正染性白質変性症の1剖検例

AN AUTOPSY CASE OF LEUCODYSTROPHY WITH SIMPLE PRELIPID BREAKDOWN PRODUCTS

緒言

　大脳白質の広範な硬化を主病変とする汎発性硬化症には炎症型—Schilder病と変性型—Leucodystropyが含まれる。しかしSchilder病は多発性硬化症，視束脊髄炎，Balo病などに近縁の疾患として考えられている。そもそもLeucodystrophyという言葉はBielschowskyand Henneberg^1）が今日の異染性汎発硬化症にLeuco—dystrophia cerebri progressiva hereditariaに対して用いたのであるが，その後それが一般的な概念となり，異染性の有無にかかわらず，いわゆる変性形汎発硬化症を総称するようになつた。

　変性型白質変性症はDiezel^2），Peiffer^3），Richardson^4），　Poser^5），Hallervorden^6）等により種々の分類が試みられている。

A girl, 31 years old, whose illness started at 12-13 years of age with headache and mental deterio-lation, was admitted for urinary infection. Physical examination revealed bradylalia, disturbances in articulation, mental deteriolation, less active body movement and increased deep tendon reflex of the right upper and lower extremities. In the final stage she showed nystagmus, spastic paralysis of the extremities, positive bilateral Babinski's sign and urinary and fecal incontinence. She died of status epilepticus.

Pathologically, widespread demyelination associ-ated with gliosis was observed in the white matter of the cerebral cortex. U-fibers were intact from demyelination. These changes were most promi-nent in the frontal lobe and spongy change was found around the anterior horn. Perivascular in-flammatrory cell infiltration was almost absent. A few microglial phagocytes and free fatty granules were only observed in the perivascular areas where demyelination took place. Histochemically these glanucles were not metachromatic and were con-sidered to be prelipid