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Progression and prognosis in multiple system atrophy presenting with REM behavior disorder
- Source :
- Neurology. 94:e1828-e1834
- Publication Year :
- 2020
- Publisher :
- Ovid Technologies (Wolters Kluwer Health), 2020.
-
Abstract
- ObjectivesTo investigate (1) the prevalence of REM sleep behavior disorder (RBD) as mode of disease onset in a cohort of patients with multiple system atrophy (MSA) and (2) disease progression and prognosis in patients with MSA with RBD predating (pre-RBD) and following (post-RBD) disease onset.MethodsWe retrospectively identified all patients with a clinical diagnosis of MSA evaluated at least once a year during the disease course. Type of onset was defined by the first reported motor or autonomic symptom/sign related to MSA. The occurrence of symptoms/signs and milestone of disease progression, and their latency from disease onset, were collected. Survival data were calculated. RBD was confirmed by video-polysomnography.ResultsOf a total of 158 patients, pre-RBD represented the mode of disease onset in 27% of patients, preceding disease onset according to the international criteria with a median of 3 (2–5) years. Comparing pre-RBD and post-RBD patients, the first group showed an increased prevalence of autonomic onset of disease, a reduced prevalence of parkinsonism, an earlier onset of stridor, pyramidal signs, symptomatic orthostatic hypotension, urinary dysfunction, severe dysphagia, and wheelchair dependency. The risk of death was higher in patients with pre-RBD.ConclusionsIn our MSA cohort, RBD represented the most frequent mode of disease presentation. A more rapid progression of disease was observed in the pre-RBD group. These findings suggested a careful assessment of sleep disorders to early recognize RBD and a closer follow-up of autonomic dysfunction and stridor in patients with pre-RBD.
- Subjects :
- Male
Pediatrics
medicine.medical_specialty
Stridor
REM Sleep Behavior Disorder
Disease
REM sleep behavior disorder
03 medical and health sciences
Orthostatic vital signs
0302 clinical medicine
Atrophy
Prevalence
medicine
Humans
Aged
Retrospective Studies
030214 geriatrics
business.industry
Parkinsonism
Retrospective cohort study
Middle Aged
Multiple System Atrophy
Prognosis
medicine.disease
Disease Presentation
Disease Progression
Female
Neurology (clinical)
Multiple system atrophy Autonomic diseases Cohort studies Natural history studies (prognosis) REM sleep behaviour disorder
medicine.symptom
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 1526632X and 00283878
- Volume :
- 94
- Database :
- OpenAIRE
- Journal :
- Neurology
- Accession number :
- edsair.doi.dedup.....b11331b551bf87df4195ba332f9f1386