THE NEUROPATHOLOGY OF CAG REPEAT DISEASES - REVIEW AND UPDATE OF GENETIC AND MOLECULAR-FEATURES

Classification of inherited neurodegenerative diseases is increasingly based on their genetic features, which supplement, clarify, and somet imes replace the older clinical and pathologic schemata. This change h as been particularly rapid and impressive for the CAG repeat disorders . In Huntington's disease, X-linked spinobulbar muscular atrophy, dent atorubropallidoluysian atrophy, and a series of autosomal dominant cer ebellar atrophies, genetic advances have resolved many nosologic issue s, and opened new avenues for exploration of pathogenesis, In this rev iew, we summarize classic and current concepts in neuropathology of th ese CAG repeat diseases.