Multiple system atrophy

Abstract

Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder that usually manifests in the early fifties and progresses relentlessly with a mean survival of 9 years. Clinically, MSA is dominated by autonomic/urogenital failure which may be associated with either levodopa unresponsive parkinsonism in 80p.cent of cases (MSA-P subtype) or with cerebellar ataxia in 20p.cent of cases (MSA-C subtype). Pathologically, MSA is characterized by a neuronal multisystem degeneration and abnormal glial cytoplasmic inclusions containing alpha-synuclein aggregates. Pharmacological treatment of motor features is disappointing except for a transient L-Dopa response in a minority of MSA-P patients. In contrast, autonomic and urogenital features of MSA should be identified early on because they can be treated effectively in many instances. To improve the outcome of this devastating disorder which causes early severe and progressive disability more research into etiology, pathogenesis, early diagnosis and therapeutic management is necessary. Several research groups have been formed in Europe (EMSA-SG, NNIPPS) and USA (NAMSA-SG) to address these unmet needs.