Multiple system atrophy (MSA)

Abstract

Multiple system atrophy (MSA) is a sporadic, rapidly progressive neurodegenerative disorder characterized clinically by a combination of cerebellar dysfunction or parkinsonism and autonomic failure. The histopathological hallmark of MSA is the presence of glial cytoplasmic inclusions composed predominantly of α-synuclein within multiple systems of the brain and the spinal cord accompanied by gliosis and neuronal loss. In the past few years there has been substantial progress in the understanding of the pathogenesis of this fatal neurodegenerative disease. The most recent studies indicate that this is a primary oligodendroglial disorder with synucleinopathy leading to subsequent neurodegeneration. The authors review the epidemiology, clinical features, diagnostic criteria, neuropathology, pathogenesis, management and therapeutic approaches to this disease.