Deep brain stimulation for monogenic dystonia

Bhooma R. Aravamuthan, Jeff L. Waugh, Scellig S. Stone

Research output: Contribution to journalReview article

9 Scopus citations

Abstract

Purpose of review Deep brain stimulation (DBS) has recently emerged as an important management option in children with medically refractory dystonia. DBS is most commonly used, best studied, and thought to be most efficacious for a select group of childhood or adolescent onset monogenic dystonias (designated with a standard 'DYT' prefix). We review how to clinically recognize these types of dystonia and the relative efficacy of DBS for key monogenic dystonias. Recent findings Though used for dystonia in adults for several years, DBS has only lately been used in children. Recent evidence shows that patients with shorter duration of dystonia often experience greater benefit following DBS. This suggests that early recognition of the appropriate dystonic phenotypes and consideration of DBS in these patients may improve the management of dystonia. Summary DBS should be considered early in patients who have medically refractory dystonia, especially for the monogenic dystonias that have a high response rate to DBS. It is important to differentiate between these monogenic dystonias and dystonias of other causes to properly prognosticate for these patients and to determine whether DBS is an appropriate management option.

Original languageEnglish (US)
Pages (from-to)691-696
Number of pages6
JournalCurrent Opinion in Pediatrics
Volume29
Issue number6
DOIs
StatePublished - Dec 1 2017

Keywords

  • deep brain stimulation
  • dystonia
  • DYT1
  • DYT11
  • DYT6

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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