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Table 3 Summary of selection of treatment modalities in dystonia

From: Medical treatment of dystonia

  1. After excluding disorders with etiology-specific therapies as shown in Table 1, symptomatic therapy in this table is then considered. This table summarizes treatment modalities in each dystonic disorder. Three major treatment modalities include medications, botulinum toxin injections and deep brain stimulation, among others. Of note, since DRD is an important “don’t-miss” diagnosis, “levodopa trial” is also included here under “Medication”. Of note, levodopa in this case serves for diagnostic and therapeutic purposes (“etiology-specific therapy”). In DYT5 or DRD, levodopa is the specific therapy which typically leads to dramatic and sustained benefit. If levodopa is used for other purposes such as “symptomatic therapy” for dystonia or coexisting parkinsonism, it is shown under “Others”
  2. “++” represents first-line modality or, for DBS, there is a low threshold to consider; “+” represents second-line or adjunctive modality when used as a combination therapy; “+/−” means benefits remain unclear. “?+” means that levodopa trial in cervical dystonia is questionable: it may be considered but not as strongly indicated as in limb-onset or generalized dystonia in adults
  3. 1LD trial in this case includes only for diagnostic and therapeutic purposes, particularly when DRD is suspected or cannot be excluded. LD as a symptomatic therapy is shown under “Others”
  4. 2BoNT can be used to target focally at the most debilitating muscle group(s) e.g. to relieve discomfort or improve range of motion for rehabilitation or hygiene
  5. 3DBS has been reported. These disorders are expanding indications for DBS, and may require further studies to confirm benefits
  6. 4Especially the ones with limb-onset dystonia
  7. 5DBS in DYT6 dystonia is generally less beneficial than DYT1 dystonia, however it has still been performed
  8. 6Pallidal (GPi) stimulation relieves both dystonia and myoclonus
  9. 7LD in this case is used as a symptomatic therapy to treat coexisting parkinsonism
  10. 8Avoding triggers is helpful in paroxysmal dyskinesias such as avoiding caffeine, alcohol and sleep deprivation in PNKD, and avoiding strenuous exercise in PED
  11. 9Need to search for GLUT1 deficiency syndrome in PED. Ketogenic diet can be initiated once the diagnosis is confirmed
  12. 10Especially when there is involvement of complex muscle groups or tongue
  13. 11Adductor spasmodic dysphonia (ADSD) typically has better response to BoNT than abductor spasmodic dysphonia (ABSD)
  14. 12Not recommended in embouchure dystonia
  15. 13In order to rule out DRD, and also Parkinson’s disease presenting with foot or lower limb dystonia
  16. Abbreviations: BoNT botulinum toxin injection, CBZ carbamazepine, CD cervical dystonia, CLZ clonazepam, CP cerebral palsy, DBS deep brain stimulation, GHB γ-hydroxybutyric acid or sodium oxybate, GLUT1 glucose transporter type 1, GPi globus pallidus interna, iLCrD idiopathic lower cranial dystonia, ITB intrathecal baclofen, LD levodopa, LVT levetiracetam, PED paroxysmal exercise-induced dystonia, PKD paroxysmal kinesigenic dyskinesia, PNKD paroxysmal non-kinesigenic dyskinesia, Rx, treatment, SD, spasmodic dysphonia, Sym Rx symptomatic treatment, TSFD task-specific focal dystonia