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.     

Wernicke's encephalopathy presenting with upbeating nystagmus.

The case of a 49-year-old woman with Wernicke's encephalopathy is described, in which primary position upbeating nystagmus was the chief ocular sign. Although there was no history of excessive alcohol consumption, Wernicke's encephalopathy was diagnosed on a background of anorexia nervosa. The diagnosis was supported by the patient's symptomatic and clinical recovery following thiamine therapy.     

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.     

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.     

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.     

Wernicke's encephalopathy in non-alcoholics. An autopsy study

In a 5-year autopsy material constituting 6,964 autopsies, there were 52 cases of Wernicke's encephalopathy of which 12 (23%) occurred in non-alcoholics. Among 18 cases with active (acute) disease, 7 cases (39%) were found in non-alcoholics. Only 4 cases of active Wernicke's disease were diagnosed clinically, all of them in alcoholics. The predominant clinical symptoms were disorientation and depressed levels of consciousness, whereas eye symptoms were recorded in only 3 cases. None of the non-alcoholics were given specific thiamine therapy, whereas some alcoholics received large doses of the vitamin as a routine procedure. However, the thiamine therapy was often instituted too late. It is concluded that active Wernicke's encephalopathy should be considered in all patients with prolonged malnutrition and that disorientation and depressed levels of consciousness may be the predominating symptoms of the disease. Even the slightest suspicion of Wernicke's encephalopathy should prompt immediate administration of large doses of thiamine parenterally.