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    Arch Neurol. 1994 Jan;51(1):90-4.

    Dementia of adult polyglucosan body disease. Evidence of cortical and subcortical dysfunction.

    Rifai Z, Klitzke M, Tawil R, Kazee AM, Shanske S, DiMauro S, Griggs RC.

    Source

    Department of Neurology, University of Rochester, NY.

    Abstract

    OBJECTIVES:

    To characterize the dementia associated with adult polyglucosan body disease (APBD) and to correlate the cognitive deficits with abnormalities found on magnetic resonance imaging (MRI).

    METHODS:

    Quantitative neuropsychological testing and MRI in one man with APBD and a review of the literature.

    RESULTS:

    The dementia of APBD affects cortical and subcortical functions. The cognitive deficits correlate with MRI findings of cortical atrophy and white-matter abnormalities.

    CONCLUSION:

    Neuropsychological testing and MRI are helpful in the evaluation of patients with APBD.

    PMID:
    8274116
    [PubMed - indexed for MEDLINE]

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