Anti IgLON5 antibody syndrome: treatment

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Anti IgLON5 antibody syndrome: treatment. Methylprednisolone, Plasma exchange (PE), Rituximab. Fully referenced neurology checklist.

Treatment

  • Methylprednisolone
  • Plasma exchange (PE)
  • Rituximab
  • Intravenous immunoglobulins (IVIg)
  • Adjunctive cyclophosphamide: case report

Predictors of treatment response

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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. Grüter T, Gaig C, Crijnen YS, et al. Early treatment with intravenous immunoglobulins and outcomes of patients with anti-IgLON5 disease. JAMA Neurol 2025; 82:1040-1047.
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