Kompoliti K, Goetz C G, Gajdusek D C, Cubo E
Rush-Presbyterian-St. Luke's Medical Center, Rush University, Chicago, Illinois, USA.
Mov Disord. 1999 Sep;14(5):800-4. doi: 10.1002/1531-8257(199909)14:5<800::aid-mds1012>3.0.co;2-x.
To describe the gamut of movement disorders (MD) seen during the clinical course of kuru.
Kuru is a subacute spongiform encephalopathy that was confined to several adjacent cultures in the Eastern Highlands of New Guinea and resulted from contamination with brain tissue during the ritual endocannibalism practiced in those societies. This unique neurologic disease was recorded extensively with film between 1957 and 1976, and these comprehensive research documents have been donated to the American Academy of Neurology archives by one of the authors (DCG).
The comprehensive assembly of film record of kuru, which was collected by one of the authors (DCG) was reviewed. This comprised two parts: The first were films from 1957-1964 and included 17.397 ft of 16-mm film featuring 204 patients (children and adults); the second is assembled from films made from 1967-1976 and includes 9138 ft. of film featuring 47 adult patients. Two MD specialists categorized all MDs observed and a representative videotape was produced.
Tremor is the most frequently encountered MD in kuru and is typically of the action/intention type, which appears early in the disease and is soon associated with other clinical signs of cerebellar dysfunction. Widespread clonus is characteristic of advanced disease and can be difficult to differentiate from tremor. Dystonia/athetosis and choreiform jerks also appear as the disease progresses. Dystonia can involve the torso, distal limbs, neck, or jaw. Myoclonic jerks can be superimposed on the cerebellar or dystonic features usually with an enhanced startle response. Parkinsonian symptomatology, other than resting tremor is frequent among the filmed subjects especially in the second stage of the disease.
The clinical manifestations of kuru involved a wide array of MDs during all three stages of the degenerative illness.
描述库鲁病临床病程中出现的运动障碍范围。
库鲁病是一种亚急性海绵状脑病，局限于新几内亚东部高地的几个相邻文化群体，是由于这些社会中实行的仪式性食人习俗导致脑组织污染所致。1957年至1976年间，这部独特的神经疾病被大量拍摄记录下来，这些全面的研究资料已由其中一位作者（DCG）捐赠给美国神经病学学会档案馆。
回顾了由其中一位作者（DCG）收集的库鲁病电影记录的全面汇编。这包括两部分：第一部分是1957 - 1964年的影片，包括17397英尺的16毫米胶片，记录了204名患者（儿童和成人）；第二部分是1967 - 1976年拍摄的影片汇编，包括9138英尺胶片，记录了47名成年患者。两名运动障碍专家对观察到的所有运动障碍进行了分类，并制作了代表性录像带。
震颤是库鲁病中最常见的运动障碍，通常为动作性/意向性震颤，在疾病早期出现，很快与小脑功能障碍的其他临床体征相关联。广泛的阵挛是晚期疾病的特征，可能难以与震颤区分开来。随着疾病进展，肌张力障碍/手足徐动症和舞蹈样抽搐也会出现。肌张力障碍可累及躯干、远端肢体、颈部或下颌。肌阵挛性抽搐通常叠加在小脑或肌张力障碍特征上，通常伴有增强的惊吓反应。在拍摄的受试者中，除静止性震颤外，帕金森样症状很常见，尤其是在疾病的第二阶段。
在库鲁病这种退行性疾病的所有三个阶段中，其临床表现涉及广泛的运动障碍。
