Adrenoleukodystrophies ALDs

Adrenoleukodystrophies (ALDs) (described in detail in section on peroxisomal diseases) are characterized by a failure of degradation of saturated very long-chain fatty acids (VLCFAs), which then accumulate in the cerebral white matter, adrenals, and practically all tissues. Neonatal ALD, is autosomal recessive and results from a disorder of peroxisomal biogenesis. Childhood, juvenile, and adult ALDs are X-linked recessive and result from single-enzyme defects. The pathology, common to all forms, is distinguished by lymphocytic perivascular infiltrations in the demyelinated areas (Fig. 9.10).

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