Progressive Subcortical Gliosisの1例—Pick病の司法鑑定例から

A CASE OF PROGRESSIVE SOBCORTICAL GLIOSIS

I．はじめに

　初老期痴呆の1つとされているPick病^{2,3,4,13,14）}の病理学所見は，前頭葉及び側頭葉の限局性萎縮，大脳皮質の神経細胞の脱落，Pick細胞，嗜銀球の出現，及び皮質下白質を中心としたGlioseを特徴としている。しかし，1949年，Neumannら^8）は，臨床的にはPick病あるいはAlzheimer病様の所見を呈しながらも，病理学的にはこれと異なり，脳萎縮は軽度で，大脳皮質神経細胞の変性も乏しく，一方，大脳半球白質に軽度のびまん性脱髄，大脳，脳幹，延髄，脊髄に及ぶ広範囲なGlioseを特徴的な所見とする症例を見い出し，それをPick病のII型と呼び，1967年，これら疾患をProgressivesubcortical gliosis^9）と命名した。本邦では，これまで2例の報告がある。しかし，それらの臨床像はAlzheimer病（皆川ら^6）），および，初老期痴呆（調ら^12））であつた。著者らは，臨床的には，司法鑑定により，Pick病と診断したが，組織病理学的にProgressive subcorticalgliosisと考えられる1例を経験した。しかし著者らの狩猟した限りではわが国において，Pick病の殺人鑑定例の報告は見当らない^5,7）。従つて，この面からも本症例の臨床・病理を報告することは，意義あることと考える。

A-54-year-old man was admitted to the TomobeHospital, Ibaraki Prefecture, in February 1967, forthe purpose of Psychiatric Evidence on the murder.

At the time of admission there were characterchange, memory impairment, perseveration, disori-entation and amnestic aphasia. His clinical picturewas considered as Pick's disease. Gradually, hemanifested an organic dementia and finally diedof Ileus on September 1970.

Total duration of illness was approximately 8years. Autopsied findings are as follows ; Macro-scopically, the leptomeninges on the left frontallobe were turbid, and brown pigments were seenin the area from the frontal pole to the centralgyrus.

There were mild atrophies in both frontal andtemporal lobes bilaterally.

The lateral and third ventricles showed milddilatation. Microscopically, diffuse incomplete de-myelination and marked gliosis occured in the whitematter of bilateral hemispheres.

Gliosis was also seen elsewhere, i. e. the hypo-thalamus, substantia nigra, inferior olives andanterior horns of the spinal cords.

On the cerebral cortices some nerve cells wereshrunk, particularly on the 3 rd layer, while cyto-architecture was relatively well preserved.

Occasionaly, some nerve cells were swollen andladen with Lipofuscin granules.

From the above described histopathological find-ings, this case was considered as Progressive sub-cortical gliosis.(Neumann et al.)

Authors discussed on relationship between Pick'sdisease and Progressive subcortical gliosis.