anti-IgLON5 disease
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Epidemiology
- mean age: 64 years
Clinical manifestations
- parasomnias
- 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]
More general terms
Additional terms
References
- ↑ 1.0 1.1 Gaig C, Graus F, Compta Y et al. Clinical manifestations of the anti-IgLON5 disease. Neurology 2017 Apr 5; PMID: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/28878050