0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Original Investigation |

Cognitive and Motor Function in Long-Duration PARKIN-Associated Parkinson Disease

Roy N. Alcalay, MD, MSc1,2; Elise Caccappolo, PhD1; Helen Mejia-Santana, MSc1; Ming Xin Tang, PhD1,2; Llency Rosado, MD1; Martha Orbe Reilly, MD1; Diana Ruiz, BSc1; Elan D. Louis, MD, MSc1,2,3,4; Cynthia L. Comella, MD5; Martha A. Nance, MD6; Susan B. Bressman, MD7,8; William K. Scott, PhD9; Caroline M. Tanner, MD, PhD10; Susan F. Mickel, MD11; Cheryl H. Waters, MD1; Stanley Fahn, MD1; Lucien J. Cote, MD1,3; Steven J. Frucht, MD1; Blair Ford, MD1; Michael Rezak, MD, PhD12; Kevin E. Novak, PhD13,14; Joseph H. Friedman, MD15,16; Ronald F. Pfeiffer, MD17; Laura Marsh, MD18; Bradley Hiner, MD19; Haydeh Payami, PhD20; Eric Molho, MD21; Stewart A. Factor, DO22; John G. Nutt, MD23; Carmen Serrano, MD24; Maritza Arroyo, MD24; Ruth Ottman, PhD1,3,4,25; Michael W. Pauciulo, MBA26; William C. Nichols, PhD26; Lorraine N. Clark, PhD2,27,28; Karen S. Marder, MD, MPH1,2,3,29
[+] Author Affiliations
1Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York
2Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York
3Gertrude H. Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, New York
4Department of Epidemiology, Mailman School of Public Health, Columbia University, New York, New York
5Department of Neurological Sciences, Rush University Medical Center, Chicago, Illinois
6Struthers Parkinson's Center, Park Nicollet Clinic, Golden Valley, Minnesota.
7The Alan and Barbara Mirken Department of Neurology, Beth Israel Medical Center, New York, New York
8Department of Neurology, Albert Einstein College of Medicine, Bronx, New York
9Dr John T. Macdonald Foundation, Department of Human Genetics, Miami Institute for Human Genomics, Miller School of Medicine, University of Miami, Miami, Florida
10Parkinson's Institute, Sunnyvale, and Department of Health Research and Policy, Stanford University, Palo Alto, California
11Marshfield Clinic, Department of Neurology, Marshfield, Wisconsin
12Central DuPage Hospital, Neurosciences Institute, Movement Disorders Center, Winfield, Illinois
13Department of Neurology, NorthShore University Health System, Evanston, Illinois
14Department of Neurology, University of Chicago, Pritzker School of Medicine, Chicago, Illinois
15Department of Neurology, Butler Hospital, Providence, Rhode Island
16Department of Neurology, Alpert Medical School, Brown University, Providence, Rhode Island
17Department of Neurology, College of Medicine, University of Tennessee Health Science Center, Memphis
18Morris 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
19Department of Neurology, Medical College of Wisconsin, Milwaukee
20New York State Department of Health Wadsworth Center, Albany, New York
21Parkinson's Disease and Movement Disorders Center of Albany Medical Center, Albany, New York
22Department of Neurology, Emory University, Atlanta, Georgia
23Portland VA Medical Center, Parkinson Disease Research, Education and Clinical Center, and Oregon Health and Science University, Portland
24Department of Neurology, University of Puerto Rico, San Juan
25Division of Epidemiology, New York State Psychiatric Institute, New York, New York
26Division of Human Genetics, Cincinnati Children’s Hospital Medical Center, and Department of Pediatrics; University of Cincinnati College of Medicine, Ohio
27Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York, New York
28Center for Human Genetics, College of Physicians and Surgeons, Columbia University, New York, New York
29Department of Psychiatry, Columbia University Medical Center, New York, New York
JAMA Neurol. 2014;71(1):62-67. doi:10.1001/jamaneurol.2013.4498.
Text Size: A A A
Published online

Importance  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.

Design, Setting, and Participants  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.

Main Outcomes and Measures  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 and Relevance  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.

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Figures

Tables

References

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Topics
PubMed Articles
Jobs
JAMAevidence.com

The Rational Clinical Examination
Make the Diagnosis: Parkinsonism

The Rational Clinical Examination
Original Article: Does This Patient Have Parkinson Disease?

brightcove.createExperiences();