Abstract
Multiple system atrophy (MSA) is a rare, progressive, fatal, neurodegenerative disorder. There are two main types: the parkinsonian type (MSA-P) and cerebellar type (MSA-C). The disease usually presents with genitourinary dysfunction, orthostatic hypotension, and rapid eye movement (REM) sleep behavior disorder. Patients rapidly develop balance, speech, and coordination abnormalities. We present a review of the clinical picture and the actualized treatment modalities of the MSA cerebellar type. For the study methods, a PubMed search was done using the following medical subject headings (MeSH) terms: “multiple system atrophy/therapy". Inclusion criteria included studies in English, full papers, human studies, and publications in the last 30 years. Case reports and series were excluded. A total of 157 papers were extracted after applying the inclusion and exclusion criteria, and 41 papers were included for the discussion of this review. This review underlines the therapeutic strategies as well as the clinical picture of multiple system atrophy, and how MSA-C and MSA-P differ from each other. We discussed this review in four topics: ataxia, autonomic dysfunction (neurogenic orthostatic hypotension and urinary disorders), parkinsonism, and REM sleep disorder. In conclusion, the treatment of MSA-C is mainly symptomatic; there are not many studies on MSA-C. The ataxic component and fewer parkinsonian symptoms are the main difference of MSA-C as opposed to MSA-P.
Highlights
BackgroundMultiple system atrophy (MSA) is a part of the family of α-synucleinopathies
We present a review of the clinical picture and the actualized treatment modalities of the MSA cerebellar type
We discussed this review in four topics: ataxia, autonomic dysfunction, parkinsonism, and rapid eye movement (REM) sleep disorder
Summary
Multiple system atrophy (MSA) is a part of the family of α-synucleinopathies. The disease is rapidly progressive and has two main types: multiple system atrophy parkinsonian type (MSA-P) and multiple system atrophy cerebellar type (MSA-C) [1]. The incidence and prevalence of this disease are 0.6 to 0.7 cases per 100,000 and 3.4 to 4.9 cases per 100,000 population, respectively [2]. The average age of diagnosis is between 55 and 60 years, and the average survival from the onset of motor symptoms is eight to nine years [3]. Cerebellar atrophy is due to olivopontocerebellar atrophy degeneration and, to a lesser extent, striatonigral degeneration (SND) [1]. Cerebellar ataxia is the main distinctive feature of the MSA-C phenotype [2]
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