非定型ピック病の1剖検例

An Autopsied Case of Atypical Pick's Disease

　【抄録】　非定型的な神経病理学的所見を呈したピック病の1剖検例を報告した。症例は74歳，女性。物忘れで発症し進行性の痴呆を呈したが，人格変化や滞続言語は認められず，アルツハイマー病と診断された。しかし，アルツハイマー病としても若干非定型的な点も認められた。神経病理学的には前頭葉眼窩面および側頭葉の限局性脳萎縮，同部位に強調される神経細胞の脱落，皮質下白質の高度なグリオーシスからピック病と診断された。しかし，海馬付近に限局した多数の神経原線維変化の出現やマイネルト基底核，青斑核の強い病変に加え，両側の錐体路変性など，通常ピック病にはみられない特異的な所見が認められた。

　The patient was a 74-year-old woman who had a fourteen-year history of progressive dementia. As her initial symptom was memory disturbance and the CT scan showed diffuse brain atrophy, she was diagnosed as having Alzheimer's disease. “Triebhafte Enthemmung”, “Denkfaulheit” and “stehende Symptome”, which are characteristic of Pick's disease, were not ditected.

　Neuropathologically, this case was diagnosed as a temporal-orbital type of Pick's disease. However, the following atypical pathological findings were found.; 1) the appearence of many neurofibrillary tangles in the hippocampus and parahippocampal cortex, 2) severe neuronal loss with gliosis in the hippocampus, nucleus basalis of Meynert and locus ceruleus, and 3 ) bilateral pyramidal tract degeneration.