Okumiya Kiyohito, Wada Taizo, Fujisawa Michiko, Ishine Masayuki, Garcia Del Saz Eva, Hirata Yutaka, Kuzuhara Shigeki, Kokubo Yasumasa, Seguchi Harumichi, Sakamoto Ryota, Manuaba Indrajaya, Watofa Paulina, Rantetampang Andreas L, Matsubayashi Kozo
Research Institute for Humanity and Nature, Kyoto, Japan.
BMJ Open. 2014 Apr 16;4(4):e004353. doi: 10.1136/bmjopen-2013-004353.
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.
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.
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.
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.
自1962 - 1981年盖杜谢克及其同事进行调查以来，印度尼西亚巴布亚仅开展过一项关于肌萎缩侧索硬化症（ALS）和帕金森症的随访研究。因此，为阐明巴布亚南部沿海地区ALS和帕金森症的临床流行病学情况，对该地区这些疾病的临床特征和患病率进行了检查和评估。
在2001 - 2012年对印度尼西亚巴布亚伊亚河、埃德拉河、杜穆特河和奥巴阿河沿岸的巴德及其他村庄进行的一项调查中，对ALS和帕金森症病例进行了临床检查。经认证的神经科医生根据埃尔埃斯科里亚尔标准对可能、很可能及确诊的ALS进行临床诊断。帕金森症的诊断标准为存在以下四个体征中的至少两个：震颤、强直、运动迟缓以及姿势障碍，且病程呈进行性。
在调查期间，在7000人（2001 - 2002年）至13900人（2007 - 2012年）的人群范围内诊断出46例ALS和/或帕金森症病例。这46例病例包括17例很可能 - 确诊的ALS病例，其中3例伴有认知障碍（CI）；13例重叠的可能、很可能或确诊的ALS和帕金森症病例，其中5例伴有CI；以及16例帕金森症病例，其中1例伴有CI。2007年或部分地区的2010年，单纯ALS的粗点患病率估计至少为73（95%可信区间0至156）至133（27至240）/10万人，重叠的ALS和帕金森症的粗点患病率至少为53（0至126）至98（2至193）/10万人。
虽然在过去约30 - 35年中，巴布亚ALS的患病率有所下降，但仍高于全球平均水平。ALS、帕金森症和CI重叠的患病率较高，此前在关岛和纪伊也有过相关报道。
