Literature DB >> 8274116

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

Z Rifai1, M Klitzke, R Tawil, A M Kazee, S Shanske, S DiMauro, R C Griggs.   

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.

Entities:  

Mesh:

Year:  1994        PMID: 8274116     DOI: 10.1001/archneur.1994.00540130124021

Source DB:  PubMed          Journal:  Arch Neurol        ISSN: 0003-9942


  4 in total

1.  Adult polyglucosan body disease: Natural History and Key Magnetic Resonance Imaging Findings.

Authors:  Fanny Mochel; Raphael Schiffmann; Marjan E Steenweg; Hasan O Akman; Mary Wallace; Frédéric Sedel; Pascal Laforêt; Richard Levy; J Michael Powers; Sophie Demeret; Thierry Maisonobe; Roseline Froissart; Bruno Barcelos Da Nobrega; Brent L Fogel; Marvin R Natowicz; Catherine Lubetzki; Alexandra Durr; Alexis Brice; Hanna Rosenmann; Varda Barash; Or Kakhlon; J Moshe Gomori; Marjo S van der Knaap; Alexander Lossos
Journal:  Ann Neurol       Date:  2012-09       Impact factor: 10.422

2.  Dementia of frontal lobe type due to adult polyglucosan body disease.

Authors:  P Boulan-Predseil; A Vital; B Brochet; D Darriet; P Henry; C Vital
Journal:  J Neurol       Date:  1995-08       Impact factor: 4.849

3.  Neural correlates of adaptive working memory training in a glycogen storage disease type-IV patient.

Authors:  Kristin Lee; Thomas Ernst; Gro Løhaugen; Xin Zhang; Linda Chang
Journal:  Ann Clin Transl Neurol       Date:  2017-02-23       Impact factor: 4.511

Review 4.  Characterization of cognitive impairment in adult polyglucosan body disease.

Authors:  Paul Theo Zebhauser; Isabell Cordts; Holger Hengel; Bernhard Haslinger; Paul Lingor; Hasan Orhan Akman; Tobias B Haack; Marcus Deschauer
Journal:  J Neurol       Date:  2022-01-08       Impact factor: 6.682

  4 in total

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