title: Amyotrophic lateral sclerosis and parkinsonism in Papua, Indonesia: 2001-2012 survey results.
abstract: OBJECTIVE
  Only one previous follow-up study of amyotrophic lateral sclerosis (ALS) and parkinsonism in Papua, Indonesia has been carried out since a survey undertaken in 1962-1981 by Gajdusek and colleagues. Therefore, to clarify the clinical epidemiology of ALS and parkinsonism in the southern coastal region of Papua, the clinical characteristics and prevalence of the diseases in this region were examined and assessed.
  
  
  METHODS
  Cases of ALS and parkinsonism were clinically examined during a 2001-2012 survey in Bade and other villages along the Ia, Edera, Dumut and Obaa rivers in Papua, Indonesia. Possible, probable and definite ALS was diagnosed clinically by certified neurologists based on El Escorial criteria. The criteria for a diagnosis of parkinsonism were the presence of at least two of the four following signs: tremor, rigidity, bradykinesia and postural impairment with a progressive course.
  
  
  RESULTS
  During the survey, 46 cases of ALS and/or parkinsonism were diagnosed within a population range of 7000 (2001-2002) to 13 900 (2007-2012). The 46 cases consisted of 17 probable-definite cases of ALS, including three with cognitive impairment (CI), 13 cases of overlapping possible, probable or definite ALS and parkinsonism, including five with CI, and 16 cases of parkinsonism, including one with CI. The crude point prevalence rate of pure ALS was estimated to be at least 73 (95% CI 0 to 156) to 133 (27 to 240)/100 000 people and that of overlapping ALS and parkinsonism at least 53 (0 to 126) to 98 (2 to 193)/100 000 in 2007, or 2010 in some regions.
  
  
  CONCLUSIONS
  While the prevalence of ALS in Papua has decreased over the past ∼30-35 years, it remains higher than the global average. There was a high prevalence of overlapping ALS, parkinsonism and CI, which has also been previously reported in Guam and Kii.
authors.affiliation: Research Institute for Humanity and Nature, Kyoto, Japan.
authors.forename: Kiyohito
authors.initials: K
authors.lastname: Okumiya
authors.forename: Taizo
authors.initials: T
authors.lastname: Wada
authors.forename: Michiko
authors.initials: M
authors.lastname: Fujisawa
authors.forename: Masayuki
authors.initials: M
authors.lastname: Ishine
authors.forename: Eva
authors.initials: E
authors.lastname: Garcia Del Saz
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Hirata
authors.forename: Shigeki
authors.initials: S
authors.lastname: Kuzuhara
authors.forename: Yasumasa
authors.initials: Y
authors.lastname: Kokubo
authors.forename: Harumichi
authors.initials: H
authors.lastname: Seguchi
authors.forename: Ryota
authors.initials: R
authors.lastname: Sakamoto
authors.forename: Indrajaya
authors.initials: I
authors.lastname: Manuaba
authors.forename: Paulina
authors.initials: P
authors.lastname: Watofa
authors.forename: Andreas L
authors.initials: AL
authors.lastname: Rantetampang
authors.forename: Kozo
authors.initials: K
authors.lastname: Matsubayashi
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000453:epidemiology*; D002648:Child; D003072:Cognition Disorders / Q000453:epidemiology; D005260:Female; D006306:Health Surveys; D006801:Humans; D007214:Indonesia / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D020734:Parkinsonian Disorders / Q000453:epidemiology*; D015995:Prevalence; D012737:Sex Factors; D055815:Young Adult
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