DBS for Dystonia

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  • Indicated for
    • Generalized and segmental primary (inherited and idiopathic) dystonia
    • Cervical dystonia
    • Some childhood generalized dystonias (first line)
    • Medical refractory
    • Symptoms disabling enough to justify surgical risk
    • Botulinum toxin refractory
  • CI
    • If pallidium is damage DBS unlikely to work
      • Anoxia damage
      • Post Traumatic dystonia
  • Target
    • Posteroventral lateral GPi
      • Side effects
        • Bradykinesia and gait problems (unsure mech)
      • There are encouraging results in (outcome of DBS is usually excellent, rapid and sustained-up to 10 yrs)
        • Cervical dystonia,
        • Myoclonus-dystonia
        • Tardive dystonia
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  • Researched
    • STN
    • Thalamus
      • Sensorimotor thalamus
        • VIM for more tremor
      • Not sure which part of the ventrolateral thalamus is the best to stimulate
        • The anterior (that is, the Voa according to Hassler),
        • Posterior (the Vim)
        • Intermediate regions (the region formerly termed Vop).
  • Outcome
    • Symptoms may take months to improve
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  • Best prognostic factor
    • DYT1 better outcome than DYT2 gene
    • Younger age
    • Shorter duration of symptoms
    • Lack of psychiatric / psychological co-morbidities
  • If stimulated for long it can cause chronic changes that when the DBS is off the therapeutic effects of DBS is still seen for weeks → possible that DBS modulate disease progression → need future study to see if DBS early can be more beneficial (another EARLYSTIM trial for dystonia)
  • Dystonia emergencies related to DBS
    • Sudden DBS failure
    • Risk of dystonic crisis “status dystonicus”/ rhabdomyolysis etc morbidity +++
    • Admit to HDU if has generalised dystonia, especially if genetic
    • Speak to neurology + ICU teams.
    • Supportive management. Drugs: baclofen/benzodiazepines/ anticholinergics etc +- sedation with propofol
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