January 1994

Dementia of Adult Polyglucosan Body DiseaseEvidence of Cortical and Subcortical Dysfunction

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).

Arch Neurol. 1994;51(1):90-94. doi:10.1001/archneur.1994.00540130124021

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.