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 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).


METHODS
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.


RESULTS
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.


CONCLUSIONS
The clinical manifestations of kuru involved a wide array of MDs during all three stages of the degenerative illness.
Kompoliti
K
K
Rush-Presbyterian-St. Luke's Medical Center, Rush University, Chicago, Illinois, USA.
Goetz
C G
CG
Gajdusek
D C
DC
Cubo
E
E
D000328:Adult; D018450:Disease Progression; D005260:Female; D006801:Humans; D007729:Kuru / Q000150:complications* / Q000453:epidemiology; D008297:Male; D009069:Movement Disorders / Q000175:diagnosis* / Q000209:etiology*; D009512:New Guinea / Q000453:epidemiology; D015995:Prevalence; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014741:Video Recording