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Hypocretin-1 CSF levels in anti-Ma2 associated encephalitis
S Overeem1, J Dalmau, L Bataller
1Department of Neurology, Leiden University Medical Centre, the Netherlands.
Neurology
|January 14, 2004
Summary
Idiopathic narcolepsy involves hypocretin deficiency. Anti-Ma2 encephalitis patients with sleepiness also showed low hypocretin-1, suggesting immune-mediated hypocretin dysfunction in this condition.
Area of Science:
- Neurology
- Immunology
- Sleep Medicine
Background:
- Idiopathic narcolepsy is linked to reduced hypocretin neurotransmission.
- Narcolepsy-like symptoms are observed in paraneoplastic encephalitis associated with anti-Ma2 antibodies.
Purpose of the Study:
- To investigate hypocretin-1 levels in anti-Ma2 encephalitis patients.
- To screen idiopathic narcolepsy patients for anti-Ma antibodies.
Main Methods:
- Cerebrospinal fluid (CSF) hypocretin-1 levels were measured in six anti-Ma2 encephalitis patients.
- Idiopathic narcolepsy patients were tested for anti-Ma autoantibodies.
Main Results:
- Anti-Ma autoantibodies were absent in idiopathic narcolepsy patients.
- Four anti-Ma2 encephalitis patients exhibited excessive daytime sleepiness.
- Hypocretin-1 was undetectable in the CSF of these four patients.
Conclusions:
- The findings suggest immune-mediated hypocretin dysfunction in anti-Ma2 encephalitis.
- This contrasts with idiopathic narcolepsy, where anti-Ma antibodies were not found.