Cargando…
Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement
Pallidal deep brain stimulation is an established treatment in dystonia. Available data on the effect in DYT-THAP1 dystonia (also known as DYT6 dystonia) are scarce and long-term follow-up studies are lacking. In this retrospective, multicenter follow-up case series of medical records of such patien...
Autores principales: | , , , , , , , , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2019
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6947218/ https://www.ncbi.nlm.nih.gov/pubmed/31817799 http://dx.doi.org/10.3390/jcm8122163 |
_version_ | 1783485499085684736 |
---|---|
author | Danielsson, Annika Carecchio, Miryam Cif, Laura Koy, Anne Lin, Jean-Pierre Solders, Göran Romito, Luigi Lohmann, Katja Garavaglia, Barbara Reale, Chiara Zorzi, Giovanna Nardocci, Nardo Coubes, Philippe Gonzalez, Victoria Roubertie, Agathe Collod-Beroud, Gwenaelle Lind, Göran Tedroff, Kristina |
author_facet | Danielsson, Annika Carecchio, Miryam Cif, Laura Koy, Anne Lin, Jean-Pierre Solders, Göran Romito, Luigi Lohmann, Katja Garavaglia, Barbara Reale, Chiara Zorzi, Giovanna Nardocci, Nardo Coubes, Philippe Gonzalez, Victoria Roubertie, Agathe Collod-Beroud, Gwenaelle Lind, Göran Tedroff, Kristina |
author_sort | Danielsson, Annika |
collection | PubMed |
description | Pallidal deep brain stimulation is an established treatment in dystonia. Available data on the effect in DYT-THAP1 dystonia (also known as DYT6 dystonia) are scarce and long-term follow-up studies are lacking. In this retrospective, multicenter follow-up case series of medical records of such patients, the clinical outcome of pallidal deep brain stimulation in DYT-THAP1 dystonia, was evaluated. The Burke Fahn Marsden Dystonia Rating Scale served as an outcome measure. Nine females and 5 males were enrolled, with a median follow-up of 4 years and 10 months after implant. All benefited from surgery: dystonia severity was reduced by a median of 58% (IQR 31-62, p = 0.001) at last follow-up, as assessed by the Burke Fahn Marsden movement subscale. In the majority of individuals, there was no improvement of speech or swallowing, and overall, the effect was greater in the trunk and limbs as compared to the cranio-cervical and orolaryngeal regions. No correlation was found between disease duration before surgery, age at surgery, or preoperative disease burden and the outcome of deep brain stimulation. Device- and therapy-related side-effects were few. Accordingly, pallidal deep brain stimulation should be considered in clinically impairing and pharmaco-resistant DYT-THAP1 dystonia. The method is safe and effective, both short- and long-term. |
format | Online Article Text |
id | pubmed-6947218 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-69472182020-01-13 Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement Danielsson, Annika Carecchio, Miryam Cif, Laura Koy, Anne Lin, Jean-Pierre Solders, Göran Romito, Luigi Lohmann, Katja Garavaglia, Barbara Reale, Chiara Zorzi, Giovanna Nardocci, Nardo Coubes, Philippe Gonzalez, Victoria Roubertie, Agathe Collod-Beroud, Gwenaelle Lind, Göran Tedroff, Kristina J Clin Med Article Pallidal deep brain stimulation is an established treatment in dystonia. Available data on the effect in DYT-THAP1 dystonia (also known as DYT6 dystonia) are scarce and long-term follow-up studies are lacking. In this retrospective, multicenter follow-up case series of medical records of such patients, the clinical outcome of pallidal deep brain stimulation in DYT-THAP1 dystonia, was evaluated. The Burke Fahn Marsden Dystonia Rating Scale served as an outcome measure. Nine females and 5 males were enrolled, with a median follow-up of 4 years and 10 months after implant. All benefited from surgery: dystonia severity was reduced by a median of 58% (IQR 31-62, p = 0.001) at last follow-up, as assessed by the Burke Fahn Marsden movement subscale. In the majority of individuals, there was no improvement of speech or swallowing, and overall, the effect was greater in the trunk and limbs as compared to the cranio-cervical and orolaryngeal regions. No correlation was found between disease duration before surgery, age at surgery, or preoperative disease burden and the outcome of deep brain stimulation. Device- and therapy-related side-effects were few. Accordingly, pallidal deep brain stimulation should be considered in clinically impairing and pharmaco-resistant DYT-THAP1 dystonia. The method is safe and effective, both short- and long-term. MDPI 2019-12-06 /pmc/articles/PMC6947218/ /pubmed/31817799 http://dx.doi.org/10.3390/jcm8122163 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Danielsson, Annika Carecchio, Miryam Cif, Laura Koy, Anne Lin, Jean-Pierre Solders, Göran Romito, Luigi Lohmann, Katja Garavaglia, Barbara Reale, Chiara Zorzi, Giovanna Nardocci, Nardo Coubes, Philippe Gonzalez, Victoria Roubertie, Agathe Collod-Beroud, Gwenaelle Lind, Göran Tedroff, Kristina Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement |
title | Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement |
title_full | Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement |
title_fullStr | Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement |
title_full_unstemmed | Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement |
title_short | Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement |
title_sort | pallidal deep brain stimulation in dyt6 dystonia: clinical outcome and predictive factors for motor improvement |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6947218/ https://www.ncbi.nlm.nih.gov/pubmed/31817799 http://dx.doi.org/10.3390/jcm8122163 |
work_keys_str_mv | AT danielssonannika pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT carecchiomiryam pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT ciflaura pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT koyanne pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT linjeanpierre pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT soldersgoran pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT romitoluigi pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT lohmannkatja pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT garavagliabarbara pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT realechiara pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT zorzigiovanna pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT nardoccinardo pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT coubesphilippe pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT gonzalezvictoria pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT roubertieagathe pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT collodberoudgwenaelle pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT lindgoran pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement AT tedroffkristina pallidaldeepbrainstimulationindyt6dystoniaclinicaloutcomeandpredictivefactorsformotorimprovement |