title: Cognitive and motor function in long-duration PARKIN-associated Parkinson disease.
abstract: OBJECTIVE
  Data on the long-term cognitive outcomes of patients with PARKIN-associated Parkinson disease (PD) are unknown but may be useful when counseling these patients.
  
  
  OBJECTIVE
  Among patients with early-onset PD of long duration, we assessed cognitive and motor performances, comparing homozygotes and compound heterozygotes who carry 2 PARKIN mutations with noncarriers.
  
  
  METHODS
  Cross-sectional study of 44 participants at 17 different movement disorder centers who were in the Consortium on Risk for Early-Onset PD study with a duration of PD greater than the median duration (>14 years): 4 homozygotes and 17 compound heterozygotes (hereafter referred to as carriers) and 23 noncarriers.
  
  
  METHODS
  Unified Parkinson Disease Rating Scale Part III (UPDRS-III) and Clinical Dementia Rating scores and neuropsychological performance. Linear regression models were applied to assess the association between PARKIN mutation status and cognitive domain scores and UPDRS-III scores. Models were adjusted for age, education, disease duration, language, and levodopa equivalent daily dose.
  
  
  RESULTS
  Carriers had an earlier age at onset of PD (P < .001) and were younger (P = .004) at time of examination than noncarriers. They performed better than noncarriers on the Mini-Mental State Examination (P = .010) and were more likely to receive lower scores on the Clinical Dementia Rating (P = .003). In multivariate analyses, carriers performed better than noncarriers on the UPDRS-III (P = .02) and on tests of attention (P = .03), memory (P = .03), and visuospatial (P = .02) cognitive domains.
  
  
  CONCLUSIONS
  In cross-sectional analyses, carriers demonstrated better cognitive and motor performance than did noncarriers with long disease duration, suggesting slower disease progression. A longitudinal follow-up study is required to confirm these findings.
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York2Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Roy N
authors.initials: RN
authors.lastname: Alcalay
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Elise
authors.initials: E
authors.lastname: Caccappolo
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Helen
authors.initials: H
authors.lastname: Mejia-Santana
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York2Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Ming Xin
authors.initials: MX
authors.lastname: Tang
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Llency
authors.initials: L
authors.lastname: Rosado
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Martha
authors.initials: M
authors.lastname: Orbe Reilly
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Diana
authors.initials: D
authors.lastname: Ruiz
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York2Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York3Gertru.
authors.forename: Elan D
authors.initials: ED
authors.lastname: Louis
authors.affiliation: Department of Neurological Sciences, Rush University Medical Center, Chicago, Illinois.
authors.forename: Cynthia L
authors.initials: CL
authors.lastname: Comella
authors.affiliation: Struthers Parkinson's Center, Park Nicollet Clinic, Golden Valley, Minnesota.
authors.forename: Martha A
authors.initials: MA
authors.lastname: Nance
authors.affiliation: The Alan and Barbara Mirken Department of Neurology, Beth Israel Medical Center, New York, New York8Department of Neurology, Albert Einstein College of Medicine, Bronx, New York.
authors.forename: Susan B
authors.initials: SB
authors.lastname: Bressman
authors.affiliation: Dr John T. Macdonald Foundation, Department of Human Genetics, Miami Institute for Human Genomics, Miller School of Medicine, University of Miami, Miami, Florida.
authors.forename: William K
authors.initials: WK
authors.lastname: Scott
authors.affiliation: Parkinson's Institute, Sunnyvale, and Department of Health Research and Policy, Stanford University, Palo Alto, California.
authors.forename: Caroline M
authors.initials: CM
authors.lastname: Tanner
authors.affiliation: Marshfield Clinic, Department of Neurology, Marshfield, Wisconsin.
authors.forename: Susan F
authors.initials: SF
authors.lastname: Mickel
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Cheryl H
authors.initials: CH
authors.lastname: Waters
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Stanley
authors.initials: S
authors.lastname: Fahn
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York3Gertrude H. Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Lucien J
authors.initials: LJ
authors.lastname: Cote
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Frucht
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York.
authors.forename: Blair
authors.initials: B
authors.lastname: Ford
authors.affiliation: Central DuPage Hospital, Neurosciences Institute, Movement Disorders Center, Winfield, Illinois.
authors.forename: Michael
authors.initials: M
authors.lastname: Rezak
authors.affiliation: Department of Neurology, NorthShore University Health System, Evanston, Illinois14Department of Neurology, University of Chicago, Pritzker School of Medicine, Chicago, Illinois.
authors.forename: Kevin E
authors.initials: KE
authors.lastname: Novak
authors.affiliation: Department of Neurology, Butler Hospital, Providence, Rhode Island16Department of Neurology, Alpert Medical School, Brown University, Providence, Rhode Island.
authors.forename: Joseph H
authors.initials: JH
authors.lastname: Friedman
authors.affiliation: Department of Neurology, College of Medicine, University of Tennessee Health Science Center, Memphis.
authors.forename: Ronald F
authors.initials: RF
authors.lastname: Pfeiffer
authors.affiliation: Morris K. Udall Parkinson's Disease Research Center of Excellence and Departments of Psychiatry and Behavioral Sciences and Neurology and Neurological Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Laura
authors.initials: L
authors.lastname: Marsh
authors.affiliation: Department of Neurology, Medical College of Wisconsin, Milwaukee.
authors.forename: Bradley
authors.initials: B
authors.lastname: Hiner
authors.affiliation: New York State Department of Health Wadsworth Center, Albany, New York.
authors.forename: Haydeh
authors.initials: H
authors.lastname: Payami
authors.affiliation: Parkinson's Disease and Movement Disorders Center of Albany Medical Center, Albany, New York.
authors.forename: Eric
authors.initials: E
authors.lastname: Molho
authors.affiliation: Department of Neurology, Emory University, Atlanta, Georgia.
authors.forename: Stewart A
authors.initials: SA
authors.lastname: Factor
authors.affiliation: Portland VA Medical Center, Parkinson Disease Research, Education and Clinical Center, and Oregon Health and Science University, Portland.
authors.forename: John G
authors.initials: JG
authors.lastname: Nutt
authors.affiliation: Department of Neurology, University of Puerto Rico, San Juan.
authors.forename: Carmen
authors.initials: C
authors.lastname: Serrano
authors.affiliation: Department of Neurology, University of Puerto Rico, San Juan.
authors.forename: Maritza
authors.initials: M
authors.lastname: Arroyo
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York3Gertrude H. Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, New York4Department of Epidemiology, Mailman School of P.
authors.forename: Ruth
authors.initials: R
authors.lastname: Ottman
authors.affiliation: Division of Human Genetics, Cincinnati Children's Hospital Medical Center, and Department of Pediatrics; University of Cincinnati College of Medicine, Ohio.
authors.forename: Michael W
authors.initials: MW
authors.lastname: Pauciulo
authors.affiliation: Division of Human Genetics, Cincinnati Children's Hospital Medical Center, and Department of Pediatrics; University of Cincinnati College of Medicine, Ohio.
authors.forename: William C
authors.initials: WC
authors.lastname: Nichols
authors.affiliation: Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York27Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New Yor.
authors.forename: Lorraine N
authors.initials: LN
authors.lastname: Clark
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York2Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York3Gertru.
authors.forename: Karen S
authors.initials: KS
authors.lastname: Marder
mesh_terms: D017668:Age of Onset; D000368:Aged; D003072:Cognition Disorders / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology*; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005260:Female; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D019957:Motor Skills Disorders / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D010300:Parkinson Disease / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology*; D044767:Ubiquitin-Protein Ligases / Q000235:genetics*
chemical_list: D044767:Ubiquitin-Protein Ligases; C111567:parkin protein