CREUTZFELDT-JAKOB DISEASE PRESENTING AS WERNICKE-KORSAKOFF-SYNDROME
CREUTZFELDT-JAKOB DISEASE PRESENTING AS WERNICKE-KORSAKOFF-SYNDROME
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DOI：
10.1016/0022-510x(92)90045-m
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发表时间：
1992-04-01
影响因子：
4.4
通讯作者：
PIETRINI, V
中科院分区：
文献类型：
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作者：
PIETRINI, V
一名47岁的男子开始遭受进行性躯干共济失调和精神改变典型的韦尼克-科萨科夫综合征。 他表现出精神错乱、幻觉、嫉妒谵妄和近期记忆严重受损。 该疾病持续了13个月，但仅在最后几周才并发肌阵挛。 三相假周期性尖波的特征脑电图记录只有在最后阶段。 脑的肉眼检查显示乳头体和上级蚓部明显萎缩。 然而，组织学特征与Creutzfeldt-Jakob病（CJD）一致，后者结构的变化局灶性加重。 这一病例支持CJD改变开始于中枢神经系统，随后可能通过中枢轴突沿着神经通路扩散的假设。 只有在最后阶段，病理过程才涉及灰质的大部分。 在这个病例中，小脑-乳头-丘脑系统的CJD过程的局灶性加重导致了Wernicke-Korsakoff样综合征。
A 47-year-old man began to suffer from progressive truncal ataxia and mental alterations typical of Wernicke-Korsakoff syndrome. He showed confusional state, hallucinations, delirium of jealousy and a serious impairment of recent memory. The symptomatology lasted 13 months, but only in the last weeks was it complicated by myoclonias. Triphasic pseudoperiodic sharp-waves characterized the EEG-recordings only in the final stage. Macroscopic examination of the brain showed marked atrophy of the mammillary bodies and superior vermis. However, the histological features were consistent with Creutzfeldt-Jakob disease (CJD) with focal accentuation of the changes in the latter structures. This case supports the hypothesis that CJD-changes begin focally in the CNS and, subsequently, spread along neuronal pathways, probably via central axons. Only in the final stage does the pathological process involve most parts of the gray matter. A focal accentuation of the CJD process in the cerebello-mammillo-thalamic system caused in this case a Wernicke-Korsakoff-like syndrome.