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. (C) 2004 Movement Disorder Society.
Chronic bilateral subthalamic deep brain stimulation in a patient with homozygous deletion in the parkin gene / Capecci, M; Passamonti, Luca; Annesi, F; Annesi, G; Bellesi, M; Candiano, Ic; Ricciuti, R; Iacoangeli, M; Scerrati, M; Zappia, Michele; Tarantino, P; De Marco, Ev; Civitelli, D; Carrideo, S; Provinciali, L; Ceravolo, Mg; Quattrone, A.. - In: MOVEMENT DISORDERS. - ISSN 0885-3185. - STAMPA. - 19:12(2004), pp. 1450-1452. [10.1002/mds.20250]