Schilder氏病（炎症性汎発硬化症）の1剖検例

AN AUTOPSY CASE OF SCHILDER'S CEREBRAL SCLEROSIS

　典型的なSchilder氏病の1例を報告した。

　7歳9カ月の男子。臨床的に不活発，視覚聴覚の障害に始り，進行性の痴呆，四肢硬直，錐体路障害等を主症状とし，次第に増悪して約1年の経過で死亡した。髄液の蛋白増量，脳波の広汎徐波傾向を認めた。治療としてDexamethasoneを使用したが無効であつた。

　剖検により大脳半球白質の特に後頭葉に強い広汎な脱髄巣を認め，組織学的に炎症像が著明で炎症性汎発硬化症，すなわちSchilder氏病と老えられた。橋上部の孤立性脱髄巣が特に印象的であつた。組織化学的検索により診断をさらに確かめまた脳脂質の生化学的分析も行なつて，結果をCumingsらの報告と比較検討した。

An autopsy case of Schilder's cerebral scl-erosis was reported.

Clinical history:

A previously healthy boy developed the dis-ease with loss of vision and failure of hearing at the age of 6 yrs. 6 mons. These were fol-lowed by spastic paraplegia and progressive mental deterioration. Because of above symp-toms he was admitted to our hospital 7 months after the onset of the illness.

On admission rigidity of the extremities with pyramidal signs, cortical amaurosis, and sen-sory aphasia were the main neurological find-ings. Cerebrospinal fluid showed an increase of protein and in EEG dysrhythmia with slow waves was observed. On the basis of these clinical and laboratory findings, diagnosis of Schilder's disease was made. As the treatment oral administration of dexamethasone 2 mg./ day was tried, but the symptoms progressed relentlessly and after a course of about 1 year death occurred in a state of almost complete decerebrated rigidity.

Pathological findings:

The brain had normal appearance, weighing 1310g. On the frontal section the cerebral white matter showed yellow gelatinous appe-arance especially in the occipital lobe. Micro-scopically, sharply demarcated demyelination with diffuse gliosis and proliferation of the plump astrocytes and granular cells were ob-served. Perivascular lymphocytic infiltration was remarkable. In the upper part of the pons and bilateral cerebellar white matter, there were the isolated demyelinating lesions. The pyramidal tracts showed secondary degenera-tion.

Several histochemical studies showed that the myelin break-down products consited main-ly of the sudanophilic lipids, and the diagno-sis of Schilder's disease was established.

The chemical analysis of the brain lipids was performed and compared with the results of other authors.