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December 1972

Kuru-Like Neuropathological Changes in a North American

Author Affiliations

Canada; Bronx, NY
From the Department of Pathology, the Saul R. Korey Department of Neurology (Drs. Powers and Schaumburg), and the Rose F. Kennedy Center for Mental Retardation and Human Development, Albert Einstein College of Medicine (Dr. Horoupian), Bronx, NY. Dr. Horoupian is on sabbatical leave from the Winnipeg General Hospital, Canada.

Arch Neurol. 1972;27(6):555-561. doi:10.1001/archneur.1972.00490180091018

Kuru is a fatal progressive disease of the central nervous system with clinical manifestations of ataxia, dementia, and myoclonus. So far, kuru and Creutzfeldt-Jakob disease (C-J) are the only two forms of transmissible subacute spongiform viral encephalopathy known to affect humans. All of the reported cases of kuru have occurred in a restricted area of New Guinea, while Creutzfeldt-Jakob has had a wide distribution. We have examined postmortem tissue from a 54-year-old Jamaican woman with an 11-month progressive ataxic and dementing illness, clinically diagnosed as the ataxic form of C-J. Light and electron microscopic examination of the brain showed the distribution and changes described in kuru, including the presence of numerous PAS-positive kuru plaques in the cerebellum.

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