Laboratory of Combined Multi-organ Transplantation, Hangzhou 310003, China; Key Laboratory of the diagnosis and treatment of organ Transplantation, Chinese Academy of Medical Sciences, Hangzhou 310003, China; Key Laboratory of Organ Transplantation, Research Center for Diagnosis and Treatment of Hepatobiliary Diseases, Zhejiang Province, Hangzhou 310003, China
| | - Li-Shan Deng
- Culture and Sports Center for the Disabled of Jiangxi Province, Nanchang 330000, China
| | - Jun-Jie Qian
- Division of Hepatobiliary Pancreatic Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China; NHFPC Key Laboratory of Combined Multi-organ Transplantation, Hangzhou 310003, China; Key Laboratory of the diagnosis and treatment of organ Transplantation, Chinese Academy of Medical Sciences, Hangzhou 310003, China; Key Laboratory of Organ Transplantation, Research Center for Diagnosis and Treatment of Hepatobiliary Diseases, Zhejiang Province, Hangzhou 310003, China
| | - Gang Liu
- Department of Transplantation, Jiangxi Provincial People's Hospital, Nanchang 330000, China
| | - Lin Zhou
- NHFPC Key Laboratory of Combined Multi-organ Transplantation, Hangzhou 310003, China; Key Laboratory of the diagnosis and treatment of organ Transplantation, Chinese Academy of Medical Sciences, Hangzhou 310003, China; Key Laboratory of Organ Transplantation, Research Center for Diagnosis and Treatment of Hepatobiliary Diseases, Zhejiang Province, Hangzhou 310003, China; Collaborative Innovation Center for Diagnosis Treatment of Infectious Diseases, Hangzhou 310003, China
| | - Shu-Sen Zheng
- Division of Hepatobiliary Pancreatic Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China; NHFPC Key Laboratory of Combined Multi-organ Transplantation, Hangzhou 310003, China; Key Laboratory of the diagnosis and treatment of organ Transplantation, Chinese Academy of Medical Sciences, Hangzhou 310003, China; Key Laboratory of Organ Transplantation, Research Center for Diagnosis and Treatment of Hepatobiliary Diseases, Zhejiang Province, Hangzhou 310003, China; Collaborative Innovation Center for Diagnosis Treatment of Infectious Diseases, Hangzhou 310003, China.
| |
Collapse
|
|
32
|
Ishikawa S, Ando K, Katakami T, Kawamoto M. Cervical cord lesions in Wernicke's encephalopathy. Radiol Case Rep 2022; 17:2424-2427. [PMID: 35601381 PMCID: PMC9118102 DOI: 10.1016/j.radcr.2022.04.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2022] [Revised: 04/04/2022] [Accepted: 04/05/2022] [Indexed: 11/23/2022] Open
Abstract
A 30-year-old woman suffering from an eating disorder and alcoholism presented with a progressively worsening gait disturbance lasting 2 weeks. Her neurological findings included impaired ocular motility and trunk ataxia. Fluid-attenuated inversion recovery imaging of the brain showed hyperintensity in the dorsal brainstem, aqueduct, thalamus, and cerebral cortex. A long hyperintense segment on T2-weighted imaging was visible in the central gray matter of the cervical spinal cord. No restricted diffusion was observed; thus, T2 elongation in the spine was suggested to be due to vasogenic edema. We diagnosed the patient with Wernicke's encephalopathy and initiated vitamin supplementation. Thereafter, her symptoms rapidly improved; magnetic resonance imaging on the 11th day of hospitalization showed normalization of the signals in her brain and spinal cord. As our case demonstrates, Wernicke's encephalopathy can induce vasogenic edema of the spinal cord, which can rapidly improve with early therapeutic intervention.
Collapse
|
|
33
|
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.
Collapse
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
| |
Collapse
|
|
34
|
Brainstem lesions: MRI review of standard morphological sequences. Acta Neurol Belg 2022; 122:597-613. [PMID: 35428930 DOI: 10.1007/s13760-022-01943-y] [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: 07/29/2021] [Accepted: 03/23/2022] [Indexed: 11/01/2022]
Abstract
MRI signal changes in the brainstem are observed in a multitude of disorders including vascular diseases, neoplastic lesions, degenerative diseases, inflammatory disorders, metabolic diseases, infections, and trauma. In some diseases, brainstem involvement is typical and sometimes isolated, while in other diseases, brainstem lesions are only observed occasionally in the presence of other typical extra-brainstem abnormalities. In this review, we will discuss the MRI characteristics of brainstem lesions observed in different disorders associated with frequent and less frequent brainstem involvement. Identification of the origin of the brainstem lesion depends on the exact localisation of the lesion(s) inside the brainstem, the presence and the characteristics of associated lesions seen outside the brainstem, the signal changes on different MRI sequences, the evolution over time of the radiological abnormalities, the history and clinical state of the patient, and other radiological and non-radiological examinations.
Collapse
|
|
35
|
Skok H, Jabour J, Betcher J. Wernicke Korsakoff syndrome in a teenage female as a complication of COVID‐19. J Am Coll Emerg Physicians Open 2022; 3:e12735. [PMID: 35505931 PMCID: PMC9051863 DOI: 10.1002/emp2.12735] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2022] [Revised: 04/09/2022] [Accepted: 04/14/2022] [Indexed: 12/13/2022] Open
Abstract
Wernicke encephalitis (WE) is usually associated with alcohol use disorder and caused by a deficiency in thiamine. Classic findings include confusion, ataxia, and ophthalmoplegia. This case is a unique presentation of WE in a 14‐year‐old female related to prior coronavirus disease infection. She had persistent dysgeusia and developed thiamine deficiency. She presented with confusion, ataxia, and changes in speech. She had a prolonged hospitalization but was discharged to an inpatient rehab facility with persistent symptoms. It is prudent to include thiamine deficiency in the differential for patients with any symptoms of WE and a history of nutritional deficiency.
Collapse
Affiliation(s)
- Hannah Skok
- Department of Emergency Medicine Trinity Health West Michigan Muskegon Michigan USA
| | - Joseph Jabour
- Department of Emergency Medicine Henry Ford Health System Detroit Michigan USA
| | - Joseph Betcher
- Department of Emergency Medicine Trinity Health West Michigan Muskegon Michigan USA
| |
Collapse
|
|
36
|
Ahmad S, Ikram S, Dunn BK. Fatal Wernicke's Encephalopathy with Cardiovascular Involvement in a Young Psychiatric Patient. Am J Med Sci 2021; 363:273-278. [PMID: 34861213 DOI: 10.1016/j.amjms.2021.10.015] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2021] [Revised: 06/26/2021] [Accepted: 10/21/2021] [Indexed: 11/01/2022]
Abstract
Wernicke's encephalopathy (WE) is an acute neurological disorder caused by thiamine deficiency that is frequently missed in non-alcoholic patients. Coma and cardiomyopathy are uncommon presentations of WE that have been rarely reported in the literature. We report the case of a 36-year-old male with a known history of schizophrenia who presented with coma and vasopressor refractory hypotension. Initial computed tomography (CT) of the head at admission was unremarkable. Transthoracic echocardiogram showed diffuse myocardial hypokinesia with a left ventricular ejection fraction of 40%-45%. Due to persistent encephalopathy, a repeat non-enhanced CT head was obtained on the second day of hospital admission followed by magnetic resonance imaging (MRI) of brain that showed findings suggestive of WE. The patient was immediately started on high-dose intravenous (IV) thiamine therapy. Although his hemodynamic parameters significantly improved following thiamine replacement, he did not show signs of neurological recovery and resulted in a dismal outcome. This case illustrates the importance of early recognition of thiamine deficiency in critically ill patients to prevent fatal outcomes. Immediate parenteral thiamine administration should be considered in all patients presenting with coma, cardiomyopathy, and refractory hypotension regardless of their body mass index, and alcohol use status.
Collapse
Affiliation(s)
- Soban Ahmad
- Department of Internal Medicine, East Carolina University, Greenville, North Carolina, USA.
| | - Sundus Ikram
- Department of Internal Medicine, SEGi University, Kota Damansara, Malaysia
| | - Bryan K Dunn
- Division of Pulmonary, Critical Care, and Sleep Medicine, East Carolina University, Greenville, North Carolina, USA
| |
Collapse
|
|
37
|
Clinical diagnosis, outcomes and treatment of thiamine deficiency in a tertiary hospital. Clin Nutr 2021; 41:33-39. [PMID: 34864453 DOI: 10.1016/j.clnu.2021.10.021] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2021] [Revised: 10/09/2021] [Accepted: 10/29/2021] [Indexed: 11/23/2022]
Abstract
BACKGROUND Acute thiamine deficiency can occur in patients with or without history of alcohol abuse and can lead to life-threatening complications. Clinical diagnosis is challenging, often resulting in delayed recognition and treatment. Patients may present with heterogenous symptoms, more diverse than the historical neurological description. Cerebral MRI can contribute to the diagnosis in patients with neurological signs but it is not always feasible in emergency settings. Prompt parenteral supplementation is required to obtain the improvement of symptoms and avoid chronic complications. AIMS To describe the clinical presentation of reported cases of thiamine deficiency, assess prescription and results of cerebral imaging, review treatments that had been prescribed in accordance or not with available guidelines, and study the short-term outcome of these patients. METHODS This is a monocentric retrospective analysis of all reported cases of thiamine deficiency in a French tertiary hospital between January 1st 2008 and December 31st 2018. RESULTS Fifty-six cases were identified during the study period. Forty-five (80%) patients had a history of alcohol abuse. Most patients were diagnosed based on neurological symptoms but non-specific and digestive symptoms were frequent. Thirty-four percent of patients fulfilled clinical criteria for malnutrition. A brain MRI was performed in 54% of patients and was abnormal in 63% of these cases. Eighty-five percent of patients were treated by parenteral thiamine administration and the supplementation was continued orally in 55% of them. The majority of patients initially received 1000 mg daily of IV thiamine but the dose and duration of thiamine supplementation were variable. At the time of discharge, partial or complete improvement of symptoms was noted in 59% of patients. CONCLUSION This study highlights the clinical and radiological heterogeneity of thiamine deficiency. These observations should encourage starting thiamine supplementation early in patients with risk factors or suggestive symptoms even in non-alcoholic patients, and underline the importance of early nutritional support.
Collapse
|
|
38
|
Walker MA, Miranda M, Allred A, Mootha VK. On the dynamic and even reversible nature of Leigh syndrome: Lessons from human imaging and mouse models. Curr Opin Neurobiol 2021; 72:80-90. [PMID: 34656053 PMCID: PMC8901530 DOI: 10.1016/j.conb.2021.09.006] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2021] [Revised: 08/01/2021] [Accepted: 09/07/2021] [Indexed: 12/13/2022]
Abstract
Leigh syndrome (LS) is a neurodegenerative disease characterized by bilaterally symmetric brainstem or basal ganglia lesions. More than 80 genes, largely impacting mitochondrial energy metabolism, can underlie LS, and no approved medicines exist. Described 70 years ago, LS was initially diagnosed by the characteristic, necrotic lesions on autopsy. It has been broadly assumed that antemortem neuroimaging abnormalities in these regions correspond to end-stage histopathology. However, clinical observations and animal studies suggest that neuroimaging findings may represent an intermediate state, that is more dynamic than previously appreciated, and even reversible. We review this literature, discuss related conditions that are treatable, and present two new LS cases with radiographic improvement. We review studies in which hypoxia reverses advanced LS in a mouse model. The fluctuating and potentially reversible nature of radiographic LS lesions will be important in clinical trial design. Better understanding of this plasticity could lead to new therapies.
Collapse
Affiliation(s)
- Melissa A Walker
- Howard Hughes Medical Institute, Department of Molecular Biology, Massachusetts General Hospital, United States; Broad Institute of Harvard, MIT, United States; Department of Neurology, Massachusetts General Hospital, United States.
| | - Maria Miranda
- Howard Hughes Medical Institute, Department of Molecular Biology, Massachusetts General Hospital, United States; Broad Institute of Harvard, MIT, United States
| | - Amanda Allred
- Howard Hughes Medical Institute, Department of Molecular Biology, Massachusetts General Hospital, United States
| | - Vamsi K Mootha
- Howard Hughes Medical Institute, Department of Molecular Biology, Massachusetts General Hospital, United States; Broad Institute of Harvard, MIT, United States.
| |
Collapse
|
|
39
|
Jeon SJ, Choi SS, Kim HY, Yu IK. Acute Acquired Metabolic Encephalopathy Based on Diffusion MRI. Korean J Radiol 2021; 22:2034-2051. [PMID: 34564957 PMCID: PMC8628163 DOI: 10.3348/kjr.2019.0303] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/15/2019] [Revised: 11/02/2020] [Accepted: 11/20/2020] [Indexed: 11/22/2022] Open
Abstract
Metabolic encephalopathy is a critical condition that can be challenging to diagnose. Imaging provides early clues to confirm clinical suspicions and plays an important role in the diagnosis, assessment of the response to therapy, and prognosis prediction. Diffusion-weighted imaging is a sensitive technique used to evaluate metabolic encephalopathy at an early stage. Metabolic encephalopathies often involve the deep regions of the gray matter because they have high energy requirements and are susceptible to metabolic disturbances. Understanding the imaging patterns of various metabolic encephalopathies can help narrow the differential diagnosis and improve the prognosis of patients by initiating proper treatment regimen early.
Collapse
Affiliation(s)
- Se Jeong Jeon
- Department of Radiology, Wonkwang University Hospital, Iksan, Korea
| | - See Sung Choi
- Department of Radiology, Wonkwang University Hospital, Iksan, Korea
| | - Ha Yon Kim
- Department of Radiology, Eulji University Hospital, Deajeon, Korea
| | - In Kyu Yu
- Department of Radiology, Eulji University Hospital, Deajeon, Korea.
| |
Collapse
|
|
40
|
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]
|
|
41
|
Moya M, San Felipe D, Ballesta A, Alén F, Rodríguez de Fonseca F, García-Bueno B, Marco EM, Orio L. Cerebellar and cortical TLR4 activation and behavioral impairments in Wernicke-Korsakoff Syndrome: Pharmacological effects of oleoylethanolamide. Prog Neuropsychopharmacol Biol Psychiatry 2021; 108:110190. [PMID: 33271211 DOI: 10.1016/j.pnpbp.2020.110190] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/29/2020] [Revised: 11/27/2020] [Accepted: 11/27/2020] [Indexed: 01/19/2023]
Abstract
Wernicke-Korsakoff Syndrome (WKS) is a neuropsychiatric disorder whose etiology is a thiamine deficiency (TD), with alcoholism being the main underlying cause. Previous evidence suggests the presence of initial neuroinflammation and oxidative/nitrosative stress in the physiopathology, although the specific molecular mechanisms underlying TD-induced brain damage and behavioral disabilities are unknown. We explored the specific role of the innate immune receptor TLR4 in three murine models of WKS, based on the combination of a thiamine-deficient diet and pyrithiamine injections (0.25 mg/kg, i.p.) over time. The Symptomatic Model (SM) allowed us to describe the complete neurological/neurobehavioral symptomatology over 16 days of TD. Animals showed an upregulation of the TLR4 signaling pathway both in the frontal cortex (FC) and cerebellum and clear motor impairments related with cerebellar dysfunction. However, in the Pre-Symptomatic Model (PSM), 12 days of TD induced the TLR4 pathway upregulation in the FC, which correlated with disinhibited-like behavior, but not in the cerebellum, and no motor impairments. In addition, we tested the effects of the biolipid oleoylethanolamide (OEA, 10 mg/kg, i.p., once daily, starting before any symptom of the pathology is manifested) through the Glucose-Precipitated Model (GPM), which was generated by glucose loading (5 g/kg, i.v., last day) in thiamine-deficient animals to accelerate damage. Pretreatment with OEA prevented the TLR4-induced signature in the FC, as well as an underlying incipient memory disability and disinhibited-like behavior. This study suggests a key role for TLR4 in TD-induced neuroinflammation in the FC and cerebellum, and it reveals different vulnerability of these brain regions in WKS over time. Pre-treatment with OEA counteracts TD-induced TLR4-associated neuroinflammation and may serve as co-adjuvant therapy to prevent WKS-induced neurobehavioral alterations.
Collapse
Affiliation(s)
- Marta Moya
- Department of Psychobiology and Behavioral Sciences Methods, Faculty of Psychology, Universidad Complutense de Madrid (UCM), Madrid, Spain; Red de Trastornos Adictivos (RTA) del Instituto de Salud Carlos III (ISCIII), Spain
| | - Diego San Felipe
- Department of Genetics, Physiology and Microbiology, Faculty of Biology, UCM, Spain
| | - Antonio Ballesta
- Department of Psychobiology and Behavioral Sciences Methods, Faculty of Psychology, Universidad Complutense de Madrid (UCM), Madrid, Spain
| | - Francisco Alén
- Department of Psychobiology and Behavioral Sciences Methods, Faculty of Psychology, Universidad Complutense de Madrid (UCM), Madrid, Spain
| | - Fernando Rodríguez de Fonseca
- Department of Psychobiology and Behavioral Sciences Methods, Faculty of Psychology, Universidad Complutense de Madrid (UCM), Madrid, Spain; Instituto de Investigación Biomédica de Málaga (IBIMA), UGC Salud Mental, Hospital Regional de Málaga, Spain; Red de Trastornos Adictivos (RTA) del Instituto de Salud Carlos III (ISCIII), Spain
| | - Borja García-Bueno
- Department of Pharmacology and Toxicology, Faculty of Medicine, UCM, Centro de Investigación Biomédica en Red de Salud Mental (CIBERSAM), Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12), Instituto Universitario de Investigación en Neuroquímica UCM, Spain
| | - Eva M Marco
- Department of Genetics, Physiology and Microbiology, Faculty of Biology, UCM, Spain; Red de Trastornos Adictivos (RTA) del Instituto de Salud Carlos III (ISCIII), Spain
| | - Laura Orio
- Department of Psychobiology and Behavioral Sciences Methods, Faculty of Psychology, Universidad Complutense de Madrid (UCM), Madrid, Spain; Red de Trastornos Adictivos (RTA) del Instituto de Salud Carlos III (ISCIII), Spain.
| |
Collapse
|
|
42
|
Ayroulet C, Roussin C, Vanhecke C. Wernicke encephalopathy, dysautonomia and excessive soft drink consumption: An unusual triad. NUTR CLIN METAB 2021. [DOI: 10.1016/j.nupar.2021.02.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
|
|
43
|
Abdi R, Siham C, Maadan A, Sekhsoukh R. [A Wernicke's encephalopathy with ophthalmological revelation]. J Fr Ophtalmol 2021; 44:e385-e387. [PMID: 33902935 DOI: 10.1016/j.jfo.2020.11.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2020] [Revised: 11/03/2020] [Accepted: 11/16/2020] [Indexed: 10/21/2022]
Affiliation(s)
- R Abdi
- Service d'ophtalmologie, CHU Mohammed VI, 60049 Oujda, Maroc.
| | - C Siham
- Service d'ophtalmologie, CHU Mohammed VI, 60049 Oujda, Maroc
| | - A Maadan
- Service d'ophtalmologie, CHU Mohammed VI, 60049 Oujda, Maroc
| | - R Sekhsoukh
- Service d'ophtalmologie, CHU Mohammed VI, 60049 Oujda, Maroc
| |
Collapse
|
|
44
|
Wang Q, Charmchi Z, George IC. Restrictive diet in a patient with irritable bowel syndrome leading to Wernicke encephalopathy. BMC Gastroenterol 2021; 21:179. [PMID: 33879093 PMCID: PMC8056557 DOI: 10.1186/s12876-021-01758-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/03/2020] [Accepted: 04/09/2021] [Indexed: 12/02/2022] Open
Abstract
Background We present a case of a woman with a past medical history of irritable bowel syndrome (IBS) and anxiety, who presents with ophthalmoplegia, ataxia and memory loss, characteristic of Wernicke encephalopathy. Case presentation A 64-year-old woman presented with double vision, unsteady gait and memory loss. These symptoms began after 3 months on an unfortified restricted diet, which she initiated to alleviate IBS symptoms. Magnetic resonance imaging of the brain demonstrated hyperintense T2-weighted signal in the dorsomedial aspect of bilateral thalami, periaqueductal grey matter and around the third ventricle. The patient’s visual symptoms improved significantly after thiamine supplementation, although her memory deficits persisted. Conclusion Although WE is often associated with chronic alcohol abuse, this case demonstrates the importance of recognizing WE in any patient with a restricted diet and subsequent timely initiation of thiamine.
Collapse
Affiliation(s)
- Qiang Wang
- Department of Neurology, Kings County Hospital Center, SUNY Downstate Medical Center and Maimonides Medical Center, SUNY Downstate Health Sciences University, Brooklyn, NY, USA
| | - Zeinab Charmchi
- Department of Neurology, Kings County Hospital Center, SUNY Downstate Medical Center and Maimonides Medical Center, SUNY Downstate Health Sciences University, Brooklyn, NY, USA
| | - Ilena C George
- Department of Neurology, Massachusetts General Hospital, 55 Fruit St, Boston, MA, 02114, USA.
| |
Collapse
|
|
45
|
Encephalopathy responsive to thiamine in severe COVID-19 patients. Brain Behav Immun Health 2021; 14:100252. [PMID: 33817670 PMCID: PMC8011322 DOI: 10.1016/j.bbih.2021.100252] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2021] [Revised: 03/27/2021] [Accepted: 03/28/2021] [Indexed: 12/25/2022] Open
Abstract
Encephalopathy is one of the most frequent neurological complications of severe Coronavirus Disease 2019 (COVID-19) patients. Cytokine storm and sepsis, hypercatabolic states, the use of furosemide and dialytic therapy represent risk factors for thiamine deficiency and are also found in patients with severe COVID-19. In this retrospective case series, we report clinical and neurological findings of fifteen patients with COVID-19-associated Wernicke Encephalopathy (WE) and their response to treatment with intravenous thiamine. All patients had encephalopathy, with 67% displaying at least one additional sign of classic WE triad (ophthalmoparesis and ataxia). Two patients (13%) had the classic triad. All COVID-19 patients had significant improvement of the neurological manifestations between two to five days after intravenous thiamine administration. Eleven patients (73%) had good neurological outcome at hospital discharge and only two patients (13%) died. This case series suggests that thiamine deficiency may be an etiology of encephalopathy in severe COVID-19 patients and its treatment may represent a safety and low-cost response to reduce the neurological burden.
Collapse
|
|
46
|
Mutti C, Ciliento R, Parrino L, Florindo I, Pavesi G, Zinno L. Apathetic encephalopathy in thyreotoxicosis: an unsual cause of wernicke encephalopathy and osmotic demyelinating syndrome. ACTA BIO-MEDICA : ATENEI PARMENSIS 2021; 92:e2021055. [PMID: 33682841 PMCID: PMC7975956 DOI: 10.23750/abm.v92i1.9473] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/30/2020] [Accepted: 04/06/2020] [Indexed: 11/23/2022]
Abstract
Non-alcoholic Wernicke Encephalopathy (WE) is an overlooked complication of malnourishment in all its forms including undernutrition, inadequate vitamine intake and endocrinological diseases. Both delay in treatment and overtreatment can lead to severe neurological life-long consequences. Inadequate management in patients with chronic malnutrition may cause abrupt osmolytes unbalance and subsequent osmotic demyelination syndromes. We describe a 65-year old man with apathetic encephalopathy caused by thyreotoxicosis and associated with malnutrition and severe thiamine depletion.
Collapse
Affiliation(s)
| | - Rosario Ciliento
- Neurology Unit, Department of Medicine and Surgery, University of Parma, Parma, Italy.
| | - Liborio Parrino
- Sleep Medicine Center, Neurology Unit, Department of General and Specialistic Medicine, University Hospital of Parma, Parma, Italy.
| | - Irene Florindo
- Neurology Unit, Department of Medicine and Surgery, University of Parma, Parma, Italy.
| | - Giovanni Pavesi
- Neurology Unit, Department of Medicine and Surgery, University of Parma, Parma, Italy.
| | - Lucia Zinno
- Neurology Unit, Department of Medicine and Surgery, University of Parma, Parma, Italy.
| |
Collapse
|
|
47
|
Tarhan B, Rahman S, Joseph N, Hyder D, Zingariello C, Borum PR, Sladky J, Winesett SP. Vitamins are Indeed Vital Amines: A Discussion of 3 Deficiencies With Neurologic Manifestations. Child Neurol Open 2021; 8:2329048X211046440. [PMID: 34708143 PMCID: PMC8544767 DOI: 10.1177/2329048x211046440] [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: 06/17/2021] [Accepted: 08/27/2021] [Indexed: 11/15/2022] Open
Abstract
Optimal functioning of the human nervous system depends on a constant supply of nutrients, vitamins, and minerals. In the developed world, nutritional deficiencies are relatively rare and infrequently present with neurologic manifestations. These neurologic disorders can be mistaken for inflammatory and/or autoimmune phenomena. This manuscript describes 2 pediatric cases with neurologic signs/symptoms arising from vitamin deficiencies-(1) optic neuropathy and (2) Wernicke encephalopathy associated with a Guillain-Barre-like pattern of weakness. The 2 cases and the subsequent discussion of vitamin A, B1, and B12 deficiencies underscore the value of taking a thorough dietary history and emphasize risk factors for these 3 nutritional deficiencies.
Collapse
Affiliation(s)
- Bedirhan Tarhan
- University of Florida College of Medicine, Gainesville, FL, USA
| | - Sydur Rahman
- University of Florida College of Medicine, Gainesville, FL, USA
| | - Nancy Joseph
- University of Florida College of Medicine, Gainesville, FL, USA
| | - Douglas Hyder
- University of Florida College of Medicine, Gainesville, FL, USA
| | | | - Peggy R. Borum
- University of Florida College of Medicine, Gainesville, FL, USA
| | - John Sladky
- University of Florida College of Medicine, Gainesville, FL, USA
| | | |
Collapse
|
|
48
|
Khan F, Sharma N, Ud Din M, Bansal V. Isolated Pulvinar/Hockey Stick Sign in Nonalcoholic Wernicke's Encephalopathy. AMERICAN JOURNAL OF CASE REPORTS 2020; 21:e928272. [PMID: 33380716 PMCID: PMC7784713 DOI: 10.12659/ajcr.928272] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
BACKGROUND Wernicke's encephalopathy (WE), a commonly misdiagnosed and underdiagnosed pathology, presents with altered mental status, ataxia, and ophthalmoplegia. WE is most commonly caused by excessive alcohol use, but also has diverse nonalcoholic etiologies. Here we describe 2 cases of nonalcoholic WE with different etiologies that were initially misdiagnosed due to lack of correlation of magnetic resonance imaging (MRI) findings with clinical information. CASE REPORT Patient A, a 50-year-old woman with recent gastric sleeve surgery, presented with horizontal gaze-evoked nystagmus, ataxia, and altered mental status. MRI fluid-attenuated inversion recovery (FLAIR) revealed isolated bilateral, symmetrical, thalamic hyperintensities, initially diagnosed as variant Creutzfeldt-Jakob disease. A review of imaging and clinical presentation provided an alternate diagnosis of nonalcoholic WE secondary to nutritional deficiency. Intravenous (IV) thiamine improved symptoms with resolution of MRI findings 6 months later. Patient B, a 64-year-old woman, presented with nausea, vomiting, dizziness, altered mental status, and weight loss. MRI FLAIR revealed isolated bilateral, symmetrical, thalamic hyperintensities, initially determined to be ischemia, prompting stroke management. A diagnosis of nonalcoholic WE was suggested, given the patient's low thiamine levels and history of malnutrition, and was confirmed by her excellent therapeutic response to IV thiamine. CONCLUSIONS Nonalcoholic WE remains a challenging diagnosis because of the variable clinical presentation, myriad of underlying etiologies, and lack of standardized diagnostic laboratory tests. A multidisciplinary approach with close collaboration between the radiologist and clinical care team is critical to narrow down the differential and initiate correct management. WE is a reversible disease with catastrophic consequences if it is not recognized and treated promptly.
Collapse
Affiliation(s)
- Faisal Khan
- Department of Neurology, Sugar Land Neurology and Sleep, Sugar Land, TX, USA.,Department of Neurology, Sam Houston University College of Osteopathic Medicine, Hunstville, TX, USA
| | - Neha Sharma
- Research Fellow, Houston Medical Clerkship, Sugar Land, TX, USA
| | - Moin Ud Din
- Research Fellow, Houston Medical Clerkship, Sugar Land, TX, USA
| | - Vivek Bansal
- Department of Neuroradiology, Radiology Partners Gulf Coast, Houston, TX, USA
| |
Collapse
|
|
49
|
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
| |
Collapse
|
|
50
|
Shah R, Malhamé I, Fayek M, Merolli A, Mehta N. Wernicke's encephalopathy: An uncommon complication from hyperemesis gravidarum. Obstet Med 2020; 13:198-200. [PMID: 33343698 PMCID: PMC7726169 DOI: 10.1177/1753495x18811515] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2018] [Accepted: 10/16/2018] [Indexed: 11/15/2022] Open
Abstract
Hyperemesis gravidarum is a complication of pregnancy associated with severe nausea and vomiting that can lead to fluid-electrolyte imbalances and nutritional deficiencies. Wernicke's encephalopathy is a neurologic manifestation of acute thiamine (vitamin B1) deficiency. We describe a case of hyperemesis gravidarum presenting with gait ataxia and nystagmus which led to a diagnosis of Wernicke's encephalopathy.
Collapse
Affiliation(s)
- Roshan Shah
- Department of Medicine, Women and Infants Hospital, Providence, USA
| | - Isabelle Malhamé
- Department of Medicine, Women and Infants Hospital, Providence, USA
| | - Mariam Fayek
- Department of Medicine, Women and Infants Hospital, Providence, USA
| | | | - Niharika Mehta
- Department of Medicine, Women and Infants Hospital, Providence, USA
| |
Collapse
|