Abstract
Multiple system atrophy (MSA) is an adult-onset, sporadic, progressive neurodegenerative disease, characterized by varying severity of parkinsonian features, cerebellar ataxia, autonomic failure, urogenital dysfunction, and corticospinal disorders. In the past, MSA has been known as olivopontocerebellar atrophy, Shy-Drager syndrome, and striatonigral degeneration.
Consensus criteria retain the diagnostic categories of (1) MSA with predominant parkinsonism (MSA-P) and (2) MSA with predominant cerebellar ataxia (MSA-C) in order to emphasize the predominant motor features. Dysautonomia is a constant and is usually a presenting feature in both types. These criteria also retain the designations of definite, probable, and possible MSA. Definite MSA requires neuropathologic demonstration of alpha-synuclein-positive glial cytoplasmic inclusions with neurodegenerative changes in striatonigral or olivopontocerebellar structures.
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References
Bhidayasiri R, Ling H. Multiple system atrophy. Neurologist. 2008;14:224–37.
Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008;71:670–6.
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The patient exhibits jerky myoclonus (“minipolymyoclonus”) of the fingers with both hands outstretched. Bradykinesia is present for rapid finger tapping and hand movements. Facial masking, reduced blink frequency, global akinesia, and a mild Pisa syndrome with body tilt to the right are present.
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Bhidayasiri, R., Tarsy, D. (2012). Multiple System Atrophy. In: Movement Disorders: A Video Atlas. Current Clinical Neurology. Humana, Totowa, NJ. https://doi.org/10.1007/978-1-60327-426-5_15
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DOI: https://doi.org/10.1007/978-1-60327-426-5_15
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