title: Teaching neuroimages: Pseudohypertrophic cerebral cortex in end-stage Creutzfeldt-Jakob disease.
abstract: A 43-year-old woman presented with 1 month of progressive lower limb burning sensation, blurred vision, and gait disturbance. Her mother died of Creutzfeldt-Jakob disease (CJD). Neurologic examination revealed only cerebellar ataxia. EEG revealed periodic generalized discharges. MRI (figure 1) showed FLAIR hyperintensity in caudate and lenticular nuclei. Molecular analysis confirmed genetic CJD (PRNP E200K mutation). One month later, she became comatose. After 10 months, EEG showed diffuse background flattening without periodic abnormalities, and MRI (figure 2) disclosed diffuse pseudohypertrophy of cerebral cortex. MRI in this patient with end-stage CJD allowed us to reveal in vivo the macroscopic spongiform changes usually observed at autopsy.
authors.affiliation: Magna Græcia University of Catanzaro, Bianchi-Melacrino-Morelli Hospital, Reggio Calabria, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Gasparini
authors.forename: Edoardo
authors.initials: E
authors.lastname: Ferlazzo
authors.forename: Damiano
authors.initials: D
authors.lastname: Branca
authors.forename: Angelo
authors.initials: A
authors.lastname: Labate
authors.forename: Vittoria
authors.initials: V
authors.lastname: Cianci
authors.forename: Maria Adele
authors.initials: MA
authors.lastname: Latella
authors.forename: Umberto
authors.initials: U
authors.lastname: Aguglia
mesh_terms: D000328:Adult; D002540:Cerebral Cortex / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics / Q000473:pathology*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D009460:Neurologic Examination; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics
chemical_list: C512656:PRNP protein, human; D000072002:Prion Proteins; D011328:Prions