Skip Navigation

Publication Detail

Title: Cognitive and motor function in long-duration PARKIN-associated Parkinson disease.

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

Published In JAMA Neurol, (2014 Jan)

Abstract: 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.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.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.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.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.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.

PubMed ID: 24190026 Exiting the NIEHS site

MeSH Terms: Age of Onset; Aged; Cognition Disorders/genetics*; Cognition Disorders/metabolism; Cognition Disorders/physiopathology*; Cross-Sectional Studies; Disease Progression; Female; Heterozygote; Humans; Male; Middle Aged; Motor Skills Disorders/genetics; Motor Skills Disorders/metabolism; Motor Skills Disorders/physiopathology; Parkinson Disease/genetics*; Parkinson Disease/metabolism; Parkinson Disease/physiopathology*; Ubiquitin-Protein Ligases/genetics*

Back
to Top