Leukodystrophy with Diffuse Rosenthal Fiber Formation（Alexander's Disease）の1剖検例

Case of Leukodystrophy with Diffuse Rosenthal Fiber Formation (Alexander's Disease)

I．緒言

　本疾患ははなはだ稀でその剖検例は世界でも10例以下で，本邦ではいまだその発表例をみていない。家族性で乳幼時に発生するといわれ，脳は一般に非常に大きくて重い（megalobarencephaly）。顕徴鏡的にはとくに髄膜下および血管周囲にエオジン強染の細長い構造物の集積を認める。これはAlexander^2）によりfibrinoid，Crome^5）によりhyaiin body，さらにHallervorden^7）によりRosenthal fiberと呼ばれたもので，膠細胞の代謝障害による産物であるとされている。われわれは，生後より脳症状を呈した乳児に本疾患剖検例を経験したので報告する。

　This rare condition has been given various names characterized by abundant deposits of amorphous material in the form of round, ovoid and elongated bodies specially around the blood vessels and below the pia matter.

　This 1 year and 3 months old boy had marked internal hydrocephalus with ventricular empyema. The brain weighs 880 grams. There are massive accumulation of Rosenthal fibers specially at the perivascular and subpial areas and small amount of Rosenthal fiber accumulation is scattered in the cerebral white matter throughout, where demyelination and dysmyelination are found to be more diffuse. The enlarged fibrous glias contain coarsely granulated material having a similar staining characters to that of Rosenthal fiber. In the brain stem the myelin sheath is fairly well preserved with exception of the perivascular areas where the myelin is compressed and sometimes demyelinized. It is most likely that metabolic derangement of astrocytes is basic factor of this illness and disorder of myelin sheath could be secondary.