title: A unique retinal epitheliopathy is associated with amyotrophic lateral sclerosis/Parkinsonism-Dementia complex of Guam.
abstract: BACKGROUND
  The aim of this work was to examine whether a linear retinal pigment epitheliopathy is associated with the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam.
  
  
  METHODS
  A total of 918 Guamanian Chamorros, with and without amyotrophic lateral sclerosis/parkinsonism-dementia complex, were examined cross-sectionally for linear retinal pigment epitheliopathy (LRPE). Overall, 239 Guamanians, who were neurologically asymptomatic, were followed for up to 20 years to determine the risk of developing amyotrophic lateral sclerosis/parkinsonism-dementia complex.
  
  
  RESULTS
  The epitheliopathy was present in 59.7% (117 of 196) patients with amyotrophic lateral sclerosis/parkinsonism-dementia complex, but in only 24.7% (178 of 722) of subjects who were neurologically asymptomatic (age- and sex-adjusted risk difference: 35.0%; 95% confidence interval [CI]: 27.5-42.6; p < 0.0001). Prospectively, 15 of 50 cases with epitheliopathy developed amyotrophic lateral sclerosis/parkinsonism-dementia complex, compared to 4 of 189 cases without epitheliopathy (age- and sex-adjusted hazard ratio: 13.1; 95% CI: 4.0-43.1; P < 0.0001).
  
  
  CONCLUSIONS
  Amyotrophic lateral sclerosis/parkinsonism-dementia complex is associated with an LRPE and predicts future neurological disease. Identifying the cause of this retinopathy could provide an understanding about the pathogenesis of amyotrophic lateral sclerosis/parkinsonism-dementia complex and related diseases.
authors.affiliation: Guam Memorial Hospital, Tamuning, Guam.
authors.forename: John C
authors.initials: JC
authors.lastname: Steele
authors.affiliation: Seventh-day Adventist Guam Clinic; Guam Memorial Hospital; and Loma Linda University, Loma Linda, California, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Wresch
authors.affiliation: University Eye Institute, University of Houston, Houston, Texas, USA.
authors.forename: Samuel D
authors.initials: SD
authors.lastname: Hanlon
authors.affiliation: Tropical Disease Unit, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Jay
authors.initials: J
authors.lastname: Keystone
authors.affiliation: School of Social and Community Medicine, University of Bristol, Bristol, United Kingdom.
authors.forename: Yoav
authors.initials: Y
authors.lastname: Ben-Shlomo
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000453:epidemiology / Q000473:pathology*; D003430:Cross-Sectional Studies; D003704:Dementia / Q000150:complications / Q000473:pathology*; D005260:Female; D006141:Guam / Q000453:epidemiology; D006801:Humans; D053208:Kaplan-Meier Estimate; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000150:complications / Q000175:diagnosis; D010300:Parkinson Disease / Q000150:complications / Q000473:pathology*; D012160:Retina / Q000473:pathology*; D055213:Retinal Pigment Epithelium / Q000473:pathology*; D012189:Retrospective Studies
keywords: Guam; Parkinson's disease with dementia; amyotrophic lateral sclerosis; neuroepidemiology; parkinsonism