title: Abnormal distribution of GABAAreceptors in brain of duchenne muscular dystrophy patients.
abstract: In this study we sought to: (1) determine the distribution of GABAAreceptors (GABAA-Rs) in the brain of Duchenne muscular dystrophy (DMD) patients; and (2) ascertain if the distribution pattern correlates with cognitive dysfunction.
  
  
  
  Fourteen DMD patients [young adult (n = 7, 18-25 years old) and older adult (n = 7, 30-37 years old) groups] and 16 age-matched normal volunteers participated. GABAA-R distribution was assessed using123I-IMZ-SPECT. Neuropsychological assessments were performed using 3 different test batteries, the WAIS-III, WMS-R, and Wisconsin Card Sorting Test (WCST).
  
  
  
  All DMD patients showed significant decline in123I-IMZ uptake in the prefrontal cortex (P < 0.05). Although no differences were detected in the WAIS-III and WMS-R, the WCST scores of DMD patients (2.8 ± 1.9) were significantly lower (P < 0.01) than those of normal volunteers (5.4 ± 0.7). Both abnormalities were more pronounced in older adult patients.
  
  
  
  The findings demonstrate that DMD is accompanied by a reduction in the prefrontal cortex distribution of GABAA-Rs. Muscle Nerve 55: 591-595, 2017.
authors.affiliation: Center for Integrated Human Brain Science, Brain Research Institute, University of Niigata, 1-757 Asahimachi, Niigata, 951-8585, Japan.|National Hospital Organization, Niigata National Hospital, Niigata, Japan.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Suzuki
authors.affiliation: National Hospital Organization, Niigata National Hospital, Niigata, Japan.
authors.forename: Shinya
authors.initials: S
authors.lastname: Higuchi
authors.affiliation: National Hospital Organization, Niigata National Hospital, Niigata, Japan.
authors.forename: Izumi
authors.initials: I
authors.lastname: Aida
authors.affiliation: National Hospital Organization, Niigata National Hospital, Niigata, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Nakajima
authors.affiliation: Center for Integrated Human Brain Science, Brain Research Institute, University of Niigata, 1-757 Asahimachi, Niigata, 951-8585, Japan.
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Nakada
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism*; D016022:Case-Control Studies; D005260:Female; D005442:Flumazenil / Q000031:analogs & derivatives / Q000378:metabolism; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000000981:diagnostic imaging / Q000473:pathology*; D009483:Neuropsychological Tests; D011963:Receptors, GABA-A / Q000378:metabolism*; D012189:Retrospective Studies; D015899:Tomography, Emission-Computed, Single-Photon; D055815:Young Adult
chemical_list: D011963:Receptors, GABA-A; D005442:Flumazenil; C063038:iomazenil
keywords: 123I-iomazenil-SPECT; Duchenne muscular dystrophy; GABAA receptor; brain; cognitive dysfunction