Typical and atypical MRI abnormalities in Wernicke's encephalopathy: Correlation with blood vitamin B1 levels.

PURPOSE: Clinical features of Wernicke's encephalopathy (WE) confirmed strictly through the low blood vitamin B1 (VB1) levels are limited. This study aimed to analyse magnetic resonance imaging (MRI) findings, and clinical characteristics, in patients with WE who have confirmed low blood VB1 levels. METHODS: Clinical and laboratory records of 12 consecutive patients with WE admitted to our hospital during the past 11 years were reviewed. The WE diagnosis was confirmed based on low blood VB1 levels and the presence of at least one of the classical triad. RESULTS: Ophthalmoplegia and nystagmus were recorded in 75% and 50% of the patients, respectively. Eleven of 12 patients presented with consciousness disturbance/memory loss. All patients experienced gait disturbances. Eight of the 12 patients exhibited MRI abnormalities at typical sites (the dorsal midbrain [n = 7], medial thalamus [n = 6], mammillary bodies [n = 5], and dorsal pons [n = 5]). Of the 12 patients, six showed abnormalities at atypical sites (the splenium of the corpus callosum [n = 4], fornix [n = 3], cerebral cortex [n = 2], cerebellar vermis [n = 2], and dorsal medulla [n = 1]). Patients with positive MRI abnormalities had significantly lower blood VB1 levels than those without abnormalities (9.5 vs. 16.0 ng/mL). CONCLUSIONS: In cases of confirmed WE with low blood VB1 levels, the corpus callosum, fornix, and cerebral cortex were more frequently involved than in previous studies. MRI abnormalities at both typical and atypical sites were correlated with low blood VB1 levels in WE, suggesting that lower blood VB1 levels are associated with more severe brain damage in patients with WE.

Comprehensive review of Wernicke encephalopathy: pathophysiology, clinical symptoms and imaging findings.

Wernicke's encephalopathy (WE) is a severe and life-threatening illness resulting from vitamin B1 (thiamine) deficiency. The prevalence of WE has been estimated from 0.4 to 2.8%. If not treated properly, severe neurologic disorders such as Korsakoff psychosis and even death may occur. The classical triad of clinical symptoms (abnormal mental state, ataxia, and ophthalmoplegia) is found in only 16-33% of patients on initial examination. The originally described underlying condition of WE is alcoholism, but it accounts for about 50% of causes of WE. Nonalcoholic patients are also affected by WE and likely to present symptoms and radiological imaging findings different from patients with alcoholism, which further complicates the diagnosis of WE. Being familiar with predisposing causes, symptoms and radiological imaging findings of WE is important for radiologists and clinicians when making the diagnosis to start immediate treatment. This review discusses pathophysiologies, underlying causes, clinical symptoms, imaging findings and their mimics.

Subclinical thiamine deficiency identified by preoperative evaluation in an ovarian cancer patient: Diagnosis and the need for preoperative thiamine measurement.

OBJECTIVE: Although thiamine deficiency (TD) and Wernicke encephalopathy (WE) are not rare in cancer patients, the cases reported to date developed TD and/or WE after treatment had started. METHOD: From a series of cancer patients, we report a patient diagnosed with TD without the typical clinical symptoms of WE at the preoperative psychiatric examination. RESULT: A 43-year-old woman with ovarian cancer was referred by her oncologist to the psycho-oncology outpatient clinic for preoperative psychiatric evaluation. Her tumor had been growing rapidly before the referral. Although she did not develop delirium, cerebellar signs, or eye symptoms, we suspected she might have developed TD because of her 2-month loss of appetite as the storage capacity of thiamine in the body is approximately 18 days. The diagnosis of TD was supported by abnormally low serum thiamine levels. SIGNIFICANCE OF RESULTS: Cancer therapists need to be aware that thiamine deficiency may occur even before the start of cancer treatment. In cases with a loss of appetite of more than 2 weeks' duration, in particular, thiamine deficiency should be considered if the tumor is rapidly increasing, regardless of the presence or absence of delirium.

Progressive supranuclear palsy responding to intravenous thiamine: superimposed Wernicke's encephalopathy?

Progressive supranuclear palsy (PSP) may be a risk factor for thiamine deficiency. The classic symptoms of PSP (postural instability, supranuclear vertical gaze palsy and dementia) overlap with the clinical triad of Wernicke's encephalopathy (cognitive impairment, gait problems and ocular abnormality). Therefore, superimposed thiamine deficiency in patients with PSP may aggravate the pre-existing symptoms of PSP. Here, we are reporting a 64-year-old woman having supranuclear ocular palsy, gait instability and dementia for the past 2-3 years. The patient fulfilled the diagnostic criteria of PSP. In parallel, she fulfilled the Caine's criteria of Wernicke's encephalopathy. Her serum thiamine level was low. Supplementation of thiamine led to marked improvement in the symptoms which had been present for many years. These symptoms were originally presumed to be due to PSP. This case highlights the needs to identify superimposed thiamine deficiency in patients with PSP.

[Thiamine in patients with alcohol use disorder and Wernicke's encephalopathy]. / Thiamine bij een alcoholstoornis en Wernicke-encefalopathie.

- Patients with alcohol use disorder frequently have a thiamine deficiency.- A potential life-threatening complication of thiamine deficiency is Wernicke's encephalopathy.- Since it is clinically difficult to recognize Wernicke's encephalopathy, this condition is often treated inadequately. - Early supplementation of thiamine is important to avoid irreversible neurological damage. - There are differences between the Dutch guidelines regarding the supplementation of thiamine for the treatment of alcoholic use disorder, and those for Wernicke's encephalopathy. - There are no solid evidence-based recommendations about the best dosage, route of administration and duration of thiamine supplementation for the treatment of alcohol use disorder and Wernicke's encephalopathy. - Based on the pharmacokinetic properties of thiamine, it is more appropriate to give patients with alcohol use disorder 25 mg four times a day rather than 50 mg twice a day. - Patients at high risk of Wernicke's encephalopathy should immediately receive an intravenous or intramuscular dose of thiamine; patients with suspected Wernicke's encephalopathy should preferably receive an intravenous dose.- Reports of anaphylactic reaction to parenteral administration of thiamine are rare and are not a reason to refrain from parenteral treatment.

Wernicke encephalopathy in a patient after liver transplantation: A case report.

Wernicke encephalopathy (WE) is an acute neurological disorder resulting from vitamin B1 deficiency, which is common in chronic alcoholism and is rare in acute liver failure. So far, there are 2 cases of WE reported after liver transplantation. Here, we report a case of a 45-year-old nonalcoholic male patient who developed psychiatric and neurological disturbance 15 d after receiving orthotopic liver transplantation because of hepatitis B-related cirrhosis and portal hypertension. Brain magnetic resonance imaging (MRI) showed symmetric high-signal intensities in the periaqueductal area. The patient was diagnosed with WE and given intravenous high-dose vitamin B1 immediately. His neurological disturbance resolved in 7 d after receiving the vitamin B1. Brain MRI after 5 mo showed nearly complete recovery. Most WE cases may be misdiagnosed in patients after liver transplantation, and we should pay more attention to its onset.

Cerebral vasospasm and wernicke encephalopathy secondary to adult cyclic vomiting syndrome: the role of magnesium.

BACKGROUND: Magnesium has a regulatory role in the excitability of cell membranes, and is also a cofactor in the phosphorylation of thiamine. Hypomagnesemia has been associated with coronary vasospasm, but its role in cerebrovascular pathology is controversial, and cerebral vasospasm exclusively attributable to hypomagnesemia has not been reported in humans. CASE PRESENTATION: We report the case of a 51-year-old man in whom uncontrollable vomiting, treatment with omeprazole and thiazide, and renal impairment lead to a severe hypomagnesemia (magnesium below the level of detection in blood tests), which secondarily caused Wernicke's encephalopathy and vasospasm in multiple cerebral arteries (seen with cerebral angiography and CT angiography) that presented with a complete right hemisphere neurological deficit. These disturbances completely resolved when magnesium levels were normalized and subsequent neuroimaging tests confirmed the resolution of angiographic changes. CONCLUSION: Our case suggests that hypomagnesemia should be considered in the differential diagnosis of patients with neurological symptoms and predisposing causes.

Challenges in Diagnosis and Treatment of Wernicke Encephalopathy: Report of 2 Cases.

BACKGROUND: Wernicke encephalopathy (WE) is a medical emergency caused by thiamine deficiency, characterized by cerebellar ataxia, ophthalmoplegia, and cognitive disturbances that may progress to Korsakoff amnesia. We describe 2 patients with WE who needed high-dose and long-term treatment with thiamine to obtain neurological improvement and recovery. CASE DESCRIPTION: The first patient was a woman diagnosed with non-Hodgkin lymphoma. After a gastrointestinal infection, she developed depression, memory loss, disorientation, behavioral changes, and ataxic paraplegia. Brain magnetic resonance imaging (MRI) showed bilateral alterations in thalamic, frontal, and periaqueductal regions, suggestive of WE. The second patient was a man who lost 10 kg after surgical gastrectomy; he developed diplopia, ophthalmoplegia, cerebellar ataxia, lower limb paresthesias, and amnesia. A brain MRI demonstrated contrast enhancement of mammillary bodies, compatible with WE. OUTCOME: The patients were treated with intramuscular (IM) thiamine (1200 mg/d for 2 months and 900 mg/d for a month, respectively) with gradual cognitive and behavioral improvement and brain MRI normalization, while ataxia and oculomotion improved in following months. In both patients, thiamine was gradually reduced to IM 200 mg/d and continued for a year, without clinical relapses. CONCLUSIONS: There is no consensus about dosage, frequency, route, and duration of thiamine administration in WE treatment. Based on our cases, we recommend treating patients with WE with higher doses of IM thiamine for a longer time than suggested (900-1200 mg/d for 1-2 months, in our cases) and to gradually reduce dosage after clinical and radiological improvement, maintaining IM 200 mg/d dosage for at least 1 year.

Neuromyelitis optica spectrum disorders may be misdiagnosed as Wernicke's encephalopathy.

PURPOSE: To raise doctors' attention to the differential diagnosis of neuromyelitis optica spectrum disorders (NMOSD) and Wernicke's encephalopathy (WE). PATIENTS AND METHODS: We extensively reviewed the medical records of 136 patients who had visited our hospital since 2008 and were suspected of having central nervous system demyelinating diseases. Four of those patients had somnolence, electrolyte imbalance and brain lesions around the third ventricle and were included in the study. We tested the serum of the four patients for the presence of aquaporin-4 (AQP4) M23 antibody. RESULTS: All the four patients had positive AQP4 antibody in their serum. Two of the patients were misdiagnosed as WE before AQP4 antibody detection occurred. CONCLUSIONS: NMOSD and WE have similar brain lesion locations, histopathological changes and clinical manifestations. It is important to distinguish NMOSD from WE by detecting AQP4 antibody in serum or cerebral spinal fluid. Vitamin B1 should also be administered to the patients who have a history of thiamine deficiency.

Malnutrition-induced Wernicke's encephalopathy following a water-only fasting diet.

BACKGROUND: Wernicke's encephalopathy is a critical condition of neurological dysfunction resulting from a deficiency in thiamine. Chronic alcoholism is recognized as the most common cause of Wernicke's encephalopathy, but other causes, including fasting/starvation and malnutrition, have been documented within the scientific literature. These causes may not be readily recognized by healthcare professionals and may lead to Wernicke's encephalopathy being overlooked as a diagnosis when a nonalcoholic patient presents with classic signs and symptoms of the disorder. MATERIALS AND METHODS: A narrative review of thiamine and its relationship to the development, diagnosis, and treatment of Wernicke's encephalopathy is presented based on a review of evidence-based guidelines and published research. To heighten awareness of the development of Wernicke's encephalopathy in fasted/starved and malnourished patients and to contribute to the scientific body of knowledge for the identification and management of Wernicke's encephalopathy in these patients, the clinical course and treatment of an adult woman who developed Wernicke's encephalopathy following a 40-day water-only fasting diet is outlined. RESULTS: Clinical suspicion was required to identify the patient's condition and initiate immediate intervention through parenteral thiamine administration. Oral thiamine supplementation of 100 to 800 mg per day for 6 months was required to aid recovery. OUTCOMES: The patient's clinical course and response to treatment illustrate the necessity for clinical awareness and suspicion of Wernicke's encephalopathy among healthcare professionals, timely and adequate parenteral thiamine administration, and oral thiamine supplementation at therapeutic doses to correct the nutrient deficiency, halt the progression of Wernicke's encephalopathy, and promote recovery.

Prevention of delirium in trauma patients: are we giving thiamine prophylaxis a fair chance?

BACKGROUND: Delirium is associated with increased morbidity and mortality in injured patients. Wernicke encephalopathy (WE) is delirium linked to malnutrition and chronic alcoholism. It is prevented with administration of thiamine. Our primary goal was to evaluate current blood alcohol level (BAL) testing and thiamine prophylaxis in severely injured patients. METHODS: We retrospectively reviewed the cases of 1000 consecutive severely injured patients admitted to hospital between Mar. 1, 2009, and Dec. 31, 2009. We used the patients' medical records and the Alberta Trauma Registry. RESULTS: Among 1000 patients (mean age 48 yr, male sex 70%, mean injury severity score 23, mortality 10%), 627 underwent BAL testing at admission; 221 (35%) had a BAL greater than 0 mmol/L, and 189 (30%) had a BAL above the legal limit of 17.4 mmol/L. The mean positive BAL was 41.9 mmol/L. More than 4% had a known history of alcohol abuse. More patients were assaulted (20% v. 9%) or hit by motor vehicles (10% v. 6%) when intoxicated (both p < 0.05). Most injuries occurred after falls (37%) and motor vehicle collisions (33%). Overall, 17% of patients received thiamine prophylaxis. Of the 221 patients with elevated BAL, 44% received thiamine prophylaxis. Of those with a history of alcohol abuse, 77% received thiamine prophylaxis. CONCLUSION: Despite the strong link between alcohol abuse, trauma and WE, more than one-third of patients were not screened for alcohol use. Furthermore, a minority of intoxicated patients received adequate prophylaxis against WE. Given the low risk and cost of BAL testing and thiamine prophylaxis and the high cost of delirium, standard protocols for prophylaxis are essential.

CONTEXTE: Le délire est associé à une morbidité et une mortalité accrues chez les traumatisés. L'encéphalopathie de Wernicke (EW) est un délire associé à la malnutrition et à l'alcoolisme chronique que l'on peut prévenir en administrant de la thiamine. Notre objectif principal était d'évaluer le recours actuel aux tests d'alcoolémie et au traitement prophylactique à la thiamine chez les grands traumatisés. MÉTHODES: Nous avons passé en revue de manière rétrospective 1000 cas consécutifs d'hospitalisation pour traumatismes graves entre le 1er mars 2009 et le 31 décembre 2009. Nous avons utilisé les dossiers médicaux des patients et le Registre des traumatismes de l'Alberta. RÉSULTATS: Sur 1000 patients (âge moyen 48 ans, sexe masculin 70 %, indice moyen de gravité des traumatismes 23, mortalité 10 %), 627 ont subi un test d'alcoolémie à leur admission; 221 (35 %) présentaient un taux d'alcoolémie supérieur à 0 mmol/L et 189 (30 %) avaient un taux d'alcoolémie au-dessus de la limite permise de 17,4 mmol/L. Le taux moyen des tests d'alcoolémie positifs était de 41,9 mmol/L. Plus de 4 % de ces cas avaient des antécédents d'alcoolisme. Les patients qui étaient sous l'effet de l'alcool ont davantage été victimes d'agressions (20 % c. 9 %) ou d'accidents impliquant un véhicule (10 % c. 6 %; tous deux p < 0,05). La majorité des traumatismes ont été causés par des chutes (37 %) ou des accidents de la route (33 %). Dans l'ensemble, 17 % des patients ont reçu un traitement prophylactique à la thiamine. Parmi les 221 patients qui présentaient un taux d'alcoolémie élevé, 44 % ont reçu de la thiamine en prophylaxie. Parmi ceux qui présentaient des antécédents d'abus d'alcool, 77 % ont reçu un traitement prophylactique à la thiamine. CONCLUSION: Malgré le lien étroit entre abus d'alcool, traumatismes et EW, plus du tiers des patients n'ont subi aucun test d'alcoolémie. En outre, seule une minorité de patients intoxiqués ont reçu une prophylaxie adéquate contre l'EW. Compte tenu des risques faibles et des coûts peu élevés du test d'alcoolémie et de la prophylaxie par thiamine et des coûts élevés occasionnés par les épisodes de délire, il est essentiel d'instaurer des protocoles standard de prophylaxie.

Dementia complicated with Takotsubo cardiomyopathy associated with unconsciousness induced by Wernicke's encephalopathy.

An 85-year-old woman who had been living alone and eating an unbalanced diet suddenly entered a neighbour's house. Her house was hot and humid due to lack of air conditioning caused by a loss of electrical power. After arrival, the patient exhibited disorientation, paresis of the right upper extremity, a tendency towards right conjugated deviation and perseveration. Electrocardiogram showed ST segment elevation and prolongation of the QT interval. Echocardiography suggested Takotsubo cardiomyopathy. The cardiac wall motion and neurological abnormalities improved after admission. The serum thiamine level was found to be low, which was compatible with a diagnosis of Wernicke's encephalopathy. Hasegawa dementia score was 10 points and the patient was suspected to have frontotemporal dementia. She was transferred to a nursing home with continuing dementia. In this case, psychological stress trigged by poor living circumstances induced by dementia and Wernicke's encephalopathy may result in the occurrence of Takotsubo cardiomyopathy.