HOMEPRODUCTSSERVICESCOMPANYCONTACTFAQResearchDictionaryPharmaMobileSign Up FREE or Login

Dystonic storm due to Batten's disease treated with pallidotomy and deep brain stimulation.

Abstract
To report a novel treatment approach, pallidotomy and deep brain stimulation (DBS), in two sisters with dystonic storm due to Batten's disease. This study is based on long-term follow-up of two sisters, presenting with dystonic storm and their response to pallidotomy and DBS. These sisters, who had visual loss, seizures, and progressive psychomotor decline, experienced progressive disabling abnormal movements culminating in dystonic storm at the age of 15 and 17 years, respectively. In addition to intubation and sedation, multiple medications, including botulinum toxin injections and intrathecal baclofen infusion were tried in both patients without any benefit. The old sister underwent bilateral pallidotomy. Within 10 days postoperatively, there was marked improvement in dystonic storm. She was free of abnormal movements for 9 months. Then she started having opisthotonus lasting 20 seconds to an hour several times/day, but over 6 years abnormal movements are markedly improved, and not returned to pre-pallidotomy level. The young sister underwent both bilateral pallidotomy and DBS, 3 weeks apart. She was free of abnormal movements for 7 months and able to maintain reduction in the abnormal movements by adjusting DBS settings. Pallidotomy and DBS should be considered in dystonic storm due to Batten's disease.
AuthorsMuruvet Elkay, Kenneth Silver, Richard D Penn, Arif Dalvi
JournalMovement disorders : official journal of the Movement Disorder Society (Mov Disord) Vol. 24 Issue 7 Pg. 1048-53 (May 15 2009) ISSN: 1531-8257 [Electronic] United States
PMID19353721 (Publication Type: Case Reports, Journal Article)
Copyright(c) 2009 Movement Disorder Society.
Topics
  • Adult
  • Deep Brain Stimulation (methods)
  • Dystonia (etiology, therapy)
  • Female
  • Humans
  • Longitudinal Studies
  • Neuronal Ceroid-Lipofuscinoses (complications)
  • Pallidotomy (methods)
  • Young Adult

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research network!


Choose Username:
Email:
Password:
Verify Password: