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Shy-Drager syndrome presenting as a REM behavioral disorder
B A Wright1, J R Rosen, D J Buysse
1Department of Psychiatry, Geriatric Health Services, University of Pittsburgh, School of Medicine, PA 15213.
Journal of Geriatric Psychiatry and Neurology
|April 1, 1990
Summary
Shy-Drager syndrome (SDS) can manifest with sleep disturbances, specifically REM behavioral disorder (RBD). This case report highlights RBD as a potential early indicator of SDS, preceding other symptoms by a decade.
Area of Science:
- Neurology
- Sleep Medicine
- Autonomic Dysfunction
Background:
- Shy-Drager syndrome (SDS), also known as multiple system atrophy (MSA), is a rare neurodegenerative disorder.
- SDS is characterized by autonomic failure, Parkinsonism, and cerebellar dysfunction.
- Sleep disturbances are recognized symptoms of SDS, but presentation with a primary sleep abnormality is not well-documented.
Observation:
- This case report details a patient diagnosed with SDS.
- The patient's initial presentation, approximately ten years prior to SDS diagnosis, was a progressive sleep disorder.
- Polysomnography confirmed a specific sleep disturbance: REM behavioral disorder (RBD).
Findings:
- This is the first reported case of SDS presenting with REM behavioral disorder (RBD) as the initial symptom.
- The patient's RBD preceded other clinical manifestations of SDS by a significant period.
- This suggests RBD may be an early prodromal sign of SDS.
Implications:
- REM behavioral disorder (RBD) may serve as an early diagnostic marker for Shy-Drager syndrome (SDS).
- Early identification of RBD could lead to earlier diagnosis and management of SDS.
- Further research is warranted to explore the link between RBD and the pathogenesis of SDS.