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Directly acting muscle relaxants like dantrolene and botulinum toxin (BoNT) have distinct mechanisms and applications. Dantrolene, a hydantoin derivative, acts on the ryanodine receptor (RYR1) in skeletal muscle cells. RYR1 are calcium channels present at the sarcoplasmic reticulum membrane. In response to excitation, they release calcium ions from the sarcoplasmic reticulum to the cytosol. Calcium promotes actin-myosin-mediated contraction of muscles.
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Arching deep brain stimulation in dystonia types.

Han-Joon Kim1, Beomseok Jeon2

  • 1Department of Neurology and Movement Disorder Center, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.

Journal of Neural Transmission (Vienna, Austria : 1996)
|March 19, 2021
PubMed
Summary
This summary is machine-generated.

Deep brain stimulation (DBS) offers significant improvement for dystonia patients unresponsive to medical treatments. Early consideration of DBS, especially for specific dystonia types, is recommended for better outcomes.

Keywords:
Deep brain stimulation (DBS)DystoniaInheritedPallidalSubthalamic

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Area of Science:

  • Neurology
  • Neurosurgery
  • Movement Disorders

Background:

  • Medical treatments, including botulinum toxin injections, are first-line for dystonia but often yield insufficient patient response.
  • Deep brain stimulation (DBS) presents a viable alternative for managing dystonia in non-responsive cases.

Purpose of the Study:

  • To review the efficacy and safety of deep brain stimulation (DBS) for various types of dystonia.
  • To identify factors influencing DBS outcomes in dystonia patients.
  • To guide the early consideration of DBS in indicated dystonia cases.

Main Methods:

  • Review of existing evidence on deep brain stimulation (DBS) for dystonia.
  • Analysis of DBS outcomes across different dystonia classifications (idiopathic, inherited, acquired, focal).
  • Consideration of patient-specific factors, including disease duration and musculoskeletal deformity.

Main Results:

  • Deep brain stimulation (DBS) demonstrates effectiveness and safety, particularly for idiopathic and inherited isolated generalized/segmental dystonia (e.g., DYT-TOR1A).
  • Other inherited and acquired dystonia types show variable responses to DBS.
  • Further evidence is required to establish the role of DBS in Meige syndrome, focal dystonia, and rare inherited forms.

Conclusions:

  • Early intervention with deep brain stimulation (DBS) is crucial when indicated, especially when disease duration is short and fixed deformities are absent.
  • Careful patient selection, including genetic evaluation, is essential for optimizing DBS outcomes in dystonia.
  • Deep brain stimulation (DBS) is a valuable therapeutic option for select dystonia patients, offering significant clinical benefits.