Golbe L I
Department of Neurology, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School, New Brunswick.
J Neural Transm Suppl. 1994;42:263-73. doi: 10.1007/978-3-7091-6641-3_20.
The age-adjusted prevalence of PSP as measured in central New Jersey is 1.5 cases per million population, about 1% of that of Parkinson's disease. It incidence is 3-4 new cases per million population per year, similar to that of such better-known illnesses as myasthenia gravis, the hereditary ataxias as a group and Tourette syndrome. Median actuarially adjusted survival after symptom onset is 5.9-6.9 years. PSP appears to favor no geographical, racial, ethnic or occupational group, though there is anecdotal evidence for hydrocarbon exposure as a candidate etiologic factor. No familial cases of typical PSP have been proven. The one formal case-control study failed to implicate any particular causal agent and the rural predilection of PD appears to be absent in PSP. Better diagnostic methods, more multi-center organization, additional case-control studies and new etiologic hypotheses are needed in the epidemiological investigation of PSP.
在新泽西州中部测量的经年龄调整的进行性核上性麻痹(PSP)患病率为每百万人口1.5例,约为帕金森病患病率的1%。其发病率为每年每百万人口3 - 4例新发病例,与重症肌无力、遗传性共济失调群体和图雷特综合征等更为知名的疾病相似。症状出现后经精算调整的中位生存期为5.9 - 6.9年。PSP似乎不存在地域、种族、民族或职业倾向,不过有传闻证据表明接触碳氢化合物可能是病因因素。尚未证实有典型PSP的家族病例。一项正式的病例对照研究未能发现任何特定的致病因素,而且PSP似乎不存在帕金森病那样的农村地区高发倾向。在PSP的流行病学调查中,需要更好的诊断方法、更多的多中心组织、更多的病例对照研究以及新的病因假说。