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From Biostructure and Bioimaging Institute (Drs. Varrone, Brunetti, Salvatore, and Pappatà), National Research Council, Napoli; Department of Neurological Sciences (Drs. Pellecchia, Amboni, Salvatore, Bonavita, De Michele, and Barone) and Biomorphological and Functional Sciences (Drs. Sansone and Salvatore), University "Federico II," Napoli; Department of Molecular Neurogenetics (D. Ghezzi and Dr. Garavaglia), "C. Besta" Neurological Institute, Milan, Italy; and INSERM U289 (Dr. Brice), Department of Genetics, Cytogenetics and Embriology, Pitié-Salpêtrière Hospital, Paris, France.
Address correspondence and reprint requests to Dr. Andrea Varrone, Biostructure and Bioimaging Institute, National Research Council, Via S. Pansini, 5, 80131, Napoli, Italy; e-mail: andrea.varrone{at}ibb.cnr.it
Objective: To investigate whether the presence of parkin gene mutations is associated with different nigrostriatal impairment than other early-onset parkinsonism.
Methods: Eighteen consecutive early-onset Parkinson disease (PD) patients (nine parkin and nine nonparkin patients) and six controls were studied with [123I]FP-CIT SPECT.
Results: Parkin patients had longer disease duration (15 ± 9 vs 6 ± 2 years, p = 0.008) and higher Unified Parkinsons Disease Rating Scale (UPDRS) motor score (35.8 ± 13.7 vs 22.8 ± 7.9, p = 0.025) than nonparkin patients. Caudate and putamen DAT density were reduced by 60% and 79% in parkin and by 43% and 70% in nonparkin patients. Multiple regression analysis showed that the UPDRS and the presence of parkin gene mutations, but not the disease duration, were significantly correlated with the striatal DAT density. Parkin patients showed a more symmetric DAT loss in both caudate and putamen as compared with nonparkin patients. Conclusions: Parkin-related disease may be associated with a higher degree of nigrostriatal impairment, independently of the clinical severity of the disease, and a more symmetric involvement as compared with non-parkin early-onset disease.
Received February 13, 2004. Accepted in final form July 30, 2004.
Presented in part in abstract form at the 2003 Congress of the European Nuclear Medicine Association, at the 2003 Congress of the Lega Italiana per la lotta contro la Malattia di Parkinson le Sindromi Extrapiramidali e le Demenze, and at the 2004 Congress of the Society of Nuclear Medicine.
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