性格変化と軽度の知能障害を示したオリーブ・橋・小脳萎縮症の1剖検例

AN AUTOPSY CASE OF OLIVO-PONTO-CEREBELLAR ATROPHY WITH A CHANGE OF CHARACTER AND SLIGHT DISTURBANCE OF INTELLIGENCE

I．はじめに

　オリーブ・橋・小脳萎縮症は1900年Dejerine-Thomas^5）により疾患単位として命名されて以来，多数の報告例がある^{1〜9,11〜31）}。これらの報告例のなかで精神症状を伴う症例が少数例であるが認められる^{3,4,12,14,15,19,28）}。

　われわれは特異な性格変化と軽度の知能障害を示したオリーブ・橋・小脳萎縮症の1例を経験し剖検する機会を得たので臨床症状と病理学的所見の関連について考察を加えた。

H. I., a housewife aged 55 years, began feeling a gait disturbance at 48 years old. After this she had incoordination of arms, dysarthria and tremor of hands. Aged 54 years, she could not stand up by herself. In addition to these neurological signs she had a change of character, such as losing control of herself, unreservedness and unceremoni-ousness, and slight disturbance of intelligence. She died at the age of 55 years about seven years after the onset.

Histopathologically, noticeable changes were ob-served on the medulla oblongata, pons and cere-bellum ; the severe neuronal loss of the pontine nuclei and the olivary nuclei with demyelination and gliosis of the cerebellopetal fibers. Especially a great deal of lipofuscin granules in the nerve cells of the frontal and temporal lobe were observed. In the substantia nigra some pigmented cells were deleted.

This case was diagnosed as olivo-ponto-cerebellar atrophy clinico-histopathologically. We discussed concerning the etiology of the changes of person-ality and slight disturbance of intelligence in re-lation to histopathological changes. It is speculated that the mental disorders are due to the degener-ation of the nerve cells in the frontal and temporal lobes.