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anti-IgLON5 disease
Epidemiology:
- mean age: 64 years
Clinical manifestations:
- parasomnias
- insomnia
- excessive daytime sleepiness
- sleep apnea
- gait abnormalities
- bulbar dysfunction
- dysphagia
- sialorrhea
- stridor,
- acute respiratory insufficiency
- syndrome resembling progressive supranuclear palsy
- chorea
- cognitive impairment
Laboratory:
- IgLON5 Ab in CSF
- IgLON5 Ab in serum
- IgG1 Ab in serum (> 95%)
- IgG4 Ab in serum (> 95%)
Radiology:
- magnetic resonance imaging of brain rarely shows signs of inflammation
Management:
- 1/2 of patients have mild & transient improvement with immunotherapy
- 60% of patients died during follow-up [1]
Related
Ig-like domain-containing protein ENSP00000270642 (IGLON5)
General
autoimmune disease
References
- Gaig C, Graus F, Compta Y et al.
Clinical manifestations of the anti-IgLON5 disease.
Neurology 2017 Apr 5;
PMID: 28381508
- Probasco JC
Antibody-Associated CNS Syndromes Without Inflammation in
Elders.
NEJM Journal Watch. Nov 7, 2017
Massachusetts Medical Society
(subscription needed) http://www.jwatch.org
- Escudero DEscudero D, Guasp M, Arino H et al.
Antibody-associated CNS syndromes without signs of
inflammation in the elderly.
Neurology 2017 Oct 3; 89:1471
PMID: 28878050