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Segmental Dystonia Treated with Deep Brain Stimulation

  • Roongroj Bhidayasiri
  • Daniel Tarsy
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Dystonia causes sustained muscle contractions, repetitive twisting movements, and abnormal postures of the trunk, neck, face, or extremities (see Chap. 36). Dystonic movements can be either slow or rapid, change during different activities or postures, and may become fixed in advanced cases. Except for occasional tremor or myoclonus, the rest of the neurological examination is normal. Childhood onset generalized dystonia typically begins as a focal action dystonia, usually of one foot, and progresses over several years to become generalized (see Chap. 37). Cases presenting in adolescence more commonly begin in one upper extremity or in the cervical-thoracic region and are more slowly progressive (see Chap. 37)

Keywords

Deep Brain Stimulation Botulinum Toxin Globus Pallidus Advanced Case Cervical Dystonia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Segmental dystonia.mp4 (MP4 25,315KB)

Clip 1: while seated, the patient exhibits recurrent spasmodic left rotational torticollis and retrocollis together with adduction and extension posturing of his upper extremities. While walking, he exhibits jerky right lateral deviation of his upper trunk, recurrent jerky flexion at his right elbow, hyperextension and internal rotation of his left arm, and abortive attempts to stabilize his head with his hand. While writing, he exhibits perioral dystonia, repeated elevation of his right arm, slow writing, and mild spasmodic rotational torticollis to the left side. Clip 2: following GPi-DBS, while seated he displays less severe rotational torticollis and retrocollis with absence of upper limb dystonia. While walking, he displays right rotational torticollis without trunk or limb dystonia. He now writes faster and more easily without elevation of his right arm.

References

  1. 1.
    Bressman SB, Sabatti C, Raymond D, et al. The DYT1 phenotype and guidelines for diagnostic testing. Neurology. 2000;54:1746–52.PubMedCrossRefGoogle Scholar
  2. 2.
    Fasano A, Nardocci N, Emanuele E, et al. Non-DYT1 early-onset primary torsion dystonia: comparison with DYT1 phenotype and review of the literature. Mov Disord. 2006;21:1411–8.PubMedCrossRefGoogle Scholar
  3. 3.
    Kupsch A, Benecke R, Muller J, et al. Pallidal deep brain stimulation in primary generalized or segmental dystonia. N Engl J Med. 2006;355:1978–90.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Roongroj Bhidayasiri
    • 1
    • 2
  • Daniel Tarsy
    • 3
  1. 1.Chulalongkorn Center of Excellence on Parkinson’s Disease and Related DisordersChulalongkorn University HospitalBangkokThailand
  2. 2.Department of NeurologyDavid Geffen School of Medicine at UCLALos AngelesUSA
  3. 3.Department of NeurologyHarvard Medical School Beth Israel Deaconess Medical CenterBostonUSA

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