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[匹克氏病。解剖学-临床视角]

[Pick's disease. Anatomo-clinical point of view].

作者信息

Brion S, Plas J, Jeanneau A

机构信息

Laboratoire Universitaire de Neuropathologie, Hôpital Sainte-Anne.

出版信息

Rev Neurol (Paris). 1991;147(11):693-704.

PMID:1775822
Abstract

For historical reasons, Pick's disease is sometimes misunderstood in the medical literature. However, clinical signs are quite typical, and different from those of Alzheimer's disease. The disease is characterized by a circumscribed fronto-temporal atrophy, without senile lesions. Neuronal loss and ballooned neurons are present in the atrophic cortex. In about 30 p. 100 of cases, Pick's bodies are present, with various shapes, perhaps depending upon the duration of the disease process. Other lesions are described. The symptomatology is characterized by personality changes of the frontal type, associated with verbal and behavioural stereotypies, and bulimia. There is a striking normality of the EEG, and CT scan shows the fronto-temporal cortical atrophy and atrophy of the caudate nucleus. In most cases, memory for recent events is quite normal, but sometimes an amnestic syndrome is closely related with an unusually severe atrophy of the hippocampus. The consequences of atrophy of the caudate nucleus are debated, since extrapyramidal symptoms appear to be mild and late.

摘要

由于历史原因,皮克病在医学文献中有时会被误解。然而,其临床症状相当典型,且与阿尔茨海默病不同。该疾病的特征是局限性额颞叶萎缩,无老年病变。萎缩的皮质中存在神经元丢失和气球样神经元。在约30%的病例中,可见各种形状的皮克小体,这可能取决于疾病进程的持续时间。还描述了其他病变。其症状表现为额叶型人格改变,伴有言语和行为刻板症以及贪食症。脑电图显示明显正常,CT扫描显示额颞叶皮质萎缩和尾状核萎缩。在大多数情况下,近期事件的记忆相当正常,但有时遗忘综合征与海马体异常严重的萎缩密切相关有关尾状核萎缩的后果存在争议,因为锥体外系症状似乎较轻且出现较晚。

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