Researchers describe a disease characterized by sleep apnea, parasomnias, abnormal movements, and supranuclear palsy, with tau deposition and inflammation in the hypothalamus and brainstem.
Investigators retrospectively report clinical findings in 22 patients with IgLON5 antibodies. Mean age at diagnosis was 64 years (range, 46–83 years); gender prevalence was balanced. Most patients present with worsening symptoms over several months and an average interval of 2 to 3 years from onset to diagnosis.
The main symptom was that of a sleep disorder — sleep apnea, parasomnias, stridor, and insomnia. Parasomnias included vocalizations, limb movements, and gesturing. Most patients had bulbar symptoms and a gait disorder. Abnormal movements (in 64%) could include chorea, myoclonus, tremor, dystonia, spasms, and parkinsonism. Supranuclear gaze palsy was present in >25%. Cognitive dysfunction was observed in 41%, including dementia in mor…
Reviewing Author
DisclosuresConsultant/Advisory BoardAlexion Pharmaceuticals; Amgen; Astoria; Biogen; Bristol Myers Squibb; Celltrion; Genentech; Hoffmann-La Roche; Genzyme; EMD Serono; Immpact-Bio; Immunic Therapeutics; Kyverna; Lundbeck; Novartis; Sandoz; TG Therapeutics
Grant/Research SupportNational Institutes of Health; National Multiple Sclerosis Society; U.S. Department of Defense
Leadership Positions in Professional SocietiesConsortium of Multiple Sclerosis Centers (Treasurer)
DisclosuresConsultant/Advisory BoardAlexion Pharmaceuticals; Amgen; Astoria; Biogen; Bristol Myers Squibb; Celltrion; Genentech; Hoffmann-La Roche; Genzyme; EMD Serono; Immpact-Bio; Immunic Therapeutics; Kyverna; Lundbeck; Novartis; Sandoz; TG Therapeutics
Grant/Research SupportNational Institutes of Health; National Multiple Sclerosis Society; U.S. Department of Defense
Leadership Positions in Professional SocietiesConsortium of Multiple Sclerosis Centers (Treasurer)