title: Sporadic MM2-thalamic + cortical Creutzfeldt-Jakob disease: Utility of diffusion tensor imaging in the detection of cortical involvement in vivo.
abstract: In sporadic Creutzfeldt-Jakob disease (sCJD), high signal intensity in fluid attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI) sequences in striatum and/or cortical regions of the brain are present in about 83% of cases, reflecting tissue damage, such as spongiform change and abnormal prion protein deposits. Novel diffusion sequences of MRI might improve the detection of CJD characteristic changes in the subset of patients in whom these alterations are absent or less evident. We report a neuropathologically confirmed case of the rare MM2 T + C subtype of sCJD, with mixed clinical and neuropathological features of MM2 thalamic and MM2 cortical subtypes, in whom the use of diffusion tensor imaging helped to identify cortical hyperintensities that could be easily overlooked with conventional DWI.
authors.affiliation: Neurological Tissue Bank of the Biobanc-Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain.
authors.forename: Oriol
authors.initials: O
authors.lastname: Grau-Rivera
authors.affiliation: Alzheimer disease and other cognitive disorders Unit, Department of Neurology, Hospital Clínic, IDIBAPS, Barcelona, Spain.|Department of Neurology, Hospital Clínic. IDIBAPS, Barcelona, Spain.
authors.forename: Raquel
authors.initials: R
authors.lastname: Sánchez-Valle
authors.affiliation: Radiology Department, Image Diagnosis Center, Hospital Clínic, Barcelona, Spain.|Magnetic Resonance Image core facility of IDIBAPS, Barcelona, Spain.
authors.forename: Nuria
authors.initials: N
authors.lastname: Bargalló
authors.affiliation: Alzheimer disease and other cognitive disorders Unit, Department of Neurology, Hospital Clínic, IDIBAPS, Barcelona, Spain.|Department of Neurology, Hospital Clínic. IDIBAPS, Barcelona, Spain.
authors.forename: Albert
authors.initials: A
authors.lastname: Lladó
authors.affiliation: Department of Neurology, Hospital Clínic. IDIBAPS, Barcelona, Spain.
authors.forename: Carles
authors.initials: C
authors.lastname: Gaig
authors.affiliation: General Subdirectorate of Surveillance and Response to Emergencies in Public Health, Department of Public Health in Catalonia, Barcelona, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Nos
authors.affiliation: Institut de Neuropatologia, Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain.
authors.forename: Isidre
authors.initials: I
authors.lastname: Ferrer
authors.affiliation: Department of Neurology, Hospital Clínic. IDIBAPS, Barcelona, Spain.
authors.forename: Francesc
authors.initials: F
authors.lastname: Graus
authors.affiliation: Neurological Tissue Bank of the Biobanc-Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain.
authors.forename: Ellen
authors.initials: E
authors.lastname: Gelpi
mesh_terms: D002540:Cerebral Cortex / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000000981:diagnostic imaging* / Q000473:pathology*; D056324:Diffusion Tensor Imaging / Q000379:methods*; D005260:Female; D006801:Humans; D008875:Middle Aged
keywords: Creutzfeldt-Jakob disease; MM2; diffusion tensor imaging; sporadic familial insomnia; thalamic