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  • Title: Adrenoleukodystrophy--early ultrastructural changes in the brain.
    Author: Takeda S, Ohama E, Ikuta F.
    Journal: Acta Neuropathol; 1989; 78(2):124-30. PubMed ID: 2750483.
    Abstract:
    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.
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