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Chronic bilateral subthalamic deep brain stimulation in a patient with homozygous deletion in the Parkin gene

✍ Scribed by Marianna Capecci; Luca Passamonti; Ferdinanda Annesi; Grazia Annesi; Michele Bellesi; Innocenza Claudia Cirò Candiano; Riccardo Ricciuti; Maurizio Iacoangeli; Massimo Scerrati; Mario Zappia; Patrizia Tarantino; Elvira Valeria De Marco; Donatella Civitelli; Sara Carrideo; Leandro Provinciali; Maria Gabriella Ceravolo; Aldo Quattrone

Publisher
John Wiley and Sons
Year
2004
Tongue
English
Weight
53 KB
Volume
19
Category
Article
ISSN
0885-3185

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✦ Synopsis

Abstract

Chronic subthalamic nucleus deep brain stimulation (STN‐DBS) is an efficacious treatment for idiopathic Parkinson's disease (PD) that cannot be further improved by medical therapy. We present a case of an individual with juvenile parkinsonism caused by homozygous deletion of exon 3 in the parkin gene with disabling long‐term side‐effects from levodopa who underwent bilateral STN neuromodulation. Parkin‐linked parkinsonism may show clinical features different from sporadic PD, yet it shares levodopa responsiveness. Because levodopa responsiveness is a predictor of STN‐DBS efficacy, we argued that this kind of surgical approach might be efficacious in hereditary parkin‐linked juvenile parkinsonism. We evaluated clinical and functional assessment before and 12 months after surgery. The results showed that the Unified Parkinson Disease Rating Scales Motor score improved by 84% in our patient, the levodopa equivalent daily dose medication (LEDD) was reduced by 66%, and, finally, disabling and severe dyskinesias disappeared. © 2004 Movement Disorder Society

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