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Anti-IgLON 5 Disease.

Anna Heidbreder1, Konstanze Philipp2

  • 1Institute for Sleep Medicine and Neuromuscular Disorders, University Hospital Muenster, Albert-Schweitzer- Campus 1, Building A1, 48149, Muenster, Germany. Anna.Heidbreder@ukmuenster.de.

Current Treatment Options in Neurology
|June 25, 2018
PubMed
Summary
This summary is machine-generated.

Anti-IgLON5 disease is a complex neurological disorder with varied symptoms, including sleep disturbances and movement issues. Current understanding of its pathophysiology and effective immunotherapy remains limited, requiring further research.

Keywords:
AutoimmunityNeurodegenerationObstructive sleep apneaParasomniaPolysomnography

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Area of Science:

  • Neurology
  • Immunology
  • Neurodegeneration

Background:

  • Anti-IgLON5 disease, first described in 2014, is a novel neurological disorder.
  • It presents heterogeneously with sleep, movement, neuroimmunological, and neurodegenerative features.

Purpose of the Study:

  • To provide an overview of current knowledge regarding Anti-IgLON5 disease.
  • To discuss the treatment of this novel neurological disorder.

Main Methods:

  • Review of current literature on Anti-IgLON5 disease.
  • Analysis of clinical manifestations, neuropathology, and potential genetic associations.

Main Results:

  • Clinical features include sleep disorders (parasomnia, REM sleep behavior disorder, obstructive sleep apnea), stridor, bulbar symptoms, gait abnormalities, and cognitive dysfunction.
  • Neuropathology reveals neuronal loss, gliosis, and atypical tauopathy in the brainstem and hypothalamus.
  • The mean age of diagnosis is over 60, with a balanced sex distribution. Genetic associations with HLA-DRB1*10:01 and HLA-DQB1*05:01 have been noted.

Conclusions:

  • The pathophysiology of Anti-IgLON5 disease is not fully understood and requires an interdisciplinary approach.
  • While immunotherapy has been used, its therapeutic effect is limited.
  • Further research is needed to elucidate the role of IgLON5 antibodies and improve treatment strategies.