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Dementia of Adult Polyglucosan Body Disease Evidence of Cortical and Subcortical Dysfunction

Ziad Rifai, MD; Michael Klitzke, PhD; Rabi Tawil, MD; Ann Marie Kazee, MD; Sara Shanske, PhD; Salvatore DiMauro, MD; Robert C. Griggs, MD
Arch Neurol. 1994;51(1):90-94. doi:10.1001/archneur.1994.00540130124021.
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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.


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