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A case of Creutzfeldt-Jakob disease with stroke-like episode as an initial symptom

Posted Nov 19 2009 10:00pm
A case of Creutzfeldt-Jakob disease with stroke-like episode as an initial symptom

Kenji Kamogawa‚Pj‚Qj, Takayuki Toi‚Pj, Kensho Okamoto‚Pjand Bungo Okuda‚Pj


A 79-year-old woman was admitted to our hospital, due to acute onset of left hemiparesis and disturbance of consciousness. Although her symptoms improved temporarily, she developed gait disturbance and cognitive deterioration 2 months after the onset. After that, she presented with myoclonus and startle response, followed by akinetic mutism within 8 months after the onset. Serial EEGs revealed no periodic synchronous discharge. Serial diffusion-weighted MRIs showed that high intensity lesions, which initially limited to the right cerebral cortex, gradually spread to the bilateral cerebral cortices and basal ganglia, with relative sparing of central gyri, medial occipital cortices, and hippocampus. Prion protein gene analysis revealed a point mutation (Val¨Ile) at codon 180. The result of this patient suggests that this type of CJD might be associated with an atypical clinical course such as stroke-like episode and selective involvement of cortical and subcortical resions. Key words: Creutzfeldt-Jakob disease, Prion-protein, Codon180, MRI, Stroke-like onset (Nippon Ronen Igakkai Zasshi 2009; 46: 458\461) 1jDepartment of Neurology, Ehime Prefectural Central Hospital 2jDepartment of Geriatric Medicine, Medicine and Bioscience, Graduate School of Medicine, Ehime University

Thursday, November 05, 2009

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification

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