title: Illustrating the relevance of updated diagnostic criteria for sporadic Creutzfeldt-Jakob disease: a teaching neurocase.
abstract: A 75-year-old woman with unremarkable medical history, consulted for a 5-month history of involuntary shaking of left upper limb. Clinical examination revealed polyminimyoclonus of the upper limbs with cogwheel-like rigidity, hyperreflexia, bradykinesia, inconstant spastic-like rigidity in the lower limbs and a stiff and cautious gait. These symptoms, together with the memory impairment found on neuropsychological assessment yielded suspicion for a subacute encephalopathy probably due to a non-conventional infectious agent. There was no 14-3-3 protein found in the cerebrospinal fluid and no periodic sharp wave complexes on EEG. These findings made the diagnosis of Creutzfeldt-Jakob disease (CJD) rather unlikely according to the current WHO diagnostic criteria. However, typical isolated cortical hyperintensity of right temporal, parietal and occipital lobes on MRI suggested a probable CJD and prompted cerebral biopsy which confirmed the diagnosis. This article emphasises the need to update the current WHO criteria by including radiological findings.
authors.affiliation: Department of Clinical Neurosciences, Geneva University Hospitals, Geneva, Switzerland. jkamtchum@yahoo.fr
authors.forename: Joseph
authors.initials: J
authors.lastname: Kamtchum Tatuene
authors.forename: Maria Isabel
authors.initials: MI
authors.lastname: Vargas
authors.forename: Karim
authors.initials: K
authors.lastname: Burkhardt
authors.forename: Michel
authors.initials: M
authors.lastname: Chofflon
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D000368:Aged; D001706:Biopsy; D002540:Cerebral Cortex / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000473:pathology; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D012680:Sensitivity and Specificity; D014944:World Health Organization
chemical_list: D048948:14-3-3 Proteins