Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias – Vitali et al. Neurology 76 May 17, 2011. CJD. degenerative neurological disorder that is incurable and invariably fatal due to a type of infectious protein called a prion
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Neurology 76 May 17, 2011
61 CJD (npRPD) disorders have similar presentation
48 sCJD (43/48 tested for PRNP mutation ie fCJD – all negative)
41 path proven
7 non-pathology proven cases, met WHO criteria
7 GSS (Gerstmann–Sträussler–Scheinker syndrome)
14/29 tested for PRNP: all negative
15/29 not pathologically proven, but included
survival was outside the range of sCJD subjects (> 3 years)
definitive (e.g., serological or genetic) evidence of another disorder
and/or they improved with (or without) treatment.