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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Adrenoleukodystrophy (ALD, AMN) | 1C2 Adrenoleukodystrophy (Case 1) LFB-PAS 6
1C2,3 The perivascular collections of cells are typically rich in lymphocytes. The histologic appearance of the CNS is similar to the other leuko-dystrophies except that perivascular cuffing by lymphocytes (chiefly CD8+ T cells) is unique among leukodystrophies and may superficially resemble MS. It has been proposed that damage occurs in a 2-step process: the first, dysmyelination related to VLCFA-produced myelin instability; and, the second, the result of an inflammatory demyelinative process in which CD8+ T cells, oligodendrocyte lysis, macrophages, and a variety of cytokines (especially tumor necrosis factor-alpha, TNF-a) participate.
