What are the surgical options for treating severe, treatment-resistant cervical dystonia?

Surgical Options for Severe Treatment-Resistant Cervical Dystonia

Deep brain stimulation (DBS) targeting the globus pallidus is the preferred surgical option for severe, treatment-resistant cervical dystonia that has failed to respond to at least three adequate medication trials and botulinum toxin injections. 1

Patient Selection Criteria for Surgical Intervention

• Surgical options should be considered only after failure of:

  • At least three adequate medication trials (including anti-dopaminergic drugs and alpha-2 adrenergic agonists)
  • Botulinum toxin injections (both type A and potentially type B)
  • Each medication trial should be at adequate dosage for at least 4 weeks 1

• Patients must demonstrate:

  • Stable and persistent symptoms (not transient)
  • Significant impact on health-related quality of life
  • Generally be above 20 years of age 1
  • Any comorbid psychiatric conditions should be stabilized for at least 6 months prior to surgery 1

Surgical Options Hierarchy

First-Line Surgical Option:

• Deep Brain Stimulation (DBS)
  • Target: Bilateral globus pallidus
  • Advantages:
    • Adjustable and reversible
    • Effective for complex forms of cervical dystonia
    • Suitable when more widespread dystonia is present 2
    • Maintains efficacy long-term 3
  • Considerations:
    • Improvement may not be immediate; effects can build up over weeks to months 1
    • Requires programming expertise and battery replacement
    • Higher upfront cost but potentially better long-term outcomes

Second-Line Surgical Options:

• Selective Peripheral Denervation

  • Most commonly performed peripheral surgical procedure 2
  • Modified Bertrand procedure involves:
    • C2-6 denervation
    • Myectomy of the splenius capitis and/or semispinalis capitis
    • Myotomy of the levator scapulae when indicated
    • Myotomy and selective denervation of the sternocleidomastoid 4
  • Best reserved for cervical dystonia refractory to botulinum toxin injections 3

• Selective Dorsal Ramisectomy

  • Has shown consistent results among peripheral surgical options 2
  • Lower efficacy compared to DBS but may be appropriate for patients who are not candidates for DBS

Emerging/Less Common Surgical Options:

• Cervical Dorsal Root Entry Zone (DREZ) Operation
  • May be considered as an alternative surgical treatment for segmental dystonia located in extremities
  • Can be used as a complementary procedure when other surgical interventions provide incomplete relief 5

Risk-Benefit Considerations

• Surgical risks must be weighed against potential benefits in each individual patient 1
• DBS has a relatively low risk profile and has been shown to be cognitively safe 3
• Peripheral denervation procedures carry risks of:
  • Sensory loss
  • Weakness
  • Potential for incomplete relief or recurrence

Expected Outcomes

• Response to DBS in primary dystonia is generally favorable, with marked benefit in:

  • Idiopathic generalized dystonia
  • Cervical dystonia
  • Segmental dystonia
  • Meige syndrome
  • Myoclonus-dystonia 3

• Patients with tardive dystonia may gain similar improvement to those with idiopathic dystonia 3

• Peripheral procedures typically provide less complete relief than DBS but may be appropriate in selected cases

Important Caveats

• Despite surgical advances, all current surgical treatments are palliative, not curative
• Regular follow-up is essential to monitor for complications and adjust treatment as needed
• Patients should have realistic expectations about the degree of symptom relief
• The combination of techniques utilized depends on the subtype and severity of cervical dystonia 4