title: Serial diffusion-weighted MRI and SPECT findings in a Creutzfeldt-Jakob disease patient with V180I mutation.
abstract: We report serial changes of diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) in a patient with Creutzfeldt-Jakob disease with V180I mutation (CJD180). DWI abnormalities in our patient were more predominantly observed in the left cerebral cortex than left basal ganglia. Hemilateral abnormalities progressed over 5 months to involve the contralateral side with increasing DWI signals. At 6 months, SPECT showed hypoperfusion in the left parietal and frontal lobes and the hypoperfusion region spread to the bilateral basal ganglia, right parietal and frontal lobes. SPECT imaging revealed marked cerebral blood flow reduction, predominantly in the cerebral cortex corresponding to brain areas with high-intensity DWI signals. During the follow-up period of CJD180, DWI was more sensitive than conventional FLAIR and T2-weighted images (T2WI) to detect and monitor the progression of abnormal hyperintense lesions. We suggest that serial DWI and SPECT findings are useful for not only early diagnosis of CJD but also for monitoring disease progression.
authors.affiliation: Department of Neurology, National Hospital Organization Okayama Medical Center, Okayama, Japan.
authors.forename: Syoichiro
authors.initials: S
authors.lastname: Kono
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Manabe
authors.forename: Daiki
authors.initials: D
authors.lastname: Fujii
authors.forename: Yasuko
authors.initials: Y
authors.lastname: Sakai
authors.forename: Hisashi
authors.initials: H
authors.lastname: Narai
authors.forename: Nobuhiko
authors.initials: N
authors.lastname: Omori
authors.forename: Tetsuyuki
authors.initials: T
authors.lastname: Kitamoto
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
mesh_terms: D017668:Age of Onset; D000368:Aged; D000405:Akinetic Mutism / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000235:genetics / Q000473:pathology*; D038524:Diffusion Magnetic Resonance Imaging; D018450:Disease Progression; D042241:Early Diagnosis; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D050505:Mutant Proteins / Q000235:genetics; D020125:Mutation, Missense; D017354:Point Mutation; D018620:PrPSc Proteins / Q000235:genetics*; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D050505:Mutant Proteins; D018620:PrPSc Proteins