Anti IgLON5 antibody syndrome: management

Evidence-based neurology checklist on anti iglon5 antibody syndrome: management: Pathogenesis This is a tauopathy It is caused by IgG4 or IgG1 antibodies to IgLON5 IgLON5 is a neuronal cell adhesion protein HLA allele risk factors Pathology Magnetic resonance imaging (MRI) brain Other…

Pathogenesis

  • This is a tauopathy
  • It is caused by IgG4 or IgG1 antibodies to IgLON5
  • IgLON5 is a neuronal cell adhesion protein

HLA allele risk factors

Pathology

Magnetic resonance imaging (MRI) brain

Other investigations

Treatment

Predictors of treatment response

References

  1. Sabater L, Gaig C, Gelpi E, et al. A novel non-rapid-eye movement and rapid-eye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: a case series, characterisation of the antigen, and post-mortem study. Lancet Neurol 2014; 13:575-586. 
  2. Schröder JB, Melzer N, Ruck T, et al. Isolated dysphagia as initial sign of anti-IgLON5 syndrome. Neurol Neuroimmunol Neuroinflamm 2016; 4:e302.
  3. Gaig C, Graus F, Compta Y, et al. Clinical manifestations of the anti-IgLON5 disease. Neurology 2017; 88:1736-1743. 
  4. Honorat JA, Komorowski L, Josephs KA, et al. IgLON5 antibody: Neurological accompaniments and outcomes in 20 patients. Neurol Neuroimmunol Neuroinflamm 2017; 4:e385. 
  5. Gelpi E, Höftberger R, Graus F, et al. Neuropathological criteria of anti-IgLON5-related tauopathy. Acta Neuropathol 2016; 132:531-543. 
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