非定型なAlzheimer病の1剖検例

AN AUTOPSY CASE OF ATYPICAL ALSHEIMER'S DISEASE

I．はじめに

　初老期痴呆の中で，Alzheimer病の病理組織学的特徴は肉眼的には大脳のびまん性萎縮であり，光顕的には老人斑，原線維変化，顆粒空胞変性などの老人性変化が著しく認められることである。Pick病は肉眼的所見として葉性萎縮を示し，鏡検で白質のグリオーシス，神経細胞の変性，inHated cells，嗜銀球の出現などを特徴としている。しかし，中には両者の所見が同一症例にみられたり^1,2,6,12），老人斑のないAlzheimer^{1,3,6,13,15,18）}病など非定型な初老期痴呆の剖検例も少なからずみい出される。我々もこのたび非定型初老期痴呆の1例を経験したので報告するとともに若干考察をおこないたい。

An autopsy case of atypical Alzheimer's disease was reported clinicopathologically. The case was a 48-year-old female. She developed progressive intellectual deterioration since 45 years old and could not perform the housework, and was admitted to the mental hospital. The mental examination disclosed severe dementia. The results of neuro-logic examination and laboratory data were essen-tially normal. She was well-liked and popular in the hospital. But dementia progressed steadily and she became immobile. She died at the age of 48 after the course of three years.

At autopsy the brain weighed 1000 gm. There was atrophy of the brain with focal accentuation in the frontal and temporal regions. On micro-scopic examination there lie three points of interest in this case. Firstly Alzheimer's neurofibrillary changes were enormous in the all parts of the cerebral cortex, thalamus, lenticular nuclei and some nuclei of the brain stem. On the other hand, the senile plaques were scanty and only few of atypical plaques were observed in the temporal and hippo-campal gyri. Secondly fiber gliosis of the white matter in the temporal and frontal lobes were re-cognized. A few nerve cells in the amygdaloid nucleus were ballooned and had argyrophilic in-clusions. These findings resemble those of Pick's disease. An additional observation of interest was afforded by the deposits of calcium in the falx, parietal lobe, lenticular nuclei and cerebellum. As a conclusion this case may be considered to have the morphological features of Alzheimer's and Pick's disease incompletely respectively in addition to Fahr's disease.