口腔内アフタを初発症状とし，21年後に突然神経症状を呈したneuro-Behçet症候群の1剖検例

A CASE OF NEURO-BEHÇET'S SYNDROME IN A WOMAN

I．はじめに

　Behçet症候群は，1937年Behçet^2）により"ウイルスによる口腔，眼，外陰部の再発性アフタ性潰瘍"を三主徴とする独立疾患として報告された。その後，諸家により多くの症例が検討された結果，Behçet症候群は臨床的に関節，腸管，心血管系，脳神経系などの極めて多彩な症状を示す全身疾患であることが認められている^17）。その中でも，神経症状を主症状とするいわゆるneuro—Behçet症候群は予後が悪く，精神々経学的・病理学的に興味ある疾患である。

　最近，われわれは，再発性口腔内アフタを初発症状とし，21年後に突然神経症状が現れ，その後約1年の経過で死亡したneuro-Behçet症候群の一剖検例を経験した。ここに脳神経系病変の詳細な分布を検索すると共に，他の文献と比較してその所見を報告する。

An autopsy case of Neuro-Behçet's syndrome is reported with review of the previous 13 cases.

A 48-year-old woman who had recurrent oral aphthae and genital ulcers beginning at the age of 27 years suddenly developed several neurological symptoms including headache, paraplegia and dis-turbance of speech. She died of septicemia due to the urinary infection 22 years after the appearance of the first symptoms and one year after the neuro-logical manifestation.

Autopsy revealed a marked atrophy of the frontal lobes of the cerebral hemispheres. Microscopic changes in the central nervous system were con-sisted of edema and lymphocytic cuffing around the vessels, small foci of softening of the white matter and accumulation of fat-laden cells around these foci, and demyelination. These histologic lesions showed a diffuse distribution in the entire brain without a characteristic localisation, but involve-ment of the basilar nuclei and the pons was most intense in the present case as observed in most of the previous reports.

Microscopic examination of other organs revealed severe pyelonephritis and fatty changes of the liver.