Amyotrophic lateral sclerosis and parkinsonism in Papua, Indonesia: 2001-2012 survey results

作者：Okumiya Kiyohito^*; Wada Taizo; Fujisawa Michiko; Ishine Masayuki; del Saz Eva Garcia; Hirata Yutaka; Kuzuhara Shigeki; Kokubo Yasumasa; Seguchi Harumichi; Sakamoto Ryota; Manuaba Indrajaya; Watofa Paulina; Rantetampang Andreas L; Matsubayashi Kozo

来源：BMJ Open, 2014, 4(4): e004353.

DOI：10.1136/bmjopen-2013-004353

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. %26lt;br%26gt;Methods Cases of ALS and parkinsonism were clinically examined during a 2001-2012 survey in Bade and other villages along the Ia, Edera, Dumutand Obaa rivers in Papua, Indonesia. Possible, probableand 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, bradykinesiaand postural impairment with a progressive course. %26lt;br%26gt;Results During the survey, 46 cases of ALS and/or parkinsonism were diagnosed within a population range of 7000 (2001-2002) to 13900 (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)/100000 people and that of overlapping ALS and parkinsonism at least 53 (0 to 126) to 98 (2 to 193)/100000 in 2007, or 2010 in some regions. %26lt;br%26gt;Conclusions While the prevalence of ALS in Papua has decreased over the past approximate to 30-35years, it remains higher than the global average. There was a high prevalence of overlapping ALS, parkinsonismand CI, which has also been previously reported in Guam and Kii.

• 出版日期2014