前頭葉に主病変をもつSchilder病（炎症性汎発硬化症）の1剖検例

AUTOPSY CASE OF SCHILDER'S DISEASE:CHIEF PROCESS ORIGINATED IN FRONTAL LOBES OF THE BILATERAL HEMISPHERES

　19歳男子，臨床的には，11歳の時に大発作を初発症状とし，知能低下，行動異常，人格変化，多幸症などの症状が緩徐に出現し，経過中に皮膚および頭髪の脱色素症状が出現した。これらの症状は常に進行し，つづいて失語症，高度の痴呆，高度の精神機能低下，嚥下困難，末期には除脳硬直状態を呈し，昏睡におちいり，肺炎，麻痺性イレウスを併発して死亡した。全経過は8年7ヵ月であつた。脳波は特に前頭部のいちじるしい徐波化，気脳写では，側脳室前角の拡大などの所見を示した。

　剖検所見では，病変の主座は両側の前頭葉にあり，ついで頭頂葉におよび，対称性の広汎な脱髄とグリオーゼが認められ，さらに間脳，中脳および橋にも比較的新鮮な脱髄巣がみられた。脱髄巣では軸索の変性ならびに脱落がいちじるしい。前頭葉では，グリオーゼが高度で蛮症反応はみられないが，頭頂葉自質より，内包，脳幹剖の病巣においては，炎症性細胞浸潤，顆粒細胞による清掃，肥胖性グリアの増生がみられた。

　以上，前頭葉症状より始まり，8年7ヵ月の経過をとつたSchilder病の1剖検例を報告した。臨床的にいちじるしく長期にわたり，病変の主座が前頭葉にある点などが，従来の報告例と比較して異なり，特徴的である。

A 19 years old Japanese male, developed convulsion as an initial symptom at the age of 11. In the pri-mary stage, deterioration of intellectual ability, changes both in personality and behavior, and later, euphoria gradually had occurred. Psychic disturbances were presenting symptoms throughout the course. Abnormal depigmentation of the skin and the hair had been noted with course of the illness. After a progressive march of these symptoms, he developed aphasia, de-mentia, severe mental deterioration, dysphagia and quadriplegia with decerebrate rigidity in the terminal stage (Table 1).

Finally, he died of pneumonia with paralytic ileus. The total duration of illness was 8 years and 7 months.

The EEG showed diffuse slow wave, especially, in the frontal portion (Fig. 6). The PEG showed the dilatation of the anterior horns of the lateral ventricles and the third ventricle (Fig. 5). The both optic fundi were normal.

Pathoanatomically, the main lesion was localized on the bilateral frontal lobes which was characterized by idffuse demyelination and intense gliosis except for U-fibers (Fig. 1. A. & 1. B.). The demyelinated areas in the centrum semiovale, internal capsule, midbrain and pons were of relatively fresh nature (Fig. 2. A., 4. A. & 4. C.). Swelling, fragmentation and destruction of axis cylinders were rather markedly noted (Fig. 3. B-D). Perivascular cell cuffing and proliferation of both hypertropied astrocytes and granule cells were laso noticable findings (Fig. 2. C., 2. D. & 3. A.).

These findings were discussed in especial references to so called Schilder's disease from clinical and patho-logical point of view and authers concluded that the present case is compatible with the findings of the Schilder's disease, chief process being originated in frontal lobes of the both hemispheres.

In this paper, we presented an atypical case of in-flammatory diffuse sclerosis (Schilder's disease) with its main lesion being in very rare location.