title: [Asymmetric cortical high signal on diffusion weighted-MRI in a case of Creutzfeldt-Jakob disease].
abstract: High signal in the cerebral cortex and/or basal ganglia on diffusion-weighted magnetic resonance imaging (DW-MRI) has been described as a good diagnostic marker for sporadic Creutzfeldt-Jakob disease (sCJD). We report a case of sCJD with atypical clinical evolution and unusual DW-MRI findings. A 53-year-old man was seen with a 2-year history of a rapidly progressive dementia and cerebellar ataxia. Cerebrospinal fluid analysis, including the test for 14-3-3 protein, was normal. EEG did not show periodic activity. However, DW-MRI showed gyriform hyperintensity involving practically the entire cortical ribbon of the left hemisphere, whilst being limited to the posterior cingulate gyrus in the right hemisphere. DNA analysis showed no mutations or insertions in the prion protein gene, and homozigozity for methionine in codon 129. A subsequent brain biopsy confirmed the diagnosis of CJD. Thus, high signal on DW-MRI may be limited to the cerebral cortex and may present a very asymmetric distribution in sCJD.
authors.affiliation: Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, SP, Brazil.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Nitrini
authors.forename: Renata
authors.initials: R
authors.lastname: Areza-Fegyveres
authors.forename: Vilma R
authors.initials: VR
authors.lastname: Martins
authors.forename: Rosa Maria R P S
authors.initials: RM
authors.lastname: Castro
authors.forename: Michele C
authors.initials: MC
authors.lastname: Landemberger
authors.forename: Nancy
authors.initials: N
authors.lastname: Huang
authors.forename: Luiz A
authors.initials: LA
authors.lastname: Bacheschi
authors.forename: Luiz E
authors.initials: LE
authors.lastname: Bacheschi
authors.forename: Cláudia C
authors.initials: CC
authors.lastname: Leite
authors.forename: Carlos A
authors.initials: CA
authors.lastname: Buchpiguel
authors.forename: Sérgio
authors.initials: S
authors.lastname: Rosemberg
mesh_terms: D015415:Biomarkers; D002540:Cerebral Cortex / Q000473:pathology / Q000503:physiopathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D038524:Diffusion Magnetic Resonance Imaging; D006801:Humans; D008297:Male; D008875:Middle Aged
chemical_list: D015415:Biomarkers