Lewy小体の多発したmultiple system atrophyの1剖検例—線条体黒質変性・オリーブ橋小脳萎縮・脊髄自律神経核変性

AN AUTOPSY CASE OF MULTIPLE SYSTEM ATROPHY WITH MANY LEWY BODIES:STRIATONIGRAL DEGENERATION, OLIVOPONTO-CEREBELLAR ATROPHY AND AUTONOMIC NERVE NUCLEI INVOLVEMENT IN THE SPINAL CORD

　抄録　症例は69歳頃より，動作緩慢，四肢筋固縮が出現し，またその頃より，起立性低血圧が出現したが，それ以外の自律神経症状や，小脳症状はほとんど認められなかった。パーキンソニズムと起立性低血圧は，徐々に進行し，72歳時死亡した。剖検では線条体黒質変性を中心とし，オリーブ橋小脳萎縮・脊髄中心外側核の神経細胞の変性・脱落などmultiple system atrophyの像を呈していた。本例に特徴的なことは，線条体黒質変性症では従来稀であるといわれていたLewy小体が，脳幹のメラニン色素含有神経細胞に比較的多数認められたことである。本例におけるLewy小体の出現の意義などについて考察した。

A 72 years old man developed slowness of the motion and orthostatic hypotension at the age of 69. Neurological examination showed slight finger tremor, rigidity of extremities, bradykinesia, and marked orthostatic hypotension. The illness pro-gressed steadily and the patient died of pneumonia.

At autopsy brain weighed 1220g. Grossly the putamen was bilaterally shrunken, the color of the substantia nigra and locus ceruleus became pale. Base of the pons and the cerebellum were atro-phic. Microscopically the most remarkable change was seen in the striato-nigral system. In the putamen, there were severe loss of small neurons and intense gliosis and brownish pigments were observed in the neuropil and within some of the astrocytes. There found neuronal loss and gliosis in the substantia nigra. A few Lewy bodies were seen in the substantia nigra. In the cerebellum there were slight loss of Purkinje cell and many torpedos were seen. There were demyelination and fibrirally gliosis in the cerebellar white mat-ter except the hilus of dentate nucleus. The trans-verse pontocerebellar fibers were degenerated and fibrirally gliosis was seen there. The inferior oli-vary nuclei showed neuronal loss and astrocytosis. But the degeneration of the olivo-ponto-cerebel-lar system in this case was not so severe as the typical case of OPCA. In the spinal cord there was depletion of nerve cells in the intermediolate-ral nuclei and Onufrowitz nuclei. Slight neuronal loss and many spheroids were observed in the anterior horns and there was demyelination in the corticospinal tracts.

To sum up, this case showed the features of striato-nigral degeneration, olivo-ponto-cerebellar atrophy, degeneration of the intermediolateral nu-clei and Onufrowitz nuclei and amyotrophic late-ral sclerosis like changes. Furtheremore this case was characterized by the presense of a lot of Lewy bodies in the pigmented nuclei in the brain stem, raphe nuclei, reticular formation and so on. These sites were the sites in which Lewy bodies were preferentially observed in idiopathic paraly-sis agitans. In multiple system atrophy, it has been said that Lewy bodies were rarely seen in the pigmented nuclei. Exceptional cases were re-ported by Thapedi and Guard. But our case show-ed more Lewy bodies in number and Lewy bo-dies distributed more extensively. We supposed that this case might combine idiopathic paralysis agitans with striato-nigral degeneration, olivo-ponto-cerebellar atrophy and the degeneration of the autonomic nerve nuclei.