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Images in Neurology |

Creutzfeldt-Jakob Disease With Focal Electroencephalographic and Magnetic Resonance Imaging Findings

K. Meng Tan, MD; Gregory A. Worrell, MD, PhD; Joseph E. Parisi, MD; Daniel H. Lachance, MD
Arch Neurol. 2007;64(4):600-601. doi:10.1001/archneur.64.4.600.
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A 77-year-old right-handed woman with no prior neurologic abnormalities presented with 2 months of progressive memory loss, aphasia, and upper limb clumsiness. Examination confirmed a severe global cognitive impairment, a predominantly expressive aphasia, bilateral ideomotor apraxia worse on the right side than the left, and an alien right upper limb with myoclonic jerks. Video electroencephalographic recording showed severe delta frequency slowing and periodic sharp waves over the left parietal head region (Figure 1A). The sharp waves were not synchronized with the myoclonic jerks. Brain magnetic resonance imaging demonstrated high-intensity signal within the left parietotemporal cortex on diffusion-weighted and fluid-attenuated inversion recovery sequences, with much less prominent but similar changes contralaterally (Figure 1B). The cerebrospinal fluid neuron-specific enolase level was markedly elevated at 112 ng/mL (reference range, <20 ng/mL). The patient's health continued to deteriorate rapidly and she died 4 weeks later. Autopsy revealed moderate generalized cerebral atrophy and histopathologic findings typical of sporadic Creutzfeldt-Jakob disease (Figure 2). Prion protein Western blot testing at the National Prion Disease Pathology Surveillance Center, Cleveland, Ohio, confirmed the diagnosis.

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Figure 1

Electroencephalographic and magnetic resonance imaging findings. A, Electroencephalography in longitudinal Laplacian montage (left-sided channels above, right-sided channels below) showing periodic left hemispheric sharp waves and delta frequency slowing, maximal in the parietal region. B, Diffusion-weighted magnetic resonance images showing bilateral parietotemporal cortical signal abnormality, maximal on the left (arrows).

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Figure 2

Histopathological analysis of the cerebral cortex showing spongiform change (arrows) on hematoxylin-eosin staining (A) and positive prion protein immunostaining (arrows) (B) (original magnification ×100).

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