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Table 1 Practical guide for initiation of medications and selection of symptomatic medical therapies

From: Medical treatment of dystonia

A. Questions to ask before initiating treatment:
1) “Does the patient really have dystonia?”
  - Exclude pseudodystonia and psychogenic dystonia
2) “Is there any (etiology-) specific treatment for the patient?”
  - Identify treatable dystonia (Table 2): neurometabolic disorders (DRD being the most important), heavy metal-related disorders (especially Wilson’s disease) and acquired disorders
3) Is there any coexisting phenomenology other than dystonia?
  - Identify and appropriately treat coexisting phenomenology such as parkinsonism (e.g. in RDP) or myoclonus (e.g. in myoclonus-dystonia syndrome or DYT11 dystonia)
4) “What treatment modality or modalities should be initiated?”
  - Selecting between medications vs. BoNT vs. DBS or combination (Table 3)
B. General principles of symptomatic medical treatment in dystonia
  • Trihexyphenidyl is a first-line agent
• Baclofen and clonazepam are typically second-line agent
• TBZ or clozapine may be considered as first-line agents in tardive dystonia
• Start low, go slow
 o Initiate at a low dose
 o Titrate up slowly
  ▪ Every 3–4 days in children and younger adults
  ▪ Every 1 week for older adults or patients prone to side effects
• Continue uptitration if non-sustained benefits or inadequate symptom control
• If side effects occur – may initially try holding the dose constant. If no improvement, or severe/intolerable side effects – lower the dose (modified from Ref [5])
 o If side effects disappear and patient still benefits:
  ▪ Consider combination therapy or try increasing the dose slowly again
 o If side effects disappear but no benefit:
  ▪ Consider discontinuation (may need slow tapering especially baclofen and clonazepam)
 o If side effects persist but patient still benefits:
  ▪ Consider lowering the dose further
 o If side effects persist and no benefit
  ▪ Consider discontinuation (or slow tapering)
 o If benefits are seen and symptoms are adequately controlled:
  ▪ Hold constant to see if benefits are sustained.
• Of note, sometimes trihexyphenidyl at a constant dose may require 2–4 weeks to reach peak benefit
• Trihexyphenidyl may have paradoxical effects at low doses
 o If this occurs – may try pushing to higher doses slowly
  1. A. Step-by-step approach before initiation of medical treatment in dystonia: a practical guide
  2. B. General principles of symptomatic medical treatment in dystonia. Further detail is described in the review
  3. Abbreviations: BoNT, botulinum toxin injection, DBS deep brain stimulation, DRD dopa-responsive dystonia, RDP rapid-onset dystonia parkinsonism, TBZ tetrabenazine