Adrenoleukodystrophy--early ultrastructural changes in the brain.
A light and electron microscopic study was performed on the cerebral white matter in a case of adrenoleukodystrophy (ALD) with peculiar symptoms of olivopontocerebellar atrophy. The affected white matter on the light microscope had many macrophages containing characteristic membrane-bound linear inclusions. The unaffected white matter on the light microscope demonstrated the following ultrastructural changes: (1) slight but definite degeneration of myelin sheaths scattered among apparently normal myelinated axons; (2) oligodendroglia-like cells containing membrane-free intracytoplasmic inclusions; and (3) many swollen astrocytes containing the same membrane-bound linear inclusions as those in the macrophages within the affected white matter. The mechanism of demyelination in ALD is also discussed.
Takeda
S
S
Department of Pathology, Niigata University, Japan.
Ohama
E
E
Ikuta
F
F
D000326:Adrenoleukodystrophy / Q000473:pathology*; D001921:Brain / Q000648:ultrastructure*; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000473:pathology*; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged