汎発性硬化症（Schilder病）の1例

A Case of Diffuse Cerebral Sclerosis (Schilder's Disease)

I．まえがき

　近年多発性硬化症を中核とする脱髄性脳疾患は臨床家並びに病理学者の関心を集めており，1957年の国際神経病理学会ではその中心課題の一つにあげられた。我が国に於ても内村，白木，春原の狂犬病予防注射後の精神神経障害の臨床病理学的研究，冲中，黒岩，椿らの多発性硬化症及び近縁疾患の系統的検索などから，脱髄性疾患一般に対する理解が著しく深められた。

　汎発性硬化症は多発性硬化症と近縁関係をもつ疾患で，Adams and Kubik^1）によるとSchilderがその基本像を示して以来欧米諸国では150余例の報告があるといわれる。しかし，本邦ではこの疾患も多発性硬化症と同様数が少なく，わずかに稲田（昭9）^12），石井，横井（昭34）^11），山県（昭34）^35）の報告があるにすぎない。従つて，我国の経験例について，その臨床経過及び各種検査成績を詳細に報告することは意味のあることであろう。

A 9-year-old boy was admitted to hospital inJan. 1958, because of inactiveness, impairedvision, slurring of speach and unsteady gait. Theonset of the illness was assumed to be six months.ago.

On admission he was frivolous, inattentive andunsteady. By neurological examination were foundvisual disturbances (Balints sign), ataxia andparesis in the left extremity. No referable incid-ence could be explored in the family and pasthistory. On Feb. 2, he had an convulsive seizureand ensuing coma episode, thereafter, the mental.deteroration made rapid progress. He becameunresponsive to sensory stimuli, showed bilateralspastic paralysis with positive Babinskis sign.The cerebrospinal fluid, of which pressure elevatedslightly, showed positive globulin reaction but no,increase in cell count. An air study of ventriclesdemonstrated a symmetrical dilatation of minordegree and an arteriogram of cerebral vesselswas normal. On the EEG were disclosed gener-alized irregular slow waves which werer pedomi-nant in the temporal areas. The patient died inApril in a state of decerebrate rigidity.

The autopsy revealed the brain of normal look,.weighing 1,352g. Sectioning of the brain throughthe posterior horns of the lateral ventricles disce-oesed diffuse foci of transluscent gelatinous appear-ance in the central and convolutional white matterexcepting narrow subcortical zone.

Microscopically, the lesions were characterizedby a diffuse bilateral pallor in the cerebral whitematter in myelin staining, which occupies prin-cipally the occipital and temporal lobes excludingthe arcuate fibers beneath the cortex. The lesionsshowed a concomitant destruction of axis cylindersassociated with an intense proliferation of gittercells, astrocytic elements and of glial fibers, andwas also accompanied by perivascular inflamma-tory reaction consisting of lymphocytes and plasmacells. The cortex was spared from pathologicalchanges on the whole. The demyelinating processappeared spreading forward into temporal andparietal lobes, basal ganglia and corpus callosum.The optic, acoustic and olfactory nerves were alsofound undergoing degeneration.

Histochemical examinations of lipids revealednormal myeline degradation. A secondary demy-elination of the pyramidal fiber tracts and tem-poropontine tracts of both sides could be followedto the levels of midbrain pons, medulla oblongataand spinal cord.

The histological findings were summarized asdiffuse cerebral sclerosis of inflammatory type ofSchilder.