Characterization of Recessive Parkinson Disease in a Large Multicenter Study.

Lesage, Suzanne; Lunati, Ariane; Houot, Marion; Romdhan, Sawssan Ben; Clot, Fabienne; Tesson, Christelle; Mangone, Graziella; Toullec, Benjamin Le et al. · Ann Neurol · 2020

retrospective_cohort · Level III

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Abstract

Studies of the phenotype and population distribution of rare genetic forms of parkinsonism are required, now that gene-targeting approaches for Parkinson disease have reached the clinical trial stage. We evaluated the frequencies of PRKN, PINK1, and DJ-1 mutations in a cohort of 1,587 cases. Mutations were found in 14.1% of patients; 27.6% were familial and 8% were isolated. PRKN was the gene most frequently mutated in Caucasians, whereas PINK1 mutations predominated in Arab-Berber individuals. Patients with PRKN mutations had an earlier age at onset, and less asymmetry, levodopa-induced motor complications, dysautonomia, and dementia than those without mutations. ANN NEUROL 2020;88:843-850.

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