From: Probable Creutzfeldt-Jakob disease—a case report at Suez Canal University Hospital, Egypt
Definite | Detection of protease-resistant prion protein or scrapie-associated fibrils by neuropathology, immunochemical technique, and/or Western blot. |
Probable | No findings indicating alternative diagnoses AND progressive dementia with at least 2 of (i)–(iv) AND at least one of (a)–(c). |
Possible | No findings indicating alternative diagnoses AND progressive dementia with duration of less than 2 years AND with at least 2 of (i)–(iv) AND at least one of (a)–(c). |
(i) Myoclonus | |
(ii) Visual or cerebellar problems | |
(iii) Pyramidal or extrapyramidal features | |
(iv) Akinetic mutism | |
(a) Periodic sharp wave complexes on electroencephalography | |
(b) Positive 14-3-3 protein in the cerebrospinal fluid with a disease duration of less than 2 years | |
(c) High signal abnormalities in the caudate nucleus and/or putamen on diffusion-weighted imaging (DWI) or fluid-attenuated inversion recovery (FLAIR) MRI |