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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.

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  • Publisher Full Text
  • Authors

    Kono S, Manabe Y, Fujii D, Sakai Y, Narai H, Omori N, Kitamoto T, Abe K

    Institution

    Department of Neurology, National Hospital Organization Okayama Medical Center, Okayama, Japan.

    Source

    Journal of the neurological sciences 301:1-2 2011 Feb 15 pg 100-3

    MeSH

    Age of Onset
    Aged
    Akinetic Mutism
    Brain
    Creutzfeldt-Jakob Syndrome
    Diffusion Magnetic Resonance Imaging
    Disease Progression
    Early Diagnosis
    Follow-Up Studies
    Humans
    Male
    Mutant Proteins
    Mutation, Missense
    Point Mutation
    PrPSc Proteins
    Tomography, Emission-Computed, Single-Photon

    Pub Type(s)

    Case Reports
    Journal Article

    Language

    eng

    PubMed ID

    21094959