…(1) Demyelinating diseases caused by lipid metabolism disorders include the aforementioned hypomyelination, but because myelin is lost over a wide area of the cerebral white matter, they are called leukodystrophy. However, there are other diseases for which the defective enzymes in lipid metabolism disorders have been confirmed, such as metachromatic leukodystrophy (arylsulfatase A deficiency) and Krabbe's disease (galactocerebrosidase deficiency). Adrenoleukodystrophy, previously described as Schilder's disease, is also thought to be caused by lipid metabolism disorders. … *Some of the terminology that refers to "metachromatic leukodystrophy" is listed below. Source | Heibonsha World Encyclopedia 2nd Edition | Information |
…(1)脂質代謝異常による脱髄疾患には,前述の髄鞘形成不全というべきものが含まれているが,大脳白質の広い範囲にわたって髄鞘が消失するため,白質ジストロフィーleukodystrophyと呼ばれている。しかし脂質代謝異常の欠損酵素が確定しているものとしては,異染性白質ジストロフィー(アリルスルファターゼA欠損症)や,クラッベ病Krabbe’s disease(ガラクトセレブロシダーゼ欠損症)などがある。また従来シルダー病Schilder’s diseaseとして記載されていた副腎白質ジストロフィーadrenoleukodystrophyも脂質代謝異常によるものと考えられている。… ※「異染性白質ジストロフィー」について言及している用語解説の一部を掲載しています。 出典|株式会社平凡社世界大百科事典 第2版について | 情報 |
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