Multiple System Atrophy (MSA)
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
- PDF / 198,201 Bytes
- 24 Pages / 439.37 x 666.142 pts Page_size
- 83 Downloads / 184 Views
97
Gregor K. Wenning, Florian Krismer, and Sid Gilman
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 a-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.
Introduction Multiple system atrophy (MSA) is an adult-onset rapidly progressive neurodegenerative disorder characterized by abnormal accumulation of glial cytoplasmic inclusions (GCIs) accompanied by multifocal neuronal degeneration. The disease is characterized clinically by either parkinsonism, usually unresponsive or poorly
G.K. Wenning (*) • F. Krismer Division of Clinical Neurobiology, Department of Neurology, Medical University, Anichstrasse 35, A-6020 Innsbruck, Austria e-mail: [email protected], [email protected] S. Gilman Department of Neurology, University of Michigan, 300 N. Ingalls 3D15, 48109-0489 Ann Arbor, MI, USA e-mail: [email protected] M. Manto, D.L. Gruol, J.D. Schmahmann, N. Koibuchi, F. Rossi (eds.), 2119 Handbook of the Cerebellum and Cerebellar Disorders, DOI 10.1007/978-94-007-1333-8_97, # Springer Science+Business Media Dordrecht 2013
2120
G.K. Wenning et al.
responsive to carbidopa/levodopa, or cerebellar ataxia in association with autonomic failure, orthostatic symptoms, and urinary dysfunction. Pyramidal signs may occur as well (Wenning et al. 2004; Gilman et al. 2008). Until recently, patients with MSA were classified as one of three different disorders depending on the main clinical presentation: olivopontocerebellar atrophy (OPCA, Dejerine and Thomas 1900), striatonigral degeneration (SND, Adams et al. 1961), and Shy Drager Syndrome (SDS, parkinsonism with autonomic failure, Shy and Drager 1960). The term multiple system atrophy was first used in 1969 when Graham and Oppenheimer provided evidence that OPCA, SND, and SDS show substantial overlap in both clinical and neuropathological features and appear to represent a single disease (Graham and Oppenheimer 1969). Twenty years later, Papp et al. (1989) demonstrated argyrophilic GCIs in neuropathological specimens of patients with MSA irrespective of clinical presentation (Papp et al. 1989). Argyrophilic GCIs became the neuropathological hallmark of the disease. Deposits of GCIs are accompanied by neuronal loss in the basal ganglia, cerebellum, pons, inferior olivary nuclei, and spinal cord
Data Loading...
