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


CONCLUSIONS
Neuropsychological testing and MRI are helpful in the evaluation of patients with APBD.
Rifai
Z
Z
Department of Neurology, University of Rochester, NY.
Klitzke
M
M
Tawil
R
R
Kazee
A M
AM
Shanske
S
S
DiMauro
S
S
Griggs
R C
RC
D001927:Brain Diseases / Q000150:complications / Q000473:pathology* / Q000523:psychology; D003704:Dementia / Q000150:complications / Q000473:pathology* / Q000523:psychology*; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000473:pathology* / Q000523:psychology