Open AccessThe Neuropathology of CAG Repeat Diseases: Review and Update of Genetic and Molecular Features
Author(s) -
Robitaille Yves,
LopesCendes Iscia,
Becher Mark,
Rouleau Guy,
Clark Arthur W.
Publication year - 1997
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/j.1750-3639.1997.tb00893.x
Subject(s) - neuropathology , disease , neuroscience , atrophy , medicine , huntington's disease , pathology , biology , bioinformatics
Classification of inherited neurodegenerative diseases is increasingly based on their genetic features, which supplement, clarify, and sometimes replace the older clinical and pathologic schemata. This change has been particularly rapid and impressive for the CAG repeat disorders. In Huntington's disease, X‐linked spinobulbar muscular atrophy, dentatorubropallidoluysian atrophy, and a series of autosomal dominant cerebellar atrophies, genetic advances have resolved many nosologic issues, and opened new avenues for exploration of pathogenesis. In this review, we summarize classic and current concepts in neuropathology of these CAG repeat diseases.