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Multiple system atrophy presenting as central sleep apnoea
L J Cormican1, S Higgins, A C Davidson
1Dept of Respiratory Medicine, St. Thomas' Hospital, London, UK. liam.cormican@gstt.sthames.nhs.uk
The European Respiratory Journal
|August 31, 2004
Summary
Multiple system atrophy (MSA) can present as central sleep apnoea. This neurodegenerative disorder involves autonomic, cerebellar, and extrapyramidal dysfunction, often appearing later in the disease progression.
Area of Science:
- Neurology
- Sleep Medicine
- Neurodegenerative Diseases
Background:
- Central sleep apnoea (CSA) is a sleep disorder characterized by the absence of respiratory effort.
- Idiopathic CSA is diagnosed when no underlying cause is identified.
- Multiple system atrophy (MSA) is a progressive neurodegenerative disorder affecting multiple systems in the brain.
Observation:
- A 61-year-old male initially presented with idiopathic central sleep apnoea.
- Over four years, he developed autonomic, cerebellar, and extrapyramidal dysfunction.
- These symptoms were consistent with a diagnosis of multiple system atrophy (MSA).
Findings:
- Central sleep apnoea is a less common manifestation of MSA compared to obstructive sleep apnoea.
- CSA in MSA may result from the depletion of cholinergic neurons in the medullary arcuate nucleus via apoptosis.
- This case represents the first documented instance of MSA presenting primarily with central sleep apnoea.
Implications:
- Multiple system atrophy should be considered in the differential diagnosis of late-onset central sleep apnoea.
- This presentation highlights the importance of recognizing atypical neurological signs in sleep disorders.
- Further research is needed to understand the mechanisms linking MSA and central sleep apnoea.