Autosomal recessive juvenile parkinsonism

Autosomal recessive juvenile parkinsonism (AR-JP) is a hereditary neurodege nerative disorder characterized by levodopa-responsive parkinsonism with on set before age 40 years and a slowly progressive course. Families with this condition have been described predominantly in Japanese population, occasi onally under different names including an autosomal recessive early-onset p arkinsonism with diurnal fluctuation (AR-EPDF) or a familial form of juveni le parkinsonism. Recently, the causative gene for AR-JP was mapped on chrom osome 6q25.2-q27, and subsequently a novel gene 'parkin' was identified by means of positional cloning. In this manuscript, we review the clinical, pa thological and genetical aspects of AR-JP. It would not only promise to pro vide important insights into the molecular mechanisms of selective degenera tion of dopaminergic neurons in AR-JP, but also bring insights into the mec hanisms of degeneration of these neurons in Parkinson's disease. (C) 2000 E lsevier Science B.V. All rights reserved.