Cognitive and motor function in long-duration PARKIN-associated parkinson disease

Roy N. Alcalay, Elise Caccappolo, Helen Mejia-Santana, Ming Xin Tang, Llency Rosado, Martha Orbe Reilly, Diana Ruiz, Elan D. Louis, Cynthia L. Comella, Martha A. Nance, Susan B. Bressman, William K. Scott, Caroline M. Tanner, Susan F. Mickel, Cheryl H. Waters, Stanley Fahn, Lucien J. Cote, Steven J. Frucht, Blair Ford, Michael RezakKevin E. Novak, Joseph H. Friedman, Ronald F. Pfeiffer, Laura Marsh, Bradley Hiner, Haydeh Payami, Eric Molho, Stewart A. Factor, John G. Nutt, Carmen Serrano, Maritza Arroyo, Ruth Ottman, Michael W. Pauciulo, William C. Nichols, Lorraine N. Clark, Karen S. Marder

Research output: Contribution to journalArticlepeer-review

44 Scopus citations


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. MAINOUTCOMESAND 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.

Original languageEnglish (US)
Pages (from-to)62-67
Number of pages6
JournalJAMA neurology
Issue number1
StatePublished - Jan 2014
Externally publishedYes

ASJC Scopus subject areas

  • Clinical Neurology


Dive into the research topics of 'Cognitive and motor function in long-duration PARKIN-associated parkinson disease'. Together they form a unique fingerprint.

Cite this