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.     

Wernicke脑病

Wernicke脑病（WE）是一种急性或亚急性起病的维生素B1缺乏症。表现为眼部体征、躯干性共济失调、意识障碍和情感淡漠及多发性周围神经病。M砌的表现有助于提示本病。WE是由于胃肠道手术、酗酒和胰腺炎等病因引起,补充维生素B1有助于恢复。     

Wernicke encephalopathy after gastrointestinal surgery for cancer: causes of diagnostic failure or delay

Wernicke encephalopathy (WE) is a neurological emergency due to thiamine deficiency. We aimed to identify clinical course and causes of diagnostic delay or failure of WE in a group of patients who underwent surgery for gastrointestinal tumors. A retrospective review of clinical, laboratory, neuroimaging, and therapeutic features of 10 patients with WE following abdominal surgery for cancer was carried out. Four patients died; in these subjects, diagnosis was delayed and supplementation of vitamin was absent or likely inadequate. Diagnostic delay or failure was also related to the coexistence of several medical complications at presentation masking typical symptoms of WE. In the surviving patients, outcome was influenced by promptness and type of therapy. Postoperative abdominal bleeding and number of subsequent operations may also had an effect. Postsurgical patients with gastrointestinal tumors may develop a subtle WE. The number of subsequent operations and the severity of postoperative complications may increase the risk of unrecognized WE. The disease should be suspected in postsurgical patients who have unexpected mental status changes, even under prophylactic treatment with vitamins. We suggest that prophylaxis with high doses of thiamine should be undertaken in patients with gastrointestinal tumors before surgery.     

非酒精性Wernicke脑病二例

目的探讨Wernicke脑病患者的临床和影像学特点。方法分析2例非酒精性Wernicke脑病患者的临床资料。结果 2例患者均为消化道晚期肿瘤行手术治疗、术后有营养不均衡、发病前有葡萄糖注射液补液史。2例患者均有意识障碍和典型的头颅MRI信号改变,即双侧丘脑内侧、中脑顶盖及导水管周围的高T_2信号;维生素B_1补充治疗后患者的症状有不同程度的好转。结论导致维生素B_1缺乏的高危因素、临床表现及典型的头颅MRI表现是临床上Wernicke脑病早期诊断的重要线索。早期足量维生素B_1治疗效果好。高危患者应注意预防Wernicke脑病的发生。     

The Spectrum of Vestibular and Ocular Motor Abnormalities in Thiamine Deficiency

Purpose

The first aim of this review is to summarize recent ocular motor signs in pre-encephalopathy patients with nutritional deficiency at risk of thiamine deficiency. Timely recognition of thiamine depletion in these patients, who may have a normal brain MRI, could lead to appropriate management and prevention of Wernicke’s encephalopathy (WE) with full recovery. The second aim is to incorporate recent diagnostic testing on the revised WE diagnostic criteria and the identification of patients who may show slow, partial, or no response to treatment.

Recent Findings

Selective vulnerability of periventricular gray neurons in thiamine deficiency is well known. Involvement of the vestibular and abducens nuclei may precede encephalopathy. Studies have shown mild ophthalmoparesis and bilateral symmetric vestibular loss in thiamine deficiency. Moreover, quantitative data has shown decreased horizontal vestibulo-ocular reflex (VOR) gain and nystagmus, with a favorable response to timely treatment.

Summary

Ophthalmoparesis, horizontal nystagmus, and decreased gain of the horizontal VOR, sparing the vertical VOR, may be present in the early pre-encephalopathy stage of thiamine deficiency. Rapid response to a loading dose of parenteral thiamine might be seen in some cases and normalization, albeit slower in others. In contrast, analogous to the Korsakoff’s syndrome, ocular motor and vestibular abnormalities may show only partial improvement. Future studies in larger populations at risk are needed to confirm the results of these preliminary observations.

     

Clinical and pathological features of alcohol-related brain damage

One of the sequelae of chronic alcohol abuse is malnutrition. Importantly, a deficiency in thiamine (vitamin B(1)) can result in the acute, potentially reversible neurological disorder Wernicke encephalopathy (WE). When WE is recognized, thiamine treatment can elicit a rapid clinical recovery. If WE is left untreated, however, patients can develop Korsakoff syndrome (KS), a severe neurological disorder characterized by anterograde amnesia. Alcohol-related brain damage (ARBD) describes the effects of chronic alcohol consumption on human brain structure and function in the absence of more discrete and well-characterized neurological concomitants of alcoholism such as WE and KS. Through knowledge of both the well-described changes in brain structure and function that are evident in alcohol-related disorders such as WE and KS and the clinical outcomes associated with these changes, researchers have begun to gain a better understanding of ARBD. This Review examines ARBD from the perspective of WE and KS, exploring the clinical presentations, postmortem brain pathology, in vivo MRI findings and potential molecular mechanisms associated with these conditions. An awareness of the consequences of chronic alcohol consumption on human behavior and brain structure can enable clinicians to improve detection and treatment of ARBD.     

Wernicke encephalopathy after subtotal gastrectomy for morbid obesity

Wernicke's encephalopathy (WE) is one of the potential complications of obesity surgery. It is an acute neuropsychiatric syndrome resulting from thiamine deficiency often associated with repeated vomiting. The classic triad is frequently reported in these patients (optic neuropathy, ataxia and confusion), associated with uncommon features. Cerebral impairment affects the dorsal medial nucleus of the thalamus and the periaqueductal grey area, appearing on MRI, as hyperintense signals on T2, Flair and Diffusion weighted imaging. Early diagnosis and parenteral thiamine are required to decrease morbidity and mortality. We report a case of WE and Korsakoff's syndrome in a young obese patient after subtotal gastrectomy, who still has substantial sequelae. The contribution of MRI with diffusion-weighted imaging is illustrated. The interest of nutritional supervision in the first weeks and preventive thiamine supplementation in case of repeated vomiting are of particular importance in these risky situations.     

Wernicke encephalopathy in a chronic peritoneal dialysis patient--correlation between diffusion MR and pathological findings

Wernicke encephalopathy (WE) is a neurologic disorder caused by a nutritional deficiency of thiamine. Since the lesion in WE consists of brain edema, diffusion weighted imaging (DWI) is quite useful for detecting the pathologic changes in WE, and can differentiate between reversible extracellular (vasogenic) and irreversible cellular (cytotoxic) edema. We report here a 16-year-old man with WE who had been treated with continuous ambulatory peritoneal dialysis for chronic renal failure. He underwent repeated DWI and postmortem examination. DWI, which was performed 8 days after the onset of neurological symptoms, showed high intensity areas in the bilateral thalami, mammillary bodies, tegmentum mesencephali and pons. Apparent diffusion coefficient (ADC) map also showed slightly high intensity in the periaqueductal gray matter and pons, which indicated extracellular edema. On the other hand, ADC map showed low intensity areas in the most medial part of the thalami and marginal area of the tegmentum mesencephali, which indicated cellular edema. In the postmortem examination, the areas that showed low intensity on ADC map exhibited mild neuronal loss. Based on the correlation between the DWI and pathologic findings, the cytotoxic edema of the bilateral medial thalami and marginal tegmentum mesencephali in this patient was considered to be glial cell edema which may protect against neuronal cell damage.     

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.     

Wernicke’s encephalopathy in a patient with acute pancreatitis: unusual cortical involvement and marvelous prognosis

Wernicke's encephalopathy (WE) is a severe neurological disorder caused by thiamine deficiency. Clinically, it is most frequently observed in people with alcohol abuse. WE, however, can occur in any clinical condition associated with malnutrition or thiamine deficiency. We present the case of a 47-year-old woman with prolonged therapeutic fasting who presented with ophthalmoplegia, ataxia and deep coma. MRI showed unusual symmetric cortical abnormalities in the frontal and parietal lobes, as well as typical lesions surrounding the third ventricle and aqueduct. Although the patient entered a vegetative state, she finally regained consciousness after thiamine supplementation unexpectedly. To the best of our knowledge, it has never been reported to date that the patient with WE in a vegetative state with cortical damage shows a marvelous prognosis, which prompts us to report this case. In the present report, we highlight the role of MRI in the diagnosis of acute WE.     

Long-term cognitive sequelae in a case of Wernicke’s encephalopathy after allogeneic stem cell transplantation

ABSTRACT

We describe the case of a non-alcoholic patient with chronic myeloid leukemia who developed iatrogenic Wernicke’s encephalopathy (WE) following stem cell transplantation. Four years after the WE acute event, the patient’s cognitive profile was mainly characterized by moderate memory impairment, and functional and daily-living difficulties. Our report sustains the hypothesis that a iatrogenic form of WE may produce long-term cognitive sequelae even when thiamine therapy is administered in the acute phase until the resolution of the neurological signs.     

Treatment of Wernicke’s encephalopathy with high dose of thiamine in a patient with pyloric sub-stenosis: description of a case

Wernicke’s encephalopathy (WE) is an acute or subacute syndrome that results from a deficiency in vitamin B1 (thiamine). The syndrome is characterised by a classical triad of symptoms: nystagmus and ophthalmoplegia, mental-status changes, and unsteadiness of stance and gait. When patients with WE are inappropriately treated with low doses of thiamine, mortality rates average out at 20% and Korsakoff’s Psychosis develops in about 85% of survivors (Sechi and Serra in Lancet Neurol 6(5):442–455, 2007). We report the case of a patient with a pyloric sub-stenosis that developed a WE, and was treated with high doses of thiamine showing after few days of treatment a great improvement of neurological and neuroradiological assessment, even though cognitive impairment was still severe at discharge and at 6 months follow-up.     

Loss of astrocytic glutamate transporters in Wernicke encephalopathy

Wernicke encephalopathy (WE), a neurological disorder caused by thiamine deficiency (TD), is characterized by structural damage in brain regions that include the thalamus and cerebral cortex. The basis for these lesions is unclear, but may involve a disturbance of glutamatergic neurotransmission. We have therefore investigated levels of the astrocytic glutamate transporters EAAT1 and EAAT2 in order to evaluate their role in the pathophysiology of this disorder. Histological assessment of the frontal cortex revealed a significant loss of neurons in neuropathologically confirmed cases of WE compared with age‐matched controls, concomitant with decreases in α‐internexin and synaptophysin protein content of 67 and 52% by immunoblotting. EAAT2 levels were diminished by 71% in WE, with levels of EAAT1 also reduced by 62%. Loss of both transporter sites was confirmed by immunohistochemical methods. Development of TD in rats caused a profound loss of EAAT1 and EAAT2 in the thalamus accompanied by decreases in other astrocyte‐specific proteins. Treatment of TD rats with N‐acetylcysteine prevented the downregulation of EAAT2 in the medial thalamus, and ameliorated the loss of several other astrocyte proteins, concomitant with increased neuronal survival. Our results suggest that (1) loss of EAAT1 and EAAT2 glutamate transporters is associated with structural damage to the frontal cortex in patients with WE, (2) oxidative stress plays an important role in this process, and (3) TD has a profound effect on the functional integrity of astrocytes. Based on these findings, we recommend that early treatment using a combination of thiamine AND antioxidant approaches should be an important consideration in cases of WE. © 2009 Wiley‐Liss, Inc.     

Magnetic Resonance Reflects the Pathological Evolution of Wernicke Encephalopathy

BACKGROUND AND PURPOSE: Wernicke encephalopathy (WE) is an acute phase of Wernicke-Korsakoff syndrome. Pathologic findings change between acute and chronic phases. Only a few magnetic resonance imaging (MRI) studies have been done to date. METHODS: To correlate the MRI findings in acute and chronic stages of WE with the known pathologic information, 15 consecutive patients with WE were examined with MRI: 3 before thiamine treatment, 7 within 24 hours of thiamine treatment, 4 between the second and sixth day after thiamine treatment, and 1 fifty-five days after thiamine treatment. Nine of the patients had follow-up MRI between 2 days and 33 months. T1-weighted, proton, and T2-weighted axial images were obtained with additional 5-mm-thick T1-weighted sagittal and coronal images to better visualize the mammillary bodies. RESULTS: In the acute WE, MRI showed high signal intensityon T2-weighted images in periaqueduct and medial thalamic regions. In a few patients with alcoholism, vermian and mammillary body atrophies and third ventricular enlargements were noted. In the chronic phase of WE, T2 hyperintensity disappeared but mammillary bodies and cerebellar vermis became atrophic and third ventricular enlargements were evident. High signal intensity on T2-weighted images disappeared as early as 2 days, and atrophic changes appeared as early as 1 week. CONCLUSION: MRI is useful for in vivo monitoring and reflects the pathological evolution in acute and chronic phases of WE.     

Hypothermia, Wernicke encephalopathy and multiple sclerosis

Neurological exacerbation observed in MS patients is usually related to a demyelinating process. We report two patients where hypothermia (32.4 degrees C and 32.5 degrees C) and neurological exacerbation were probably due to a Wernicke encephalopathy (WE). The clinical features and the rapid efficiency of parenteral thiamine were suggestive of WE. Hypothermia is an exceptional symptom observed in MS and has been considered as resulting from hypothalamic demyelination; these two cases showed that WE which is another cause of reversible hypothermia, can be associated with MS.     

Atypical Wernicke's encephalopathy showing lesions in the cranial nerve nuclei and cerebellum.

Wernicke's encephalopathy (WE) is a severe neurological disorder caused by a dietary deficiency of Vitamin B1 and characterized by consciousness changes, ocular abnormalities, and ataxia. Wernicke's encephalopathy is considered a medical emergency and treatment consists of intravenous thiamine administration. Typically in alcoholic and nonalcoholic patients magnetic resonance shows alterations in the mamillary bodies, medial thalami, tectal plate, and periaqueductal regions. We report here on a nonalcoholic atypical case of WE which presented with reversible symmetrical lesions in the cranial nerves nuclei and in the cerebellum alongside the classic neuroradiological findings.     

Vitamin B1 in the treatment of Wernicke’s encephalopathy due to hyperemesis after gastroplasty

Wernicke’s encephalopathy (WE) is a severe brain disorder, first described in 1881, and is caused by a nutritional deficiency of thiamine (vitamin B1) found mostly in patients suffering from chronic alcoholism. In addition, WE can also complicate bariatric surgery if adequate vitamin supplementation is not insured. Without immediate treatment, the prognosis is poor and the mortality rate is high. Most patients present with atypical neurological symptoms, which hampers rapid diagnosis. We present a 40-year-old woman who underwent gastroplasty combined with gastric banding for severe obesity. She experienced repetitive vomiting and her diet was without vitamin supplementation. After three months she developed convergent strabismus, apathy and urinary incontinence, which was diagnosed as WE and treated as such. Six months later her recovery was incomplete, still showing gait difficulties and nystagmus. We aim to show that adequate vitamin supplementation in patients undergoing gastroplasty is necessary, especially considering the risk of permanent neurological deficits.     

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.     

尿毒症患者透析后舞蹈样并发症二例分析及文献复习

目的探讨尿毒症患者透析后舞蹈样并发症的临床表现和治疗方法。方法选择2例典型患者进行临床表现及影像学结果回顾性分析。结果尿毒症血透患者维生素B_1缺乏可由多种原因引起,常见于摄入过低和透析中丢失过多。维生素B_1不足,三羧酸循环紊乱,血乳酸增加,从而导致机体出现一系列相关症状。其临床表现多为威尼克氏脑病。结论对尿毒症血透发生基底神经节功能紊乱所引发的舞蹈样症的患者,诊断时应加强对本病的认识。血乳酸和脑基底节影像学检查可为本病提供诊断依据。维生素B_1静脉疗法对本病有明显的缓解作用。