Deep Brain Stimulation for Pantothenate Kinase-associated Neurodegeneration

Overview

Journal Case Rep Neurol Med

Publisher Wiley

Specialty Neurology

Date 2015 Mar 25

PMID 25802776

Citations 2

Authors

Pedro J Garcia-Ruiz

Joaquin Ayerbe

Lydia Vela Desojo

Cici E Feliz

Javier Del Val Fernandez

Affiliations

Soon will be listed here.

Abstract

Pantothenate kinase-associated neurodegeneration (PKAN) is usually associated with dystonia, which is typically severe and progressive over time. Pallidal stimulation (GPi DBS) has been carried out in selected cases of PKAN with drug-resistant dystonia with variable results. We report a 30-month follow-up study of a 30-year-old woman with PKAN-related dystonia treated with GPi DBS. Postoperatively, the benefit quickly became evident, as the patient exhibited a marked improvement in her dystonia, including her writing difficulty. This result has been maintained up to the present. GPi DBS should be considered in dystonic PKAN patients provided fixed contractures and/or pyramidal symptoms are not present.

Citing Articles

Clinical course of patients with pantothenate kinase-associated neurodegeneration (PKAN) before and after DBS surgery.

Svetel M, Tomic A, Dragasevic N, Petrovic I, Kresojevic N, Jech R J Neurol. 2019; 266(12):2962-2969.

PMID: 31463603 DOI: 10.1007/s00415-019-09499-3.

Medical treatment of dystonia.

Termsarasab P, Thammongkolchai T, Frucht S J Clin Mov Disord. 2016; 3:19.

PMID: 28031858 PMC: 5168853. DOI: 10.1186/s40734-016-0047-6.

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