Pathologic staging of white matter lesions in adult-onset leukoencephalopathy/leukodystrophy with axonal spheroids

摘要: The pathologic features of adult-onset leukoencephalopathy/leukodystrophy with axonal spheroids (ALAS) are variable, and this has led to different hypotheses as whether primarily demyelination or axonopathy may underlie disorder. Typical ALAS pathology is rarely accompanied by focal multiple sclerosis (MS)–like plaques. In (MS)-like plaques cases, the cannot be distinguished from those progressive MS diffusely abnormal white matter. To clarify these issues, we examined neuropathologic in 159 representative samples 5 cases (3 men 2 women aged 39–61 years) 95 3 chronic (1 man 50–73 years). matter abnormalities were characterized evolving stages: 1) numerous a background well-myelinated fibers; 2) moderate loss myelinated fibers sparse number spheroids; 3) leukodystrophy-like pattern confluent myelin loss. application staging system suggests that preceded axonopathy. could attributed both primary Some predominant difficult distinguish ALAS.