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Clinical Cases and Reviews in Epilepsy

Subcortical MRI abnormalities and sudden electroclinical worsening in sporadic Creutzfeldt-Jakob Disease (CJD)

Brief report/Clinical case, 96 - 99
doi: 10.11138/ccre/2016.1.1.096
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Abstract
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Background: sporadic Creutzfeldt-Jakob Disease (sCJD) is a fatal disease, manifesting with progressive dementia, hallucination, ataxia, myoclonus, pyramidal and extrapyramidal sign. Diagnosis of sCJD requires pathologic confirmation by brain biopsy, but is suspected clinically when a patient presents with rapidly progressive dementia, motor dysfunction, and myoclonus. In the appropriate clinical setting, the combination of electroencephalogram (EEG) and Magnetic Resonance Imaging (MRI) typical findings are strongly supportive for the diagnosis. However, there are insufficient data regarding a correlation between subcortical lesions and surface EEG.
Case presentation: a 70-year-old Italian woman presented with a 40 days history of progressive memory deficits with a mild spatial disorientation.
At admission, 50 days after the onset, her neurological examination showed temporo-spatial disorientation, ideo-motor apraxia, cerebellar gait, vertical and horizontal gaze palsy and upper limb jerks. An extensive battery of routine laboratory analysis resulted normal. Lumbar puncture with CSF analysis revealed positivity for 14-3-3 and tau protein. EEG and MRI were performed at admission.
The EEG showed generalized slow background activity prevalent over the left hemisphere together with frequent fronto-temporal spikes whereas FLAIR and diffusion hyperintensity on MRI was seen over the left caudate nucleus. Two weeks later, she quickly declined showing diffuse myoclonic jerks and akinetic mutism. The repeated EEG showed a definite generalized slow background activity with periodic and bilateral sharp wave complexes while MRI FLAIR and diffusion sequences disclosed hyperintensity of the caudate nucleus bilaterally.
Conclusion: this case nicely demonstrates as the EEG abnormalities parallel with caudate nucleus FLAIR and diffusion hyperintensity spreading from unilateral to bilateral in two weeks.

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