title: Sleep manifestations of voltage-gated potassium channel complex autoimmunity.
abstract: OBJECTIVE
  To identify the spectrum of sleep disorders associated with autoantibodies reactive with voltage-gated potassium channel (VGKC) complexes.
  
  
  METHODS
  Case series of all patients with neurologic disorders of VGKC autoimmunity evaluated in the Mayo Clinic Center for Sleep Medicine (Rochester, Minnesota) between January 1, 1994, and February 1, 2010.
  
  
  METHODS
  Academic referral center.
  
  
  METHODS
  Fifteen consecutive patients were identified with limbic encephalitis (n = 5), Morvan syndrome (n = 4), and overlapping features (n = 6).
  
  
  METHODS
  Ten patients received immunotherapy (corticosteroids, cyclophosphamide, or mycophenolate mofetil).
  
  
  METHODS
  Response to immunotherapy.
  
  
  RESULTS
  The median VGKC autoantibody value at presentation was 1.51 nmol/L (range, 0.09-4.86 nmol/L). Neoplasms were discovered in 5 patients (33%) (thymoma [n = 2], prostate adenocarcinoma, colon adenocarcinoma, and melanoma). In 14 patients (93%), serious sleep disturbances were identified (insomnia, dream enactment behavior, suspected nocturnal epilepsy, and hypersomnia). Severe insomnia occurred in 9 patients (60%), regardless of neurologic presentation. Polysomnography at presentation (7 patients) revealed a mean sleep efficiency of 19% (4 patients had complete absence of sleep). Dream enactment behavior occurred in 8 patients (53%), including 3 of 5 with limbic encephalitis and all 4 with Morvan syndrome. Two of 7 polysomnograms demonstrated loss of rapid eye movement sleep muscle atonia; absent or minimal rapid eye movement sleep precluded interpretation in 4 patients. Sleep disorders resolved completely or almost completely in 8 of 10 patients who received immunotherapy.
  
  
  CONCLUSIONS
  Sleep disorders are cardinal manifestations of VGKC complex autoimmunity in association with a spectrum of neurologic presentations. They may respond favorably to immunotherapy.
authors.affiliation: Center for Sleep Medicine, College of Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
authors.forename: Jason R
authors.initials: JR
authors.lastname: Cornelius
authors.forename: Sean J
authors.initials: SJ
authors.lastname: Pittock
authors.forename: Andrew
authors.initials: A
authors.lastname: McKeon
authors.forename: Vanda A
authors.initials: VA
authors.lastname: Lennon
authors.forename: Paula A
authors.initials: PA
authors.lastname: Aston
authors.forename: Keith A
authors.initials: KA
authors.lastname: Josephs
authors.forename: Maja
authors.initials: M
authors.lastname: Tippmann-Peikert
authors.forename: Michael H
authors.initials: MH
authors.lastname: Silber
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000096:biosynthesis / Q000097:blood; D020274:Autoimmune Diseases of the Nervous System / Q000150:complications* / Q000188:drug therapy / Q000276:immunology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D012893:Sleep Wake Disorders / Q000175:diagnosis* / Q000276:immunology*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D024642:Potassium Channels, Voltage-Gated