New Variant CJD

In 1994 and1995, a little less than 10 years after the outbreak of BSE in Europe, a new variant of CJD (nvCJD) was recognized in the United Kingdom and France. The disease is distinguished by an earlier onset of behavioral changes and psychiatric symptoms, followed by cerebellar dysfunction and myoclonus. Later in the course, dementia develops. The course is protracted. Bilateral pulvinar hyperintensities on MRI are held to be characteristic. Because PrPsc is detectable in lymphoid tissue, immunohistochemical demonstration of PrP in tonsillar biopsy supports the diagnosis. Widespread spongy degeneration and an abundance of florid

Leptomeningitis Purulenta

FIGURE 7.3

Ataxic-cerebellar variant of CJD. A 65-year-old woman presented with headaches, speech difficulty, gait impairment, and for-getfulness. Her symptoms steadily progressed. Her gait became severely ataxic, she displayed dysmetria in all extremities, and myoclonic jerks. Six months after the initial symptoms, she died. The cerebellum shows (A) severe diffuse cortical and white matter atrophy; (B) diffuse loss of Purkinje cells, rarefaction of granular cells layer, and microvacuolation in the molecular layer (HE); and (C) microvacuolation, neuronal loss and astrocytosis in basal ganglia (HE).

Microvacuolation

FIGURE 7.3

Ataxic-cerebellar variant of CJD. A 65-year-old woman presented with headaches, speech difficulty, gait impairment, and for-getfulness. Her symptoms steadily progressed. Her gait became severely ataxic, she displayed dysmetria in all extremities, and myoclonic jerks. Six months after the initial symptoms, she died. The cerebellum shows (A) severe diffuse cortical and white matter atrophy; (B) diffuse loss of Purkinje cells, rarefaction of granular cells layer, and microvacuolation in the molecular layer (HE); and (C) microvacuolation, neuronal loss and astrocytosis in basal ganglia (HE).

prion plaques (dense central cores surrounded by amyloid fibrils and outer spongiform halos) in the cerebral and cerebellar cortex characterize the histology. A causal association between this nvCJD and the consumption of meat products contaminated with BSE agent is gaining support. It has been shown that the PrPsc type in nvCJD is identical to that found in cattle infected with BSE.

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