Neuro-Behçet症候群の1剖検例

AN AUTOPSY CASE OF NEURO-BEHÇET'S SYNDROME

I．はじめに

　Behcet症候群に精神神経症状が合併すること（Neuro—Behcet症候群，以下N-B症候群）はよく知られており，このN-B症候群の臨床および神経病理学的所見に関しては，かなりの数の報告がなされている。

　ここに述べるN-B症候群の剖検例は，神経病理学的には脳幹に主座をもつ慢性播種性脳炎の像を呈し，臨床的には四肢不全麻痺に加えて無動，無言というlocked—in syndromeあるいはakinetic mutismともみなされる稀な症状を呈した。しかし本論文では，間脳，脳幹部で作製した連続切片標本で得た組織所見を主に述べ，最後に我々が経験したperivenous encephalitis （以下P．E）と比校したい。

An autopsy case of Neuro-Behçet's syndrome isreported. The patient was a 34-year-old man withneurological symptomes in addition to Behçet'ssyndrome which was characterized by recurrentoral and genital ulceration and bilateral optic atro-phy for approximately five years before death.

Neurological symptoms consisted of the brain-stem symptoms associated with pyramidal signs andalso of a peculiar clinical state resembling locked-in syndrome or akinetic mutism.

Microscopic examination of the central nervoussystem revealed chronic disseminated encephalitiswith ill-defined demyelination, perivascular cuffingand the proliferation of micro- and astroglia andmesodermal rod-like cells, involving mainly thediencephalon and the brain-stem, especially themotor tracts. No obvious abnormality was seen inin the cerebral cortex which includes the hippo-campal formation and in the white matter of thehemispheres except for part of the left temporallobe.

The demyelination was most extensive in themid-brain, while perivascular cuffing was mostconspicuous in the diencephalon. From pathologicaland clinical findings, the demyelination was con-sidered to be secondary change to inflammatoryreaction.

This syndrome was compared with the perivenousencephalitis which we examined recently, for thelatter is said to be pathologically similar to theformer. But we found that in Neuro-Behçet'ssyndrome perivascular cuffing mainly consisted oflymphocytes, in perivenous encephalitis of the cellsconsidered to be of mesodermal or adventitial origin.

The etiology of Neuro-Behçet's syndrome is ob-scure. However, from pathological findings, it issuggested that there is an etiopathogenetic differ-ence between this syndrome and perivenous ence-phalitis.