This study was undertaken to analyze and compare findings on mr imaging and neurologic symptoms at clinical presentations of patients with wernicke encephalopathy with and without a. We describe a rare manifestation of optic discs swelling in a pregnant woman that has caused a diagnostic dilemma. Reversible pulvinar sign in wernickes encephalopathy ncbi. We present a case report of motor and cognitive disorders in a 36yearold woman with a history of twelve years of heavy alcohol abuse. Reversible pulvinar sign in wernickes encephalopathy. Wernickes encephalopathy mimicking variant creutzfeldt. Wernicke s encephalopathy mimicking variant creutzfeldtjakob disease. It can occur in the context of inadequate dietary intake, and is also seen in a number of medical conditions associated with excessive. It can lead to a closely related condition, the more chronic korsakoff dementia syndrome. The pulvinar sign in a case of paraneoplastic limbic. The pulvinar sign on magnetic resonance imaging in variant creutzfeldtjakob. Join our foundation trust today and support our hospitals sign up today and stay up to date with the latest news and events. This paper reports a 59 year old woman with paraneoplastic limbic encephalitis associated with diffuse large b cell lymphoma.
Request pdf on apr 1, 2010, christian schmidt and others published pulvinar sign in wernicke s encephalopathy find, read and cite all the research you need on researchgate. The purpose of this study was to present the neuroimaging findings and differential diagnosis of bilateral thalamic lesions. Other symptoms may include movement problems, changes in mood, or changes in personality. The spectrum of patients affected continues to increase with changing medical practice.
Pulvinar sign, reversible, variant cjd, wernicke s encephalopathy introduction youngonset dementia poses a challenge to physicians as many dementias are untreatable, while some are treatable, and the physician does not want to miss a dementia with a treatable cause. Thiamine treatment reversed neurological complications. Mri is the preferred imaging method employed for the diagnosis of wernicke encephalopathy. The pulvinar sign, which is relatively sensitive and specific for vcjd, did not exclude other disorders that rarely have similar features, including wernicke s encephalopathy and inflammatory limbic encephalitis 2729. Pulvinar sign in wernicke s encephalopathy cns spectrums. Her brain magnetic resonance imaging scan showed bilateral posterior thalamic hyperintensities, similar to the pulvinar sign. The pulvinar sign in vcjd is defined as bilateral, symme trical pulvinar high signal. Pulvinar sign on mri images in variant creutzfeldtjakob disease.
Pdf wernickes encephalopathy we is a severe neurological syndrome. The limited differential diagnosis of bilateral thalamic lesions can be further narrowed with knowledge of the specific imaging characteristics of the lesions in combination with the patient history. Conclusion wernicke encephalopathy secondary to thiamine deficiency should be considered as a possible cause of acute mental status changes in patients with acute pancreatitis and malnutrition. Wernicke encephalopathy radiology reference article. A 25year old pregnant female with history of confusion and drowsiness for 02 days was referred by neurophysician for mri brain. Pulvinar sign in wernickes encephalopathy cns spectrums. The condition is part of a larger group of thiamine deficiency disorders, that includes beriberi in all its forms, and alcoholic korsakoff syndrome. The pulvinar sign has a significantly lower incidence in fabry disease than previously described. We describe a case, where a patient presented with psychosis, dementia and mri showing.
The pulvinar sign in vcjd is defined as bilateral, symmetrical pulvinar high signal relative to the signal intensity of other deep grey matter nuclei and cortical grey matter. The neuropsychiatric manifestations are varied but typically include alterations of consciousness, eye mov. Wernicke encephalopathy is a wellknown complication of thiamine deficiency, mostly associated with alcohol use disorder. This study was undertaken to analyze and compare findings on mr imaging and neurologic symptoms at clinical presentations of patients with wernicke encephalopathy with and.
Thiamine is a coenzyme which plays a central role in neurons. It is characterized by changes in consciousness, ocular abnormalities, and ataxia. This short video describes a patient with wernicke encephalopathy we. Request pdf reversible pulvinar sign in wernick es encephalopathy young onset dementia is a challenge. Wernicke s encephalopathy with chorea neuroimaging findings jivago s. Wernicke encephalopathy we, also wernicke s encephalopathy is the presence of neurological symptoms caused by biochemical lesions of the central nervous system after exhaustion of bvitamin reserves, in particular thiamine vitamin b1. Principal symptoms are ophthalmoplegia, ataxia and acute changes in consciousness. Despite our clinical suspicion, results of all the initial laboratory tests were negative. Mri demonstrated t2wflair hyper intensities in medial thalami, periaqueductal areas with variable diffusion restriction, apparent as hyper intense signal on dwi and no signal change on adc mapping that was typically consistent with wernicke s encephalopathy.
Pulvinar sign in wernickes encephalopathy request pdf. Due to the close relationship between these two disorders, people with either are usually diagnosed with wks as a single syndrome. Prion diseases or transmissible spongiform encephalopathies tses. Wernicke korsakoff syndrome is one name for two conditions that often happen together wernicke encephalopathy and korsakoff syndrome. Wernicke encephalopathy is an acute neuropsychiatric emergency due to thiamine deficiency. Pdf mr imaging findings in alcoholic and nonalcoholic acute. Wernicke encephalopathy is a potentially lifethreatening condition of abnormal carbohydrate metabolism occurring on the grounds of thiamine deficiency. Wernicke encephalopathy guidelines bmj best practice. Wernicke encephalopathy prevalence comes mainly from autopsy studies with rates ranging between 1% and 3%, and this result shows that the diagnosis of wernicke encephalopathy is often made only postmortem and less than 20% of the patients obtain the right diagnosis during life. Posterior reversible encephalopathy syndrome and wernicke. Neuroimaging findings in acute wernickes encephalopathy ajr. Neuroimaging findings in acute wernickes encephalopathy. Wernickes encephalopathy is an acute neurological syndrome resulting from.
Wernicke s encephalopathy we is an acute neurologic disorder resulting from thiamine vitamin b1 deficiency. The pulvinar sign in a case of paraneoplastic limbic encephalitis. Wernickes encephalopathy we is a severe neurological syndrome caused by. Signal changes in pulvinar on mri were noted initially in case reports of patients with variant creutzfeldtjakob disease cjd and subsequently consistent correlation of this finding prompted the recognition of this abnormality on mri as pulvinar sign. Alcohol abuse, which impairs thiamine absorption, is often the underlying cause. In some diseases, thalamic involvement is typical and sometimes isolated, while in other diseases thalamic lesions are observed only occasionally often in the presence of other typical extrathalamic lesions. Wernicke encephalopathycausessymptomstreatmentprognosis. Hepatic encephalopathy he is an altered level of consciousness as a result of liver failure. Wernicke encephalopathy references bmj best practice. Wernicke s encephalopathy, neurosarcoidosis, hypothyroidism, nonconvulsive status epilepticus, hypoxic encephalopathy, a toxicmetabolic syndrome or a functional disorder.
Her symptoms included progressive psychiatric disturbance and resembled the initial symptoms of variant creutzfeldtjakob. Wernicke encephalopathy we is an acute syndrome requiring emergent treatment to prevent death and neurologic morbidity. Many doctors think of them as different stages of the same. Wernicke korsakoff syndrome wks is the combined presence of wernicke encephalopathy we and alcoholic korsakoff syndrome. Pulvinar sign in wernickes encephalopathy volume 15 issue 4 christian schmidt, steffen plickert, david summers, inga zerr. Photophobia and bilateral pulvinar involvement in non. Caused by the acute deficiency of thiamine in a susceptible host. Eye motor abnormalities in wernicke encephalopathy, case 1.
Wernicke s encephalopathy following severe hyperemesis gravidarum is an uncommon clinical entity. Thiamine for dementia in a young woman case 23 case studies in. The focus in this presentation is the eye motor abnormalities. Mr imaging findings in alcoholic and nonalcoholic acute. Wernicke s encephalopathy was established on the basis of low serum thiamine on admission and eventual clinical improvement on highdose intravenous thiamine replacement, despite initial failure to respond to the standard dose of 100 mg daily intramuscularly. Wernicke s encephalopathy we is a clinical syndrome that results from thiamine vitamin b1 deficiency.
Role of thiamine introduction w ernicke s encephalopathy we is an acute neuropsychiatric disorder which arises as the result of an inadequate supply of thiamine to the brain. In 1881, carl wernicke 1 described a polioencephalopathia haemorrhagica superior in 3 alcoholic patients with gray matter hemorrhages in the mammillary bodies contrast enhancement in the mammillary bodies, an important imaging sign of wernicke encephalopathy we, suggests a disrupted bloodbrain barrier, which may be the cause for the microbleeds. Shajis mri centre, mini bypass road, calicut, kerala, india. Wernickes encephalopathy we is a severe neurological syndrome. Redefining the pulvinar sign in fabry disease american. It is a combination of wernicke s encephalopathy which consists of the triad of ophthalmoplegia, ataxia, and confusion and if left untreated, may result in. Wernicke encephalopathy is a neuropsychiatric condition associated with thiamine deficiency, classically presenting as a combination of acute onset ataxia, ophthalmoplegia, nystagmus and confusion.
The clinical findings that characterize the syndrome are nystagmus, ophthalmoplegia, mental status changes and cerebellar dysfunction. Pulvinar nucleus is the largest nucleus in the thalamus and is situated at the caudal extremity of the thalamus. Wernicke encephalopathy presenting in a patient with. Variant cjd showing pulvinar sign on the flair sequence. Individuals who have wernicke encephalopathy tend to develop bleeding in the lower half of the brain which includes the thalamus and the hypothalamus which control the vision, motion control, coordination, and balance. Pulvinar sign in acute disseminated encephalomyelitis. This finding, coupled with a lack of significant differences in quantitative mr imaging, allows hypothesizing that selective involvement of the pulvinar is a rare neuroradiologic sign. Thiamine in the treatment of wernicke encephalopathy in. The patient died and autopsy findings showed microhemorrhages in the thalami and also in midbrain tectum figure 3e. The pulvinar sign on magnetic resonance imaging in variant. This article reports the case of a 68yearold patient with antihu antibodies paraneoplastic encephalitis. With high index of suspicion of clinical manifestations and radiological evidences, a clinical diagnosis of wernicke s encephalopathy was made. Upadhyay j, burstein r, borsook d 2012 direct optic nerve pulvinar connections defined by diffusion tractography in humans.
Wernicke encephalopathy, also referred as wernicke korsakoff syndrome, is a form of thiamine vitamin b 1 deficiency, and is typically seen in alcoholics on imaging, it is commonly seen on mri as areas of symmetrical increased t2flair signal involving the mammillary bodies, dorsomedial thalami, tectal plate, periaqueductal area, and around the third ventricle. Recent us case of variant creutzfeldtjakob diseaseglobal. Variant creutzfeldtjakob disease and bovine spongiform. Korsakoff syndrome ks refers to a chronic neurologic condition that usually occurs as a consequence of we. Thalamic lesions are seen in a multitude of disorders including vascular diseases, metabolic disorders, inflammatory diseases, trauma, tumours, and infections. We was first described by carl wernicke in 1881 as superior acute hemorrhagic poliencephalitis in two men with alcoholism and in a woman affected by pyloric stenosis, whereas the association of we with thiamine deficiency was first suspected in the 1940s. Differential diagnosis for bilateral abnormalities of the basal. Other common causes of wernicke encephalopathy in non. Reversible pulvinar sign in wernick es encephalopathy biju gopalakrishnan 1, vv ashraf 1, praveen kumar 1, krishna kiran 2 1 malabar institute of medical sciences hospital, calicut, kerala, india 2 dr. Wernicke encephalopathy is a severe neurologic disorder that results from a dietary vitamin b1 deficiency. The clinical manifestations were atypical and the paraclinical workup, notably the magnetic resonance imaging mri showing bilateral posterior thalamic hyperintensities pulvinar sign, misleadingly pointed towards a variant creutzfeldjakob disease. We describe a case, where a patient presented with psychosis, dementia and mri showing pulvinar sign, all of this typical of variant cruetzfelt jacob disease cjd. Know the causes, symptoms, treatment and prognosis of wernicke encephalopathy. Cureus optic discs swelling procrastinates wernickes.