title: Moyamoya disease in a primarily white, midwestern US population: increased prevalence of autoimmune disease.
abstract: OBJECTIVE
  Moyamoya disease is an uncommon, cerebrovascular occlusive disease of unknown pathogenesis. Previously described Moyamoya cohorts include predominantly Asian populations or ethnically diverse North American cohorts. To gain further insight into the pathogenesis of moyamoya, we examined clinical characteristics of a primarily white, Midwestern US population
  
  
  METHODS
  Retrospective analysis of patients with angiographically confirmed moyamoya disease evaluated at our institution was performed. Prevalence of comorbidities, cerebrovascular risk factors, and autoimmune diseases were compared with the general population.
  
  
  RESULTS
  Ninety-four patients with moyamoya were evaluated; 72.3% were female. Ethnic composition was primarily white (85%). A significantly higher prevalence of autoimmune disease was seen, particularly type 1 diabetes mellitus (8.5% versus 0.4% in the general population) and thyroid disease (17.0% versus 8.0% in the institutional general patient population). Hyperlipidemia was also increased (27.7% versus 16.3% in the general population).
  
  
  CONCLUSIONS
  This study of a unique, primarily white, Midwestern population of moyamoya patients demonstrates a significantly higher prevalence of autoimmune disease than in the general population. This supports a possible autoimmune component to the pathogenesis of moyamoya disease.
authors: Department of Neurosurgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors: Regina S
authors: RS
authors: Bower
authors: Grant W
authors: GW
authors: Mallory
authors: Macaulay
authors: M
authors: Nwojo
authors: Yogish C
authors: YC
authors: Kudva
authors: Kelly D
authors: KD
authors: Flemming
authors: Fredric B
authors: FB
authors: Meyer
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000453:epidemiology*; D015897:Comorbidity; D003922:Diabetes Mellitus, Type 1 / Q000453:epidemiology; D005260:Female; D006801:Humans; D006949:Hyperlipidemias / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015146:Midwestern United States / Q000453:epidemiology / Q000208:ethnology; D009072:Moyamoya Disease / Q000453:epidemiology* / Q000209:etiology; D015995:Prevalence; D013959:Thyroid Diseases / Q000453:epidemiology; D044465:White People / Q000208:ethnology*; D055815:Young Adult
keywords: immunology; moyamoya; pathogenesis; risk factor