側頭葉の著明な萎縮と多数の神経細胞内封入体を認めた多系統萎縮症長期経過例

An Autopsy Case of Long-course Multiple System Atrophy（MSA）with Remarkable Atrophy and Numerous NCI in the Temporal Lobe.

要旨　死亡時66歳男性。51歳パ-キンソニズムで発症し，多系統萎縮症（MSA） の症状と画像に加えて，早期から痴呆と進行性の側頭葉萎縮を認めた。脳重量1,115gで，MSAの典型的病理所見とともに，前頭葉と側頭葉の強い萎縮がみられ，海馬と海馬傍回を中心に多数の神経細胞内封入体（NCI）が出現していたが，他の変性疾患の合併を示唆する所見はなかった。MSA長期例の大脳萎縮は前頭葉優位が多いが，稀ながらNCIを伴う側頭葉萎縮を呈する一亜型が存在するものと考えられた。

The patient was 66 year-old man with no family history of neurological diseases. At age 51, he showed initial symptom of parkinsonism, and was revealed a cerebellar atrophy by CT at age 52. He was suffered from malignant syndrome followed by renal dysfunction, which needed hemodialysis therapy. At age 54, he admitted to our hospital, when he showed parkinsonism, ataxia and dysautonomia. Neuroimaging study disclosed typical findings of multiple system atrophy （MSA）, and dilatation of inferior horn of the lateral ventricle. In the next year, he lost of amburatory function, and showed low cognitive function of 5 scores in HDSR. At age 57, he was tracheostomised because of complete paralysis of the vocal cord abductor muscles. MRI study disclosed marked temporal lobe atrophy. He was complicated with bladder carcinoma, and died of multiple organ failure at age 66.

　The brain weight was 1,115g. Gross neuropathological findings were temporal lobe dominant cerebral atrophy and marked pontocerebellar atrophy. Meningitis and sepsis were seen in the cerebrum, and some infarctions in the temporal and frontal lobes. Besides the typical degenerative findings of MSA, remarkable temporal lobe atrophy with enlargement of the inferior horn of lateral ventricle was observed. There were numerous number of neuron containing neuronal inclusion body （NCI）in the hippocampal cortex, dentate fascia and parahippocampal gyrus. In spite of small amount of NFT in the parahippocampal gyrus, there were no senile plaque, tau-positive structure except NFT, argyrophilic grain or Pick body.

　This case was a long-survived MSA with remarkable atrophy of the temporal lobe. The characteristic neuropathological finding was numerous numbers of neurons containing NCI in the cotices arround the inferior horn. Although frontal lobe dominant cerebral lobe atrophy is common in the long-survived MSA cases,e xtreme temporal lobe atrophy is rare condition. There may be a MSA subtype strongly affecting temporal lobe with numerous NCI.