Pathologic Features

Grossly, the brain shows variable atrophy of the convolutions, subcortical gray structures, and hemispheric white matter (Fig. 7.1). The histologic hallmark of the disease is microvacuolation and spongiform degenera

FIGURE 7.1

Creutzfeldt-Jakob disease. A. Severe diffuse cerebral atrophy. B. Cerebral cortex showing microvacuolation and neuronal losses and (C) prominent astrocytic proliferation (HE).

tion of the cerebral and cerebellar cortex and, variably, of the subcortical gray structures. The vacuoles are small, round or oval, often coalescing, and occurring in all cortical layers. Neuronal losses and astrocytosis are prominent (see Fig. 7.1). PrP precipitates as amyloid plaques in the cerebral and cerebellar cortex and, variably, in the deep gray structures. Immunohistologic stain identifies various forms of PrP deposits, including dense plaques (Kuru plaque); multicentric plaques; and granular deposits around neurons, synapses, and blood vessels and in vascular walls. In hematoxylin-eosin (HE)-stained sections, the plaques appear as homogenous eosinophilic structures.

Variants of CJD are (a) Heidenhain disease is distinguished clinically by cortical blindness and histologi-cally by spongiosis and neuronal losses, predominantly in the occipital cortex (Fig. 7.2); (b) the ataxic form shows prominent cerebellar cortical degeneration (Fig. 7.3); (c) the panencephalopathic form entails additional degeneration of the white matter; and (d) the amyotrophic and polyneuropathic forms may introduce the disease or develop during its course. PrP deposits

Histopatologi Cerebellum

FIGURE 7.2

Heidenhain variant of CJD. A 56-year-old woman presented with progressive visual loss, myoclonic jerks, and dementia. She died 5 months after onset of symptoms. Microvacuolation in the occipital cortex (HE).

FIGURE 7.2

Heidenhain variant of CJD. A 56-year-old woman presented with progressive visual loss, myoclonic jerks, and dementia. She died 5 months after onset of symptoms. Microvacuolation in the occipital cortex (HE).

are found in the spinal cord gray matter and peripheral nerves.

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