Hypothermia in multiple sclerosis.

Five patients with clinically definite multiple sclerosis are reported who presented with acute relapses associated with hypothermia. Repeated episodes of hypothermia were seen in four. Thrombocytopenia was associated with the hypothermia in four patients. Further investigation disclosed a tendency to chronic hypothermia and suggested an altered thermoregulatory set point in one patient, when MRI, endocrine, and autonomic studies failed to localise a lesion in the hypothalamus, but subsequent necropsy showed hypothalamic lesions. In such patients a predisposition to altered thermoregulation may occur due to direct involvement of the hypothalamus or from combined lesions affecting hypothalamic outflow to the brainstem and spinal cord.     

Hypothermia in three patients with multiple sclerosis

Hypothermia, defined as a core temperature less than 35 degrees C has multiple causes and several neurological consequences. The cases of three patients with definite multiple sclerosis since more than a decade are reported, who presented with several episodes of coma and hypothermia. Systematic neuropathologic examination of the hypothalamus in one case did not reveal any abnormality.     

Wernicke encephalopathy and Creutzfeldt-Jakob disease

We assessed the prevalence of Wernicke encephalopathy (WE) in all 657 cases suspected of Creutzfeldt-Jakob (CJD) referred from 2001 to 2006 to the French Neuropathology Network of CJD. Clinical, biological and imaging data were reviewed when the diagnosis of WE was made at autopsy. No CJD was found in five cases suspected of sporadic CJD. In these five cases, myoclonus had been observed in four, CSF 14-3-3 protein in two. In 14 other cases, WE was combined with CJD, 13 of which were sporadic. These belonged mainly to the molecular variants of sporadic CJD associated with a long duration of disease. This stresses the necessity of remaining alert to the diagnosis of WE when CJD is suspected.     

Clinical manifestation, imageological and pathological characteristics of Wernicke encephalopathy

BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been reported successively. But its imageological detection needs to be further investigated. OBJECTIVE: To analyze the characteristics of clinical manifestations, skull MRI examination and pathological results in patients with WE. DESIGN: Retrospective analysis. SETTING: The General Hospital of Chinese PLA. PARTICIPANTS: Ten patients of WE admitted to the Department of Neurology, General Hospital of Chinese PLA were recruited. Among them, five patients were diagnosed pathologically after death. Their pathological changes accorded with the pathological characteristics of WE. The other 5 patients were diagnosed clinically before death. Their pathological changes accorded with clinical and imageological manifestations and had definite reaction to the treatment of thiamine. Ten patients, 7 males and 3 females, were aged (47±13) years ranging from 33 to 73 years. Their disease courses averaged 6 weeks ranging from 3 to 10 weeks. They all were non-alcoholics. Four patients developed WE after acute pancreatitis, two patients after the recurrence of gastric cancer, two patients after cholecystectomy, one patient after hepatitis medicamentosa, one patient after Alzheimer disease. Informed consents were obtained from all the patients and their relatives. METHODS: After admission, clinical manifestations of patients were observed and recorded. Five patients underwent skull MRI examination and their detected results were recorded. Five dead patients underwent autopsy and brain pathological examinations. Neuropathological examination involved cerebrum, cerebellum and brain stem. MAIN OUTCOME MEASURES: Clinical manifestations, MRI examination results, pathological analysis results and prognosis of all the patients. RESULTS: Ten patients with WE were involved in the final analysis. ①Nine patients presented various degrees of mental and conscious disturbance. Six patients initially presented vertigo, nausea, and vomiting. Five patients showed opthalmoplegia. Three patients presented hypotension (BP < 120/60 mm Hg, 1 mm Hg= 0.133 kPa). Two patients showed ataxia. One patient showed severe polyneuropathy. Both lower extremities were more severe. EMG showed axonal degeneration. ②Five patients accepted skull MRI examination. Three patients showed typical high signals in periaqueductal regions and circumference of third and fourth ventriculus in T2 -weighted and flair-weighted images, two of them showed high signals in fornixes in T2 -weighted and flair-weighted images, and one of them showed high signals in optic chiasma, both mammillary bodies, and subcortical white matter. No abnormality was found in the other two patients. Two patients who accepted the supplements of thiamine showed obviously improvements in the second MRI examination. ③Macroscopically, the border between gray and white matters was clear in the coronal section of cerebrum. Congestions, edema and multiple petechial hemorrhages were found in periaqueductal regions, circumference of third and fourth ventriculus, and both mammillary bodies. Microscopically, various degrees of necrosis of parenchymal structures, loss of nerve cells and ischemic changes were found. Myelinated fibers were more affected than neurons. There were focal capillary proliferation and multiple petechial hemorrhages. Prominent astrocytic proliferations were found in gelatinous fiber staining. And demyelinations were found in myelin staining. These pathological findings were all in accord with the diagnostic criteria of WE. ④In the diagnosis before death, 4 of 5 patients who were supplemented with thiamine had obvious improvement 2 weeks later and 1 of them abandoned therapy due to progressive aggravation of jaundice caused by recurrence of stomach cancer. The other 5 patients who were not diagnosed definitely before death and not supplemented with thiamine died. Final diagnosis was performed in autopsy examination. CONCLUSION: The clinical manifestations of this group of WE patients are atypical. MRI and pathological examination results are corresponding, and both have the characteristic manifestations of WE..     

Jakob-Creutzfeldt disease associated with Wernicke encephalopathy

Wernicke disease (WD) is a complication of alcoholism and malnutrition and usually presents acutely and is characterized by disturbances of consciousness, paralysis of the external ocular muscles, and ataxia. The disease results from deficiency of vitamin B 1, or thiamine, an essential coenzyme in intermediate carbohydrate metabolism. On the other hand, Jakob-Creutzfeldt disease (J-C) results from infection with an unconventional agent with a long incubation period and is characterized by a rapidly progressive dementia and histologically by a spongiform encephalopathy associated with neuronal destruction and pronounced astrogliosis. Combination of both diseases has not been reported in the literature previously and their relationship is uncertain. We present 3 cases with this interesting association and consider their relationship.     

Anti-Ma encephalitis masquerading as Wernicke encephalopathy

BackgroundAnti-Ma encephalitis is a disease usually associated with testicular cancer in young male patients. Anti-Ma encephalitis presented as Wernicke encephalopathy-like symptoms and with gastric cancer is rare. Here, we report a case of anti-Ma encephalitis with gastric cancer in an elderly patient, which has been misdiagnosed of Wernicke encephalopathy.Case reportA 71-year old male with a history of alcohol abuse was admitted to the hospital because of progressive dizziness, diplopia and anorexia lasted for 1 month. He was initially diagnosed as Wernicke encephalopathy. However, this patient failed in the treatment of VitB1. The blood and cerebrospinal fluid examination found the presence of anti-Ma1/2 antibodies. ¹⁸F-FDG PET-MR showed symmetrical hypermetabolic changes on the bilateral hypothalamus, basal ganglion and brainstem, as well as gastric neoplasms with liver metastasis. The patient was finally diagnosed with anti-Ma encephalitis.ConclusionAnti-Ma encephalitis should be suspected in patient with Wernicke encephalopathy-like symptoms but failed VitB1 treatment.     

Hypothermia in Wernicke's encephalopathy

Two cases of Wernicke's encephalopathy with severe hypothermia as a major presenting sign are reported. The outcome was favorable with thiamine treatment. The clinical features, frequency, prognostic value and clinico-pathological correlations of hypothermia in this condition are reviewed.     

EFNS guidelines for diagnosis,therapy and prevention of Wernicke encephalopathy

Background: Although Wernicke encephalopathy (WE) is a preventable and treatable disease it still often remains undiagnosed during life. Objectives: To create practical guidelines for diagnosis, management and prevention of the disease. Methods: We searched MEDLINE, EMBASE, LILACS, Cochrane Library. Conclusions and recommendations:

• 1 The clinical diagnosis of WE should take into account the different presentations of clinical signs between alcoholics and non alcoholics (Recommendation Level C); although prevalence is higher in alcoholics, WE should be suspected in all clinical conditions which could lead to thiamine deficiency (good practice point – GPP).
• 2 The clinical diagnosis of WE in alcoholics requires two of the following four signs; (i) dietary deficiencies (ii) eye signs, (iii) cerebellar dysfunction, and (iv) either an altered mental state or mild memory impairment (Level B).
• 3 Total thiamine in blood sample should be measured immediately before its administration (GPP).
• 4 MRI should be used to support the diagnosis of acute WE both in alcoholics and non alcoholics (Level B).
• 5 Thiamine is indicated for the treatment of suspected or manifest WE. It should be given, before any carbohydrate, 200 mg thrice daily, preferably intravenously (Level C).
• 6 The overall safety of thiamine is very good (Level B).
• 7 After bariatric surgery we recommend follow‐up of thiamine status for at least 6 months (Level B) and parenteral thiamine supplementation (GPP).
• 8 Parenteral thiamine should be given to all at‐risk subjects admitted to the Emergency Room (GPP).
• 9 Patients dying from symptoms suggesting WE should have an autopsy (GPP).

     

Diffusion abnormalities in patients with Wernicke encephalopathy

Diffusion-weighted imaging (DWI) can help to diagnose acute ischemic stroke. Other nonischemic disorders may show abnormal signals with DWI. The authors report two cases of Wernicke encephalopathy with DWI signal changes in characteristic midline locations, one with reduction in apparent diffusion constant and one without. DWI abnormalities may suggest early thiamine deficiency and are useful in diagnosing Wernicke encephalopathy.     

Hypothermia and multiple sclerosis. A case with 3 episodes of transient hypothermia

On three occasions over a 21-month period, a woman with multiple sclerosis presented with hypothermia accompanied by altered consciousness, neurological signs and inappropriate antidiuretic hormone secretion. One of the episodes included hypoglycaemia. Although repeated MRI examinations, one of them with gadolinium injection, gave negative results, hypothalamic demyelination was suspected. The 4-year follow-up of this patient suggests that this lesion has no prognostic value.     

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.     

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.