Sudanophilic Leucodystrophy凍結保存脳の脂質・脂酸構成について

SUDANOPHILIC LEUCODYSTROPHY:STUDY ON LIPID, FATTY ACID COMPOSITION AND GANGLIOSIDE PATTERN IN BRAIN

はじめに

　脂質症の原因は，生化学，特に脂質分析の進歩にともない，つぎつぎに解明されてきている。大脳白質に非炎症性の広範なびまん性の脱髄巣を形成するいわゆるle—ucodystrophyに関しても，metachromatic leucodystro—phy,globoid cell leucodystrophy （Krabbe's disease）が脂質代謝における酵素欠損によるよることが明らかにされた。今までの臨床的，形態学的な検索による分類も，原因の解明とともに，酵素，分子のレベルにおける分類へと変わりつつあるといえよう^{10）19）20）}。

　sudanophilic leucodystrophyに関する生化学的な検索は，2,3報告されているにすぎず，まだその原因も明らかでない。わたくし達は，sudanophiiic leucodystro—phyの一症例を経験した。この症例の病理組織学的検索については，調らによりすでに本誌に報告された¹²。さらに，生化学的検索として，この症例の凍結保存脳について，脂質，脂酸構成，ガングリオシドの構成について検討し若干の知見を得たので報告する。

Chemical studies were done on cerebral gray and white matter of a case of sudanophilic leucodystro-phy.

A patient was a 33 years-old woman. At the age of 30 years, she became dull and hesitant with housekeeping. Next year, she became incontinent of urine and gait disturbance. Within 2 years after onset, she became akinetic and mutistic. There were bilateral hyperreflexia, clonus with Babinski, Chaddock responses and tonic seizure. She continued to be febrile with temperature up to 40°C and gradually was emaciated. The interval between onset and death was 3 years and 7 months.

Frontal lobe was kept at -20℃ for 7 months before analysis. The results of chemical analysis were as follows :

1. Amount of total cholesterol :

Total cholesterol in gray matter remained un-changed, but in white matter markedly decreased.

2. Relative lipid composition :

In white matter, significant amount of esterified choresterol and decrease of cerebroside were found. However, as a whole, relative lipid composition in gray and white matter were relatively close to normal. Trigliceride was absent in both gray and white matter.

3. Ganglioside pattern :

G 3 ganglioside moderately decreased in both gray and white matter. Moreover, a gold-brownlich unidentified component was found between G 3 and G 2 gangliosides. This finding raise an interesting question if it is a special component of sudanophilic leucodystrophy.

4. Fatty acid composition of each lipid :

In white matter, there were marked decrease oftong chain fatty acids in sphingolipids, particularly in cerebroside.

From these results and discussion of literatures, the metabolic disturbances of galactolipids, parti-cularly of cerebroside, on the level of utilization of galactose or elongation process of fatty acids may be concerned with the etiology of sudano-philic leucodystrophy.