Neurol Sci (2017) 38:1535–1537 DOI 10.1007/s10072-017-2968-8 LETTER TO THE EDITOR Sporadic Creutzfeldt-Jakob disease presenting with isolated progressive non-fluent aphasia in a young woman 1 1 2 1 Alberto Terrin & Andrea Barp & Gianluigi Zanusso & Paolo Gallo & Annachiara Cagnin Received: 20 February 2017 /Accepted: 18 April 2017 /Published online: 25 April 2017 Springer-Verlag Italia 2017 Introduction Probable sCJD was diagnosed in vivo with detection of prion protein (PrP) seeding activity through Real-Time Quaking- Creutzfeldt-Jakob disease (CJD) belongs to a family of neu- Induced Conversion (RT-QuIC) in CSF and olfactory mucosa rodegenerative diseases known as transmissible spongiform (OM) samples. encephalopathies or prion diseases. Sporadic (sCJD), the most common form, occurs at an incidence of 1/100.0000/year. Onset usually occurs in the seventh decade of life, and life Case report expectancy is about 5 months. Clinically, sCJD is character- ized by a rapidly progressive dementia, myoclonus, visual, or A 47-year-old woman was admitted to the Neurological cerebellar disturbances, pyramidal or extrapyramidal signs, Clinic, Department of Neurosciences, in Padova (Italy) for and akinetic mutism . Brain MRI can support the clinical rapidly progressive language impairment in the previous diagnosis of sCJD showing signal hyperintensity at the month. At admission, neurological evaluation
Neurological Sciences – Springer Journals
Published: Apr 25, 2017
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