Multiple System Atrophy: An Update Review

Abstract

Multiple system atrophy is a progressive fatal neurodegenerative disease, characterized alpha-sunucleine accumulation mainly in oligodendroglial cells of the white matter and to a lesser degree in neurons. Autonomic and urogenital failures are the dominant clinical features. The disease is subdivided in a predominant parkinsonian type and in a predominant cerebellar atactic type. The parkinsonian type represents 80% of the patients while the cerebellar type is found in 20% of the cases. Smoking is less frequent in patients with the parkinsonian type of multiple system atrophy. On magnetic resonance imaging middle cerebellar peduncle atrophy and hyper intensity, putaminal hypointensity and hyperintense putaminal rim in addition to pons hot bun sign and atrophy are the main features. No protective treatment is available. Only supportive measures can be proposed.