Differential Response of Dystonia and Parkinsonism Following Globus Pallidus Internus Deep Brain Stimulation in X-linked Dystonia-parkinsonism (Lubag)

Overview

Journal Stereotact Funct Neurosurg

Publisher Karger

Specialty Neurosurgery

Date 2010 Aug 18

PMID 20714213

Citations 14

Authors

Genko Oyama

Hubert H Fernandez

Kelly D Foote

Pam Zeilman

Nelson Hwynn

Charles E Jacobson 4th

Irene A Malaty

Ramon L Rodriguez

Michael S Okun

Affiliations

Soon will be listed here.

Abstract

Background: X-linked dystonia-parkinsonism (XDP; DYT3; Lubag) is an adult-onset hereditary progressive dystonia/parkinsonism which is typically minimally responsive to pharmacological treatment.

Case Report: We report a 63- year-old man with a diagnosis of XDP who underwent bilateral globus pallidus internus deep brain stimulator (GPi-DBS) placement. His course initially began with right hand tremor and dystonia at age 57 and progressed to also include bradykinesia and rigidity. The patient tolerated the procedure without significant complications. GPi-DBS improved his right hand dystonia, but did not significantly improve his parkinsonism.

Conclusion: DBS may be a therapeutic option for select cases of XDP, but its specific indications must be carefully discussed, as the available cases have had mixed responses. Whether other targets may be more effective is not known.

Citing Articles

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Establishing a natural history of X-linked dystonia parkinsonism.

Acuna P, Supnet-Wells M, Spencer N, de Guzman J, Russo M, Hunt A Brain Commun. 2023; 5(3):fcad106.

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X-linked dystonia parkinsonism: epidemiology, genetics, clinical features, diagnosis, and treatment.

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Pallidal Deep Brain Stimulation for Monogenic Dystonia: The Effect of Gene on Outcome.

Tisch S, Kumar K Front Neurol. 2021; 11:630391.

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