Wernicke encephalopathy: limitations in a laboratory and radiological diagnosis.

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.

Fetal demise and Wernicke-Korsakoff syndrome in a patient with hyperemesis gravidarum: a case report.

BACKGROUND: Wernicke-Korsakoff syndrome is a neuropsychiatric disorder caused by thiamine deficiency composed of two related disorders accounting for an acute presentation and chronic progression. Hyperemesis gravidarum presents a significant risk factor for Wernicke-Korsakoff syndrome as symptoms may rapidly progress in the setting of pregnancy. We present the first-reported case of hyperemesis-gravidarum-associated Wernicke encephalopathy in a patient in the first half of pregnancy in which a missed diagnosis led to septic shock, fetal demise, and eventual profound Korsakoff syndrome. CASE PRESENTATION: We present the case of a 33-year-old primigravid African American woman at 15 weeks gestational age who initially presented at a community emergency department with nausea and vomiting that ultimately progressed to severe hyperemesis-gravidarum-associated Wernicke-Korsakoff syndrome, fetal demise, and septic shock. The patient received a total of 6 weeks of high-dose parenteral thiamine. Magnetic resonance imaging of the head and formal neuropsychological assessment following treatment plateau confirmed the diagnosis of Wernicke-Korsakoff syndrome. CONCLUSIONS: The multisystem complications seen in severe thiamine deficiency can delay timely administration of high-dose thiamine, particularly in pregnancy, in which the classic triad of Wernicke-Korsakoff syndrome may not raise clinical suspicion due to rapid progression of neurological sequelae in this population. We advise a low threshold for parenteral thiamine repletion in pregnant women with persistent vomiting as hyperemesis gravidarum-induced severe thiamine deficiency can result in Wernicke-Korsakoff syndrome, sepsis, and fetal demise.

Cystic fibrosis associated with Wernicke's encephalopathy in an older adult.

Here we report the first case of an association between cystic fibrosis and Wernicke's encephalopathy. The patient had a history of cystic fibrosis diagnosed in her early 60s associated with pancreatitis and chronic lung disease. She presented with a traumatic hip fracture requiring operative repair. On examination, she was found to have bilateral nystagmus. MRI revealed enhancement of the mammillary bodies. Laboratory results were notable for thiamine deficiency, which in context of the radiographic and physical examination findings, confirmed a diagnosis of Wernicke's encephalopathy. The cause of her low thiamine was thought to be poor dietary intake, weight loss and malabsorption associated with exocrine pancreatic insufficiency in the setting of a history of recurrent pancreatitis. The patient had complete resolution of her symptoms with the initiation of thiamine supplementation and pancreatic enzymes. Although classically associated with fat soluble vitamin deficiencies, there are increasing reports of water-soluble vitamin deficiencies associated with cystic fibrosis.

Fatal Wernicke's Encephalopathy with Cardiovascular Involvement in a Young Psychiatric Patient.

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.

Wernicke encephalopathy with extensive cortical lesions combined with diffuse large B-cell lymphoma.

OBJECTIVE: Wernicke encephalopathy (WE) is an acute or subacute neurologic disorder resulting from thiamine deficiency. A Magnetic Resonance Imaging (MRI) test is useful in addition to the clinical manifestation, which is the main basis for the diagnosis. Typical MRI findings include areas surrounding the aqueduct and third ventricle, as well as those in the medial thalamus, dorsal medulla, tectal plate, and mamillary bodies. We reported a case of WE with extensive cortical lesions. The beneficial effects of thiamine supplementation and low dosage of glucocorticoid did not sustain after discharge. Eventually, we found that the condition he had was brought on by gastric diffuse large B-cell lymphoma. Thiamine supplements combined with glucocorticoids may be a good administration regimen. The etiology of WE is frequently disregarded. In individuals with WE, it is essential to take the underlying illness into account. Malignancy, especially gastrointestinal tract cancer, should be considered. A good administration regimen may include glucocorticoids and thiamine supplements.

Primary Central Nervous System Lymphoma Mimicking Wernicke's Encephalopathy.

Primary central nervous system lymphoma (PCNSL) is a rare disease that can be confused with Wernicke encephalopathy (WE). We have reported here the case of a 31-year-old malnourished man who presented with headache, fever, vomiting, diarrhea, and confusion. His imaging and laboratory findings were indicative of WE. His condition improved after treatment with a high dose of vitamin B1 and intravenous administration of methylprednisolone. However, after continuing to take vitamin B1 for 2 weeks, his symptoms and neuroimaging findings worsened. Increased standardized uptake values of positron emission tomography with 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography (18F-FDG-PET) and interleukin-10 (IL-10) in the cerebrospinal fluid led to the diagnosis of PCNSL. After treatment with methotrexate and calcium leucovorin, the symptoms and neuroimaging abnormalities disappeared at the 6-month follow-up examination. The possibility of PCNSL should be considered if the routine treatment for WE are ineffective. 18F-FDG PET and IL-10 may provide a new method for the early diagnosis of PCNSL.

A Case Report of Nonalcoholic Gayet-Wernicke Encephalopathy: Don't Miss Thiamine.

Gayet-Wernicke encephalopathy (WE) is an acute neurological disorder resulting from deficiency of thiamine, commonly related to chronic abuse of alcohol, but frequently missed or overlooked as a diagnosis when a nonalcoholic patient presents with atypical signs and symptoms of the disease. The diagnosis of the disease is clinical, and confirmation is done by magnetic resonance imaging. We aim to highlight a case of WE in a nonalcoholic postoperative surgical patient receiving total parental nutrition where high-dose intravenous administration of thiamine in time mitigated the symptoms of disease and prevented permanent neurological sequelae. We spotlight the significance of adequate thiamine for postoperative malnourished surgical patients.

Wernicke's encephalopathy due to malnutrition and parenteral nutrition in a patient with cerebral infarction: A case report.

INTRODUCTION: Wernicke's encephalopathy (WE) is a severe neuropsychiatric disorder, which results from a nutritional deficiency of thiamine. The occurrence of WE is rarely reported in patients with cerebral infarction, who often have complications of malnutrition. Cerebral infarction is a neurological disease, patients with cerebral infarction may show symptoms such as disturbance of consciousness and gait instability, which is difficult to differentiate from WE. Thus, early recognition and differential diagnosis of WE are important. We report a rare case of cerebral infarction patient who developed WE due to malnutrition and parenteral nutrition. PATIENT CONCERNS: A 65-year-old woman was admitted to our hospital with cerebral infarction. She had lost 15 kg of weight in the past month or so and was diagnosed with malnutrition. In order to correct malnutrition, parenteral nutrition and intravenous glucose without thiamine were administered. Cognitive dysfunction, laloplegia, sleep rhythm inversion, somnolence and bilateral lower limbs weakness were presented 20 days after admission. DIAGNOSIS: Brain magnetic resonance imaging confirmed the diagnosis of WE. INTERVENTIONS: The patient was given thiamine and nutrition support therapy. OUTCOMES: The patient's cognitive impairment, laloplegia and sleep condition improved within 4 days. Neurological status continued to improve and physical activity recovered gradually within 2 weeks. She received rehabilitation training when her condition was relatively stable, and her muscle strength of limbs and physical function gradually improved. CONCLUSION: Infarction-related malnutrition may result in nutrient deficiency-related neurological complications, such as WE. Thus, it is important to pay close attention to the nutritional status of patients with cerebral infarction. In addition, early recognition and differential diagnosis of WE in patients with infarction-related malnutrition are necessary, early treatment of replete thiamine supplementation and nutrition support therapy can reduce the risk of WE and improve the prognosis.

Recurrent Wernicke's encephalopathy in pregnancy: A case report.

INTRODUCTION: Wernicke's encephalopathy (WE) is an acute neurologic syndrome resulting from a deficiency in thiamine, also known as Vitamin B1. Thiamine stores can be depleted rapidly in patients with severe hyperemesis. Treatment with thiamine typically results in complete resolution of the neurological abnormalities. CASE REPORT: A 15-year-old G2P0010 at 13.2 weeks gestation presented with altered mental status and transaminitis. She had a medical termination in her previous pregnancy following an admission for a similar clinical scenario. She was initially thought to have a postoperative surgical complication due to recent cholecystectomy, but further evaluation revealed thiamine depletion. Magnetic resonance imaging confirmed the diagnosis of WE. Repletion of thiamine and folic acid resulted in rapid clinical improvement. CONCLUSION: WE should be considered in the differential diagnosis of pregnant patients with hyperemesis and altered mental status. A prior history of WE increases the risk of recurrence during pregnancy. Severe hyperemesis during pregnancy increases the risk of thiamine deficiency and WE. Early thiamine supplementation may reduce the risk of WE in patients with a prior clinical history or in patients with severe hyperemesis gravidarum.

An autopsied case of MM1-type sporadic Creutzfeldt-Jakob disease with pathology of Wernicke encephalopathy.

An 83-year-old Japanese man presented with gait disturbance followed by rapidly-progressive cognitive impairment. Magnetic resonance diffusion-weighted images showed extensive hyperintense regions in the cerebral cortex. Four weeks after symptom onset, myoclonus appeared, and the patient developed difficulty swallowing; intravenous peripheral continuous infusions without vitamin supplementation were administered during the last two months of the patient's life. The patient reached the akinetic mutism state and died 12 weeks after symptom onset due to sepsis. The brain weighed 940 g and showed general cerebral atrophy. Extensive spongiform change were observed in the cerebral cortex, striatum, thalamus, and cerebellar cortex, but gliosis was generally mild. Numerous newly-developed hemorrhage foci were observed in the mammillary body, the areas adjacent to the third and fourth ventricles, and the periaqueduct of the midbrain; however, proliferation of capillaries and endothelium and collections of macrophages were relatively inconspicuous. These findings suggested comorbidity with the acute stage of Wernicke encephalopathy (WE). Immunostaining showed extensive diffuse synaptic-type prion protein deposition in the gray matter. According to the neuropathological, genetic, and molecular findings, the present case was finally diagnosed as MM1-type sporadic Creutzfeldt-Jakob disease (CJD) with WE. We should remain alert to the diagnosis of WE when CJD is suspected, and it is necessary to consider the complications of both diseases. This report emphasizes the importance of pathological investigations for the diagnosis of CJD, WE, and the coexistence of both.

Conversion of upbeat to downbeat nystagmus in Wernicke encephalopathy.

OBJECTIVE: 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. METHODS: Following a literature review and study of our 2 patients, we develop hypotheses for the unusual patterns of vertical nystagmus in WE. RESULTS: 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. CONCLUSION: 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.

Non-alcoholic Wernicke's encephalopathy with cortical involvement and polyneuropathy following gastrectomy.

In this study, we present the clinical manifestations, brain magnetic resonance imaging (MRI) and concurrent polyneuropathies in two patients with non-alcoholic Wernicke's encephalopathy (WE) after gastrojejunostomy (Billroth II) anastomosis procedures. These patients developed sub-acute onset of disorientation and disturbance of consciousness following several weeks of poor intake. Peripheral neuropathy of varying severity was noted before and after the onset of WE. Brain MRI of the patients showed cerebellar vermis and symmetric cortical abnormalities in addition to typical WE changes. Electrophysiological studies demonstrated axonal sensorimotor polyneuropathy. Prompt thiamine supplement therapy was initiated and both patients gradually recovered, however mild amnesia was still noted 6 months later. We reviewed non- alcoholic WE with atypical cortical abnormalities in English language literatures and identified 29 more cases. Eight out of 31 (25.8%) patients died during follow-up. Nine patients with gait disturbance or motor paresis had showed hyporeflexia in neurological examinations. In addition to classic triad, seizure was recorded in seven patients. Dietary deprivation is a risk factor for non-alcoholic WE among elderly patients receiving gastrointestinal surgery. The prognosis is good after thiamine supplement therapy. Recognizing the MRI features and predisposing factors in patients who have undergone gastrectomy can aid in the diagnosis and management.

Epileptic seizures as the first symptom of Wernicke's encephalopathy with cerebral cortical lesions.

Wernicke's encephalopathy (WE) is acute metabolic disease of the central nervous system caused by deficiency of thiamine. Typical imaging findings are bilateral and symmetric signal in mammillary bodies, medial thalamus and periaqueductal gray. We present a 45-year-old man diagnosed for WE with two seizures and unconsciousness. The magnetic resonance imaging showed bilateral and symmetrical signal hyper-intensities in the frontal and parietal cortex, in addition to the classical MRI findings of WE. Cortical damage in WE is rare. The patient was improved significantly from unconsciousness to obeying commands and answering questions after 3days by thiamine supplementation. But the muscle strength and conscious state did not improve after 1year. This case report reminds us that we should take into account the possibility of WE when imaging shows cortical damage.

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.

A Rare Complication Developing After Hematopoietic Stem Cell Transplantation: Wernicke's Encephalopathy.

Thiamine is a water-soluble vitamin. Thiamine deficiency can present as a central nervous system disorder known as Wernicke's encephalopathy, which classically manifests as confusion, ataxia, and ophthalmoplegia. Wernicke's encephalopathy has rarely been reported following hematopoietic stem cell transplantation. Herein, we report Wernicke's encephalopathy in a patient with acute myeloid leukemia who had been receiving prolonged total parenteral nutrition after haploidentical allogeneic hematopoietic stem cell transplantation. To the best of our knowledge, this is the first case reported from Turkey in the literature.

Neuroimaging findings in acute Wernicke's encephalopathy: review of the literature.

OBJECTIVE: Wernicke's encephalopathy is an acute neurological syndrome resulting from thiamine (vitamin B1) deficiency. Early recognition is important because timely thiamine supplementation can reverse the clinical features of the disease. The aim of this article is to provide an update on the typical and atypical neuroimaging findings of the acute phase of the disease. CONCLUSION: Wernicke's encephalopathy is characterized by a quite distinct pattern of MR alterations, which include symmetrical alterations in the thalami, mamillary bodies, tectal plate, and periaqueductal area, but atypical alterations may also been seen. A thorough knowledge of the neuroimaging findings of Wernicke's encephalopathy will assist in arriving at an early diagnosis, thus reducing the morbidity and mortality associated with this disease.

Voxel-based mapping of brain hypometabolism in permanent amnesia with PET.

In this study, we used voxel-based mapping methods to compare the resting cerebral metabolic rate of glucose (CMRglc) measured with PET in five patients with permanent amnesia (three with chronic Wernicke-Korsakoff and two with postanoxia syndrome) to that of nine healthy age-matched subjects. We assessed (i) a group pattern of relative hypometabolism; and (ii) the consistency of this group pattern, if any, in individual subjects, according to etiology. The results from the group analysis documented that permanent amnesia is associated with hypometabolism in the thalamus, posterior cingulate cortex, and mesial prefrontal cortex (near the anterior cingulate gyrus), bilaterally, as well as in the left supramarginal and middle temporal gyri. The individual analysis showed that this group pattern was found in essentially each patient, regardless of the cause of amnesia. Thus, permanent amnesia is subtended by dysfunction in structures belonging to Papez/limbic circuits as well as in left-hemisphere areas typically concerned with verbal functions, probably through a mechanism of thalamo-cortical disconnection and possibly involved in retrograde amnesia. The use of a voxel-based method allowed us to map a common network of synaptic dysfunction in a neuropsychological syndrome regardless of etiology. Our results indicate that this should be a powerful method in functional neuropsychology.

[MR tomography and computer tomography of alcohol-induced brain tissue changes]. / MR-Tomographie und Computertomographie von alkoholtoxischen Hirngewebsveränderungen.

Results of MRI in five alcoholics suffering from acute neurologic disorders such as ataxia, ophthalmoplegia and confusion are presented. The detection of focal cerebral lesions of high signal intensity in T2-weighted Spin-Echo- and Gradient-Echo-images in periventricular white matter, thalamus and pons combined with patients history and clinical presentation led to the diagnosis of Wernicke-Encephalopathia and Pontine Myelinolysis. MRI was far more sensitive compared with CT.

Wernicke's encephalopathy. A case report with neurophysiologic and CT-scan studies.

A case of Wernicke's encephalopathy with ataxia, confusion, memory loss, partial seizures of complex behavior and hypothermia, subsequent to thiamine depletion due to chronic malnourishment and triggered by an episode of acute vomiting and diarrhea, is reported, Computerized tomography (CT-scan) depicted small bilateral lesions in areas adjacent to the walls of the third ventricle, common location of the lesions seen in autopsy material of Wernicke's encephalopathy. Early diagnosis and treatment with vitamin B complex supplemented with intensive mnemonic and cognitive therapy led to complete recovery in a ten day period.