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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.

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.

Author Affiliations
From the Departments of Neurology (Drs Rifai, Tawil, and Griggs), Psychiatry (Dr Klitzke), and Pathology (Drs Kazee and Griggs), University of Rochester (NY), and the MDA H. Houston Merritt Clinical Research Center for Muscular Dystrophy, Columbia-Presbyterian Medical Center, New York, NY (Drs Shanske and DiMauro).

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