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PAS-positive intracytoplasmic material is evident in multiple neurons, and biochemical assays identify a deficiency in iduronate-2-sulfatase activity in cultured dermal fibroblasts

Specifically, analyzed were the affected brain areas (04), cerebral (NTP-7, Level 3), cerebellar cortex (NTP-7, Level 6), and hippocampus, thalamus, and hypothalamus (NTP-7, Level 3) as follows (score 0 indicates no histopathologic change): score 1: small, patchy, complete, or incomplete infarcts (10% of the area affected)

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Yuan Y, Feng G, Qin W, et al