Diagnosis Of Wernicke's Encephalopathy Research Articles

Wernicke’s encephalopathy in a patient with nephrotic syndrome

We report on a 6-year-old boy with nephrotic syndrome (NS) who developed Wernicke's encephalopathy (WE) concomitantly with posterior reversible encephalopathy syndrome (PRES). In this case, the recurrence of encephalopathy with different causes made his clinical picture complex, and the follow-up findings of magnetic resonance imaging (MRI) were critically useful for the adequate diagnosis and timely management of the patient. This case suggests the need to consider WE as a possible serious complication in patients with NS, and also emphasizes the usefulness of MRI in the diagnosis of WE, especially in pediatric cases with complex clinical symptoms.

Wernicke's encephalopathy: 'plus ca change, plus c'est la meme chose'

To develop clinical guidelines to identify individuals who misuse alcohol and are at risk of developing Wernicke's Encephalopathy (WE). Non-systematic literature review of studies which includes a careful clinical record of the development of signs and symptoms of thiamine deficiency and in which the diagnosis of WE has been confirmed at autopsy. The review of the clinical findings in cases of WE, diagnosed at autopsy, shows a consistent pattern of signs and symptoms. The pattern appears to be similar regardless of whether the thiamine deficiency is related to nutritional problems alone or associated with alcohol misuse. The assessment of the degree of thiamine deficiency and the diagnosis of WE remain a clinical evaluation, and guidelines are suggested to help the clinician. Since neurotoxicity due to the metabolism of excessive alcohol in patients with chronic and severe alcohol dependence may be an important factor in determining long-term outcome of treatment, this must form part of the overall evaluation.

Clinical Characteristics and MR Imaging Features of Nonalcoholic Wernicke Encephalopathy

Nonalcoholic Wernicke encephalopathy (WE) is prone to be underestimated in clinical practice. The purpose of this study was to improve its awareness and early accurate diagnosis. We conducted a retrospective review of the cases of 12 patients with nonalcoholic WE, consisting of clinical characteristics and MR imaging features as well as follow-up after administration of thiamine. Patients with mild coma or lethargy (7/12) exhibited typical MR features of symmetric brain paraventricular damage. Patients without disturbances of consciousness or who only had drowsiness (3/12) exhibited a lesion of the periaqueductal area only. In addition to typical MR manifestations, symmetric cortical involvement was observed in 2 of 12 patients with deep coma. Gadolinium enhancement of the mammillary bodies was observed in 2 of 3 patients. No atrophy of the mammillary bodies and cerebellar vermis was found in any patients. Of 10 patients without deep coma and cortical damage, 2 missed the follow-up and 8, who recovered clinically, also showed accordant resolution of abnormal hyperintense signal intensity on T2-weighted and fluid-attenuated inversion recovery images within 2 weeks to 1 year after thiamine supplementation. Two patients with deep coma and cortical damage showed a poor prognosis:1 patient died 15 days after being diagnosed with WE, and the other entered a persistent vegetative state during a follow-up of 2 years. Typical symmetric damage of the mammillary bodies and brain paraventricular regions may permit a specific diagnosis of nonalcoholic WE. In all patients, no atrophy of the mammillary bodies and cerebellar vermis was found. Cortical involvement in patients with nonalcoholic WE may be indicative of irreversible lesions and a poor prognosis.

Wernicke Encephalopathy: the importance of the diagnosis

Wernicke Encephalopathy (WE) is a severe neurological disease caused by vitamin B1 (thiamine) deficiency, which is potentially treatable if early diagnosed. This is the clinical case of a young female patient, with renal insufficiency on haemodialysis, who has been submitted to an abdominal surgery. After the intervention, there were difficulties on beginning with enteric nutrition. Some days later she developed gait imbalance. A Brain Magnetic Resonance (MR) was performed and disclosed abnormalities suggestive of WE. After treatment with thiamine, the patient became asymptomatic. Brain MR is crucial for the confirmation of the diagnosis and early detection of WE, as the imagiologic pattern is typical and the clinical diagnosis is frequently difficult to obtain.

Visual Loss Due to Wernicke Syndrome Following Gastric Bypass

Purpose: To report a case of Wernicke encephalopathy after gastric bypass surgery resulting in vision loss, ophthalmoplegia, and ataxia, all of which reversed with a single dose of IV thiamine. Methods: Observational case report. Results: A 34-year-old woman presented with decreased vision and intermittent diplopia after gastric bypass surgery. She was found to have bilateral limitation of horizontal gaze, decreased vision with bilateral central scotoma and mild disc edema OU. Her cranial magnetic resonance imaging (MRI) was normal. A presumptive diagnosis of Wernicke encephalopathy was made. The patient was admitted, and a single dose of IV thiamine reversed the ophthalmoplegia and vision loss within 24 hours. Conclusion: Wernicke encephalopathy should be considered in patients with vision loss after gastric bypass surgery. The classic triad of confusion, ataxia, and ophthalmoplegia may not be present and, although uncommon, the findings of optic disc edema and vision loss should not deter the clinician from making the diagnosis. Replacement thiamine if given promptly may rapidly reverse the findings.

Hyperemesis Gravidarum Complicated by Wernicke Encephalopathy: Background, Case Report, and Review of the Literature

Wernicke encephalopathy (WE) is a rare but known complication of severe hyperemesis gravidarum caused by thiamine deficiency. This article presents an unusual case that occurred at our institution and reviews the 48 previously published cases of WE in pregnancy. Considering all the 49 cases, the mean (+/-standard deviation) patients' age was 26.7 +/- 4.9 years, the mean gestational age when WE manifested was 14.3 +/- 3.4 weeks, and the mean duration of vomiting and feeding difficulties was 7.7 +/- 2.8 weeks. Wernicke's classic triad (confusion, ocular abnormalities, and ataxia) manifested in only 46.9% (23 of 49) of the patients. Confusion affected 63.3% (31 of 49) of the patients, ocular signs 95.9% (47 of 49) and symptoms 57.1% (28 of 49), and ataxia 81.6% (40 of 49). Deterioration of consciousness affected 53.1% (26 of 49) of the subjects and memory impairment 61.2% (30 of 49). Complete remission of the disease occurred in only 14 of 49 cases. Symptom resolution required months and permanent impairments were common. The overall pregnancy loss rate, directly (spontaneous fetal loss) and indirectly (planned abortion) attributable to WE, was 47.9% (23 of 49). The diagnosis of WE is clinical and can be rapidly confirmed by magnetic resonance imaging. We emphasize the importance of thiamine supplementation to women with prolonged vomiting in pregnancy, especially before intravenous or parenteral nutrition. We also underline the necessity to promptly replace vitamin B1 when neurologic symptoms and/or signs develop in a patient with hyperemesis gravidarum.

MRI findings of Wernicke encephalopathy revisited due to hunger strike

Background and Purpose The purpose of this study was to determine the characteristic magnetic resonance imaging (MRI) findings among a group of patients who presented with Wernicke encephalopathy (WE) due to the neurological complications of a long-term hunger strike (HS). Methods MRI studies also including the fluid-attenuated inversion recovery (FLAIR) sequence and diffusion-weighted imaging (DWI) of six male patients with WE aged from 25 to 38 years (mean age 31 years) were evaluated. Results In all subjects, T2-weighted sequences, FLAIR and DWI revealed a signal hyperintensity within the posteromedial thalami and surrounding the third ventricle. In particular, on coronal images, the hyperintense areas around the third ventricle showed a suggestive “double wing” configuration. We observed an increased signal on proton-density and T2-weighted images in the mamillary bodies of three patients. Four patients demonstrated additional hyperintensities within the periaqueductal region and/or the tectal plate. At least one lesion area in five of six patients demonstrated contrast enhancement. Conclusion The consistent imaging findings of our study suggest that MRI is a reliable means of diagnosing WE. Acute WE is sometimes underdiagnosed, yet early diagnosis and treatment of WE is crucial in order to avoid persistent brain damage. MRI, including postcontrast T1-weighted imaging, DWI beneath standardized T2-weighted imaging, and FLAIR sequences may prove to be a valuable adjunct to clinical diagnosis and to provide additional information in acute and/or subacute WE.

You Are What You Eat

Wernicke encephalopathy consists of a triad of ophthalmoplegia, ataxia, and altered mental status. It is caused by thiamine deficiency and although it is commonly seen in alcoholics, patients undergoing gastric bypass surgery for morbid obesity could be a new group of patients to watch for. Florid papilloedema and optic neuropathy are not commonly seen but are still consistent with the diagnosis of Wernicke encephalopathy. The most striking feature remains the dramatic recovery after replacement of thiamine.

THIAMINE ADMINISTRATION IN ALCOHOL-DEPENDENT PATIENTS

( Received 28 August 2004; first review notified 12 September 2004; in revised form 28 September 2004; accepted 7 October 2004 ) Thiamine (vitamin B1) is a water-soluble vitamin that is involved in the metabolism of glucose and lipids as well as in the production of glucose-derived neurotransmitters (see Cook et al ., 1998). Its deficiency leads to a variety of neurological and cardiovascular symptoms and signs. Early symptoms may include fatigue, weakness and emotional disturbance, whereas prolonged gradual deficiency may lead to a form of polyneuritis (known as dry beriberi), cardiac failure or peripheral oedema (wet beriberi) (Thomson, 2000). Severe thiamine deficiency (TD) may result in the development of Wernicke's encephalopathy (WE). The classical signs of WE are ocular motility disorders (nystagmus, ophthalmoplegia), ataxia and mental changes (confusion, drowsiness, obtundation, clouding of consciousness, pre-coma and coma), although minor episodes of 'subclinical' encephalopathies are frequent (Reuler et al ., 1985). An appropriate treatment may correct most of these abnormalities; in contrast, the lack of a diagnosis of WE may result in serious consequences (Reuler et al ., 1985 …

Diffusion abnormalities and Wernicke encephalopathy

To the Editor: Doherty et al.1 shed new light on Wernicke encephalopathy (WE) and emphasized important but often-forgotten points on this fatal yet preventable entity. I wish to make a few relevant clinical and pathological points. Their second case illustrates that WE is not seen only in alcoholics but also in other forms of malnutrition (e.g., thiamine deficiency). I would add other circumstances, such as (but not limited to) anorexia nervosa, peritoneal dialysis or hemodialysis,2 and HIV infection,3 in which WE has occurred. In addition, I wish to emphasize that although the ocular signs are considered the hallmark of WE, it is now more recognized that the classic triad is neither consistently nor frequently encountered.4 I believe that every clinician should remember the many clinical circumstances in which WE may develop and that the classical clinical triad is not always present. Further, autopsy studies have shown that WE prevalence in both nonalcoholics and alcoholics far exceeds its recognition in living persons.4 On macroscopic examination, the involved areas are slightly shrunken and show brown discoloration due to hemosiderin deposition, typically but not limited to the mamillary bodies, and the periventricular and periaqueductal lesions often spare a slender strip of subependymal tissue.3 Microscopic sections might be needed to make the diagnosis of WE. Twenty-five percent of cases can have normal mamillary bodies on macroscopic examination, and visible hemorrhages are seen in only 5% of cases.5 # Diffusion abnormalities and Wernicke encephalopathy {#article-title-2} To the Editor: We read with great interest the paper from Doherty et al.,1 describing diffusion-weighted MR findings in two patients with WE. Typical MR alterations were found in both patients, including T2 hyperintensities …

Thiamine-Responsive Acute Neurological Disorders in Nonalcoholic Patients

Wernicke’s encephalopathy (WE) is most commonly associated with alcoholism, although other causes have also been implicated. In the years 1994–1997, 9 patients with no history of alcohol abuse presented with acute signs of ophthalmoplegia or nystagmus and ataxia which resolved within 48 h after intravenous thiamine. There were 7 women and 2 men aged 17–57 (7 below the age of 30). Precipitating events included vomiting 2, drastic weight-reducing diet 2, renal colic in a postpartum woman 1, colonic surgery 2 and chronic hemodialysis 1. In 2 patients there was no obvious precipitating event but their history was suggestive of a genetic predisposition. Mental changes were slight or absent in all patients and all of them made good functional recovery. These cases suggest that the diagnosis of WE should be considered more often in nonalcoholics in various clinical settings.

Prevention and treatment of Wernicke-Korsakoff syndrome.

Wernicke's encephalopathy (WE) is both common and associated with high morbidity and mortality and yet there is evidence that appropriate and effective prophylaxis and treatment are often not given. Effective treatment and prophylaxis may only be achieved by use of parenteral vitamin supplements, since oral supplements are not absorbed in significant amounts. Although there are rare anaphylactoid reactions associated with the use of parenteral thiamine preparations, the risks and consequences of inadequate prophylaxis and treatment, in appropriately targeted groups of patients, are far greater. It is therefore proposed that all in-patient alcohol withdrawal should be covered by prophylactic use of parenteral thiamine, that there should be a low threshold for making a presumptive diagnosis of WE, and that there is a need for guidelines to assist physicians in appropriate management of this common clinical problem.

Wernicke’s encephalopathy associated with hemodialysis: report of two cases and review of the literature

Two rare cases of Wernicke’s encephalopathy (WE) in non-alcoholic patients on hemodialysis (HD) are reported. They presented with the clinical triad of WE (ophthalmoplegia, ataxia and disturbance of consciousness) and intravenous administration of thiamine led to complete elimination of these manifestations. Reduced plasma thiamine levels prior to the administration confirmed the diagnosis of WE. Interestingly, a reduction in plasma thiamine levels by about half was seen in one of the patients on HD, suggesting that thiamine, a water-soluble vitamin, can be depleted with HD. In the literature, nine HD-dependent patients have been reported to develop WE, seven of whom were diagnosed postmortem. Their premortem diagnoses included uremic encephalopathy, dysequilibrium syndrome and dialysis dementia, which can often complicate HD and present symptoms similar to those of WE. We therefore emphasize that WE, even though a rare complication, should be suspected in all patients on HD who present with at least one of the clinical triad of WE.

Diencephalic and cerebellar pathology in alcoholic and nonalcoholic patients with end-stage liver disease

Formalin-fixed sections from the brains of 36 patients (30 alcoholic and 6 nonalcoholic) with autopsy-proven cirrhosis who died while in a hepatic coma were stained with hematoxylin and eosin, and examined for the presence of diencephalic, cerebellar, pontine, and basal ganglia lesions. Significant neuropathology was identified in 23 of 36 cases consisting of mammillary body and thalamic lesions characteristic of Wernicke encephalopathy (WE) (9 cases, all alcoholic patients) and cerebellar degeneration (20 cases, 17 alcoholic and 3 nonalcoholic patients). Clinical diagnosis of WE had been entertained during life in only 2 of these patients. All cases, alcoholic and nonalcoholic, manifested mild to severe Alzheimer's type II astrocytosis. No cases of central pontine myelinolysis nor acquired (non-Wilsonian) hepatocerebral degeneration were found. These findings show that the brains of a high proportion of cirrhotic patients with end-stage liver disease manifest concomitant unsuspected diencephalic and cerebellar pathology. The high incidence of WE underscores the need for early sustained treatment of alcoholic cirrhotic patients with vitamin B1. Evaluation of the neurological sequelae of liver transplantation, particularly of alcoholic patients with end-stage liver disease, may require a careful neurological and radiological assessment both before and after surgery. (Hepatology 1997 Oct;26(4):837-41)

Diencephalic and cerebellar pathology in alcoholic and nonalcoholic patients with end-stage liver disease.

Formalin-fixed sections from the brains of 36 patients (30 alcoholic and 6 nonalcoholic) with autopsy-proven cirrhosis who died while in a hepatic coma were stained with hematoxylin and eosin, and examined for the presence of diencephalic, cerebellar, pontine, and basal ganglia lesions. Significant neuropathology was identified in 23 of 36 cases consisting of mammillary body and thalamic lesions characteristic of Wernicke encephalopathy (WE) (9 cases, all alcoholic patients) and cerebellar degeneration (20 cases, 17 alcoholic and 3 nonalcoholic patients). Clinical diagnosis of WE had been entertained during life in only 2 of these patients. All cases, alcoholic and nonalcoholic, manifested mild to severe Alzheimer's type II astrocytosis. No cases of central pontine myelinolysis nor acquired (non-Wilsonian) hepatocerebral degeneration were found. These findings show that the brains of a high proportion of cirrhotic patients with end-stage liver disease manifest concomitant unsuspected diencephalic and cerebellar pathology. The high incidence of WE underscores the need for early sustained treatment of alcoholic cirrhotic patients with vitamin B1. Evaluation of the neurological sequelae of liver transplantation, particularly of alcoholic patients with end-stage liver disease, may require a careful neurological and radiological assessment both before and after surgery.

Esiste impregnazione contrastografica nell'encefalopatia di Wernicke in fase acuta? Descrizione di 3 casi e revisione della letteratura

MR imaging enabled diagnosis of Wernicke's encephalopathy by showing symmetric areas of increased signal intensity on proton density and T2 weighted images in the periaqueductal gray matter, mammillary bodies, medial thalami and pericentral and interhemispheric cerebral cortex in three non-alcoholic patients who were examined within 3 days of admission for acute confusional state associated with ophthalmoparesis and ataxia. In two patients the initial examination was completed with intravenous contrast administration and no lesion enhancement was seen. In the third patient computed tomography 10 days after the initial MR imaging study showed marked contrast enhancement of the lesions. Our data and a review of the literature indicate that lesion contrast enhancement presumably reflecting disruption of the blood brain barrier may be lacking in the early phases of Wernicke's encephalopathy. Acquisition and careful evaluation of long TR images is crucial to the diagnosis of Wernickes encephalopathy with MR imaging in the acute phase.

Wernicke Encephalopathy

A follow-up study by CT and MR of a case of Wernicke encephalopathy is reported. In the early stages, CT and MR revealed a characteristic topographical distribution of lesions in the midbrain, pons, and thalami. In the later stages, MR showed atrophy of the mamillary bodies and midbrain tegmentum and dilatation of the third ventricle. Both CT and MR are useful tools for the diagnosis of Wernicke encephalopathy in the early stages.

National Maternity Report: Correction

<h3>Objective</h3> To explain (1) why an initial upbeat nystagmus (UBN) converts to a permanent downbeat nystagmus (DBN) in Wernicke encephalopathy (WE) and (2) why convergence and certain vestibular provocative maneuvers may transiently switch UBN to DBN. <h3>Methods</h3> Following a literature review and study of our 2 patients, we develop hypotheses for the unusual patterns of vertical nystagmus in WE. <h3>Results</h3> Our overarching hypothesis is that there is a selective vulnerability and a selective recovery from thiamine deficiency of neurons within brainstem gaze-holding networks. Furthermore, since the circuits affected in WE are commonly paraventricular, especially medially, just under the floor of the fourth ventricle where lie structures important for control of vertical gaze, we suggest the patterns of involvement in WE also reflect a breakdown in vulnerable areas of the blood–brain barrier. Many of the initial deficits of our patients improved over time, but their DBN did not. Irreversible changes in paramedian tract neurons, which project to the cerebellar flocculus, may be the cause. Here we suggest that conversion of UBN to permanent DBN points to thiamine deficiency and may argue for a chronic, nonprogressive DBN/truncal ataxia syndrome. Finally, we posit that the transient switch of UBN to DBN reflects abnormal processing of otolith information about linear acceleration, and often points to a diagnosis of WE. <h3>Conclusion</h3> Recognizing the unusual patterns of transient switching and then permanent conversion of UBN to DBN in WE is vital since long-term disability from WE may be prevented by timely, parenteral high-dose thiamine.