Summary
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 membranebound 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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Takeda, S., Ohama, E. & Ikuta, F. Adrenoleukodystrophy — Early ultrastructural changes in the brain. Acta Neuropathol 78, 124–130 (1989). https://doi.org/10.1007/BF00688199
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DOI: https://doi.org/10.1007/BF00688199