title: [Serial magnetic resonance images of a Creutzfeldt-Jakob disease patient with V180I mutation obtained over 10 years].
abstract: We acquired serial magnetic resonance images (MRIs) of a Creutzfeldt-Jakob disease (CJD) patient carrying the V180I mutation; his symptoms slowly progressed over a period of 10 years. A 57-year-old man presented with cognitive impairment and was admitted to our hospital. Diffusion-weighted images (DWIs) and fluid-attenuated inversion recovery (FLAIR) images showed high-intensity areas (HIAs) in the cerebral cortex and basal ganglia, but not in the thalamus, brainstem, and cerebellum, until 1.5 years after symptom onset. The HIAs in the cerebral cortex and basal ganglia disappeared 4 years after symptom onset, while the atrophy in these regions progressed rapidly during this period. However, the thalamus, brainstem, and cerebellum appeared to be preserved over 10 years after symptom onset. The mechanism for the regional vulnerability in brains of CJD patients remains unclear. Further studies in additional cases are required to clarify whether differences in the mutation of the prion protein gene might be associated with the vulnerability.
authors.affiliation: Department of Neurology, Miyagi National Hospital.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shimizu
authors.forename: Yusei
authors.initials: Y
authors.lastname: Shiga
authors.forename: Arifumi
authors.initials: A
authors.lastname: Matsumoto
authors.forename: Kinya
authors.initials: K
authors.lastname: Hisanaga
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009154:Mutation; D010446:Peptide Fragments / Q000235:genetics*; D018621:PrPC Proteins / Q000235:genetics*
chemical_list: D010446:Peptide Fragments; D018621:PrPC Proteins; C521306:prion protein (180-193)