Further Observations on the Pathology of Kuru*: A Study of the Two Cerebra in Serial Section

Abstract

It is now 9 years since the first description of Kuru as a subacute degenerative disease of the central nervous system afflicting the Fore and neighboring peoples of the eastern New Guinea Highlands was published by Zigas and Gajdusek (1957) (12, 29). The etiology and pathogenesis of this disease (1–3, 6–18, 21, 24, 28) remains obscure (8, 9), but the recently reported successful transmission of a kuru-like syndrome to chimpanzees (11) suggests that a microbial, possibly a viral, agent may be involved. The histopathology of the disorder was studied by Klatzo, Gajdusek and Zigas (17, 18) and by other workers (2, 6, 21, 22, 26) in some 40 cases, and the principal anatomic lesions were described as a degeneration and a loss of neurons, microglial proliferation and fibrous astrocytosis, status spongiosus in the cerebral cortex and basal ganglia, variable white matter lesions (loss of myelinated fibers), and cortical cerebellar degeneration. Characteristic plaques were described as an outstanding feature in half of the cases, and moderate perivascular cuffing by mononuclear cells was noted. In the present study, based on the examination of 2 specimens in gapless serial section, we can corroborate most of the previous histopathological findings and, in addition, report several features not previously emphasized, such as the very wide distribution of the typical plaques observed in the white matter, as well as in the cerebral and cerebellar cortex and basal ganglia, and note for the…