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Multiple System Atrophy

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Abstract

Multiple system atrophy (MSA) is a slowly progressive neurodegenerative disease that is characterized by (i) a hypokinetic movement disorder, which is predominant in MSA of the parkinsonian type (MSA-P), or by (ii) cerebellar symptoms, which prevail in MSA of the cerebellar type (MSA-C). In both types, the movement disorder is accompanied by autonomic symptoms such as orthostatic hypotension or erectile dysfunction. The effect of dopaminergic medication on the parkinsonian symptoms is very limited. The disease progression is more rapid compared to Parkinson’s disease and is characterized by falls early in the disease course, as well as changes in articulation (dysarthria), swallowing (dysphagia), and the occasional stridor that can also help differentiate MSA-P from Parkinson’s disease.

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Abbreviations

CPAP:

Continuous positive airway pressure

FDG-PET:

Fluorodeoxyglucose positron emission tomography

GCIs:

Glial cytoplasmic inclusions

GNIs:

Glial nuclear inclusions

GWAS:

Genome-wide association studies

LBD:

Dementia with Lewy bodies

L-threo-DOPS:

L-threodihydroxy-phenylserine

MSA:

Multiple system atrophy

MSA-C:

MSA of the cerebellar type

MSA-P:

MSA of the parkinsonian type

MSCs:

Mesenchymal stem cells

NCIs:

Neuronal cytoplasmic inclusions

NNIs:

Neuronal nuclear inclusions

OH:

Orthostatic hypotension

PD:

Parkinson’s disease

PSP:

Progressive supranuclear palsy

rhGH:

Recombinant human growth hormone

SNCA:

α-Synuclein gene

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Levin, J., Maaß, S., Schuberth, M., Höglinger, G. (2017). Multiple System Atrophy. In: Falup-Pecurariu, C., Ferreira, J., Martinez-Martin, P., Chaudhuri, K. (eds) Movement Disorders Curricula. Springer, Vienna. https://doi.org/10.1007/978-3-7091-1628-9_17

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