Wernicke脑病

<正>Wernicke脑病是维生素B1缺乏导致的代谢性脑病,1881年由Wernicke首先描述,1940年Campbell和Russell提出其诱因是维生素B1缺乏。该病系维生素B1缺乏致特定区域神经细胞能量代谢障碍所致,呈急性或亚急性起病,临床主要表现为眼外肌瘫痪、共济失调和意识障碍,最常见于慢性酒精中毒和妊娠性呕吐;非维生素B1缺乏性Wernicke脑病系转酮醇酶(TK)基因     

Wernicke's encephalopathy

Experimental Wernicke's encephalopathy, induced in rhesus monkeys with a diet lacking thiamine (vitamin B1), is characterized by cavitary necrosis of the striatum as well as a microvacuolar periventricular lesion of the brain stem such as occurs in man. With high resolution light microscopy and electron microscopy, the primary structural alteration in the brain stem lesion, and probably also in the striatum, appears to be that of widespread "blister" formation due to splitting of myelin at the intraperiod line. Microvascular alterations were minimal, even in the most severely affected regions. It is the myelin blisters which give rise to the spongy texture of the neuropil. A similar splitting of myelin has been described in several other experimental encephalopathies, and it is probable that it also occurs in Wernicke's encephalopathy in man.     

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.     

Wernicke's encephalopathy in infancy

Summary Wernicke's encephalopathy documented by characteristic involvement of the mammillary bodies developed in a premature infant during parenteral nutrition. This case illustrates the need for thiamine supplementation in seriously ill patients with inadequate intake of the vitamin.     

Restless legs syndrome in dialysis patients: a comparison between hemodialysis and continuous ambulatory peritoneal dialysis

Restless legs syndrome (RLS) is common in end-stage renal disease (ESRD) patients undergoing hemodialysis (HD). A few studies so far have investigated RLS prevalence in ESRD patients undergoing continuous ambulatory peritoneal dialysis (CAPD). The aim of this study was to compare the prevalence, characteristics, consequences and predictors of RLS between HD and CAPD patients. We recruited 58 HD and 28 CAPD patients. A neurologist expert in sleep medicine performed RLS diagnosis during a face-to-face interview. The prevalence of RLS was slightly higher in HD than in CAPD patients (19 vs. 10.7%). RLS appeared after the onset of kidney complaint in HD patients; in contrast, in CAPD patients RLS preceded the occurrence of renal disease. Five HD patients reported that RLS symptoms occurred throughout the dialysis session. HD patients with RLS⁺ had a higher mean number of HD sessions per week and a longer mean duration of HD session than the RLS^? ones. Prevalence of females was significantly higher in CAPD patients with RLS⁺ than in the RLS^? ones. RLS frequently affects both HD and CAPD patients. RLS impaired sleep in both groups, but use of dopaminergic agents was uncommon in our sample. Dialysis schedule was associated with RLS in HD patients, while female sex was related to RLS in CAPD patients. Awareness concerning RLS identification and treatment in HD and CAPD patients is recommended.     

MRI in non-alcoholic Wernicke's encephalopathy

Wernicke's encephalopathy (WE) is a potentially reversible disorder. It is often not considered in non-alcoholic patients unless MRI demonstrates lesions in the appropriate sites. However, specific MRI sequences only highlight some areas of abnormality and hence WE may not be considered unless a more complete study is performed and this is highlighted in the case described herein. The neuropathological basis for the imaging findings is also discussed.     

Wernicke's encephalopathy in two patients with acquired immunodeficiency syndrome

Summary Two non-alcoholic homosexual patients with acquired immunodeficiency syndrome (AIDS) are reported who developed acute Wernicke's encephalopathy in the terminal stage of their illness. The first patient presented with vascular congestion, minute haemorrhages, proliferation of microglia and of the vessel walls at the predilection sites of the Wernicke-Korsakoff process. In the second patient only the mamillary bodies were involved. Besides Wernicke's encephalopathy, a primary cerebral immunoblastoma and cerebral toxoplasmosis were found in the first patient, whereas the second showed severe encephalitis with numerous microglial and multi-nucleated giant cells reacting positively with anti-HIV antibody. Just as in the development of Wernicke's encephalopathy in malignant diseases, the catabolic trend of the metabolism of the immunodeficient patients with consecutive thiamine deficiency must be considered the principal pathogenetic mechanism.     

Vestibular responses in Wernicke's encephalopathy

Two patients with Wernicke's encephalopathy were evaluated with quantitative vestibulo-ocular reflex and ocular motor testing. Vestibulo-ocular reflex testing included caloric irrigation, earth vertical axis rotational sinusoids, and rotational impulses. Both patients demonstrated hypoactive vestibular responses to both caloric and rotational stimuli at the time of presentation. One patient had unbeating nystagmus that diminished with upgaze, downgaze, or convergence. Following treatment with thiamine, both patients' vestibular responses improved but remained abnormal, with a short vestibulo-ocular reflex time constant and increased low-frequency rotational phase lead. Impairment of the velocity storage element attributable to damage to the vestibular nucleus and nucleus prepositus hypoglossi may account for this permanent effect on the vestibulo-ocular reflex.     

'Iatrogenic' Wernicke's encephalopathy in Japan

'Iatrogenic' Wernicke's encephalopathy has appeared to occur more frequently in Japan, probably induced by the change of our Japanese national health insurance policy in 1992. We report 4 nonalcoholic patients with such Wernicke's encephalopathy, which occurred during the early postoperative oral food intake period following intravenous nutrition without vitamin supplements. We analyzed the medical records of 4 patients, 3 men and 1 woman, aged between 55 and 71 years, who were admitted to our hospital between 1992 and 1995. Three patients underwent gastrointestinal surgery and 1 suffered chronic pyothorax. We diagnosed our patients as having Wernicke's encephalopathy based on typical neurological abnormalities, in addition to typical cranial magnetic resonance image findings, low serum vitamin B(1) levels, or both. Although all of the patients were treated with vitamin B(1) and showed some improvement, 1 patient developed Korsakoff syndrome, 2 made incomplete neurological recovery, and 1 died. We speculated that the body vitamin B(1) stores had been decreasing in our patients who did not receive any vitamin supplements during intravenous hyperalimentation or hydration. Subsequent administration of high calorie and high carbohydrate oral diets increased the demand for vitamin B(1), further depleting the vitamin stores, thereby causing 'iatrogenic' Wernicke's encephalopathy. The change of our national health insurance policy in 1992 discouraged the routine administration of vitamins, probably causing Wernicke's encephalopathy in our patients.     

Vascular changes in acute Wernicke's encephalopathy

The nature and distribution of vascular changes in acute Wernicke's encephalopathy (WE) were analyzed in three autopsy cases. Lesions of the lateral vestibular nucleus of the medulla oblongata (three cases) and lateral ventricular wall (one case) were examined by reconstruction of 200 serial sections, and the capillary diameter in the tegmentum of the medulla oblongata was measured morphometrically in all cases. The vascular changes commonly found in and around the parenchymal lesions in all cases were: (1) dilatation and endothelial swelling of almost all vessels ranging from small arteries to veins, being especially severe with undulation in small arteries and arterioles, and (2) fibrinoid degeneration and hemorrhage involving selectively the arterioles and capillaries on the arterial side. These vascular changes in the medulla oblongata were essentially the same as those in the third ventricular wall, but differed in their severity. Capillary diameter in these cases was significantly larger than that in seven control cases. Such vascular changes, especially a dysoric change on the arterial side, could not be ascribed to secondary change following changes in the parenchyma, and were, therefore, considered to be a lesion essential to WE, as in the case of the parenchymal lesion.