Movement disorders in kuru

OBJECTIVE: To describe the gamut of movement disorders (MD) seen during the clinical course of kuru. BACKGROUND: Kuru is a subacute spongiform encephalopathy that was confined to several adjacent cultures in the Eastern Highlands of New Guinea and res ulted from contamination with brain tissue during the ritual endocannibalis m practiced in those societies. This unique neurologic disease was recorded extensively with film between 1957 and 1976, and these comprehensive resea rch documents have been donated to the American Academy of Neurology archiv es by one of the authors (DCG). METHODS: The comprehensive assembly of film record of kuru, which was colle cted by one of the authors (DCG) was reviewed. This comprised two parts: Th e first were films from 1957-1964 and included 17,397 ft of 16-mm film feat uring 204 patients (children and adults); the second is assembled from film s made from 1967-1976 and includes 9138 ft. of film featuring 47 adult pati ents. Two MD specialists categorized all MDs observed and a representative videotape was produced. RESULTS: Tremor is the most frequently encountered MD in kuru and is typica lly of the action/intention type, which appears early in the disease and is soon associated with other clinical signs of cerebellar dysfunction. Wides pread clonus is characteristic of advanced disease and can be difficult to differentiate from tremor. Dystonia/athetosis and choreiform jerks also app ear as the disease progresses. Dystonia can involve the torso, distal limbs , neck, or jaw. Myoclonic jerks can be superimposed on the cerebellar or dy stonic features usually with an enhanced startle response. Parkinsonian sym ptomatology, other than resting tremor is frequent among the filmed subject s especially in the second stage of the disease. CONCLUSION: The clinical manifestations of kuru involved a wide array of MD s during all three stages of the degenerative illness.