Table 1
Statements regarding DBS in adults that reached consensus after the final stage. Of note, only the statements that reached consensus on including an item are displayed in this table. All other statements that also reached consensus on excluding items can be found in Tables 3, 4, 5, 6, 7.
| CONSENSUS STATEMENTS |
|---|
| Motor symptoms |
| 1. Scoring the severity of dystonic motor symptoms with a (validated) measurement instrument |
| 2. Video recording of the dystonic motor symptoms before surgery |
| 3. Video recording according to a set protocol |
| 4. Motor goals set by the patient he/she hopes to achieve with DBS treatment |
| Motor scales |
| 5. Generalized dystonia – BFMDRS |
| 6. Cervical dystonia – BFMDRS, TWSTRS, Tsui |
| 7. Other focal, segmental or multifocal dystonia – BFMDRS |
| 8. Myoclonus dystonia – BFMDRS, UMRS |
| Non-motor symptoms |
| 9. Determine severity of non-motor symptoms with a (validated) measurement instrument |
| 10. Preoperative assessment of cognitive status by a neuropsychologist |
| 11. By default carry out a brief neuropsychological exam, and when indicated a full neuropsychological exam |
| 12. Non-motor goals set by the patient he/she hopes to achieve with DBS treatment |
| Non-motor symptoms/domains to assess by a (validated) questionnaire |
| 13. Pain |
| 14. Depression |
| 15. Anxiety |
| 16. Cognitive functioning |
| 17. Quality of life |
| Involved care providers |
| 18. Involve an occupational therapist when considered necessary during the preoperative screening |
| 19. Involve a speech therapist when considered necessary during the preoperative screening |
| 20. Involve a physiatrist when considered necessary during the preoperative screening |
| Other aspects of importance to assess |
| 21. Participation |
| 22. Patient satisfaction |
| 23. Activities of daily living |
| Selection criteria |
| 24. Minimal number of drug trials |
| 25. Psychiatric comorbidity |
| 26. Cognitive impairments |
| 27. Etiology of dystonia |
| 28. Experienced limitations in daily life |
| 29. Quality of life |
| 30. Severity of non-motor symptoms |
| 31. Severity of motor symptoms |
| DBS related aspects |
| 32. Offer the option to patients to choose between rechargeable and non-rechargeable IPGs |
| 33. The presence of a neurologist in the operating room when there is awake DBS surgery |
| 34. Document IPG specification in the electronic patient file |
| Follow-up |
| 35. Structured protocolled post-operative follow-up moments at set intervals |
| 36. Assessment of motor symptoms during follow-up |
| 37. Assessment of non-motor symptoms during follow-up |
| 38. Video recording of dystonic motor symptoms during follow-up |
| 39. Document whether the use of pre-DBS botulinum toxin treatment is continued after DBS implantation |
| 40. Document whether medication use could be reduced or ceased after DBS implantation |
| 41. A specific scale to assess the severity of tremor |
| 42. Record side effects and complications during the course of DBS treatment |
| 43. In adults a protocolled follow-up moment between 3–5 years post-surgery |
Table 2
Statements regarding pediatric DBS that reached consensus at the final stage. Of note, only the statements that reached consensus on including an item are displayed in this table. All other statements that also reached consensus on excluding items can be found in Tables 3, 4, 5, 6, 7.
| CONSENSUS STATEMENTS |
|---|
| Motor symptoms |
| 1. Scoring the severity of dystonic motor symptoms with a (validated) measurement instrument |
| 2. Video recording of the dystonic motor symptoms before surgery |
| 3. Video recording according to a set protocol |
| 4. Motor goals set by the patient he/she hopes to achieve with DBS treatment |
| 5. Use a scale to measure severity of spasticity |
| 6. Determine motor goals not only on functional level, but also on skill level |
| Motor scales |
| 7. Generalized dystonia – BFMDRS, BADS |
| 8. Cerebral palsy – BFMDRS, BADS |
| 9. Other focal, segmental or multifocal dystonia – BFMDRS |
| 10. Myoclonus dystonia – BFMDRS, UMRS |
| Non-motor symptoms |
| 11. Determine severity of non-motor symptoms with a (validated) measurement instrument |
| 12. Preoperative assessment of cognitive status by a neuropsychologist |
| 13. Non-motor goals set by the patient he/she hopes to achieve with DBS treatment |
| Non-motor symptoms to assess by a (validated) questionnaire |
| 14. Pain |
| 15. Depression |
| 16. Anxiety |
| 17. Insomnia |
| 18. Cognitive functioning |
| 19. Quality of life |
| Involved care providers |
| 20. Standard involvement of occupational therapist during preoperative screening |
| 21. Standard involvement of speech therapist during preoperative screening |
| 22. Standard involvement of physiatrist during preoperative screening |
| 23. Standard involvement of physical therapist during preoperative screening |
| Other aspects of importance to assess |
| 24. Functioning at school |
| 25. Patient/Parent defined goals |
| 26. Patient satisfaction |
| 27. Parent/caretaker satisfaction |
| 28. Activities of daily living |
| Selection criteria |
| 29. Psychiatric comorbidity |
| 30. Etiology of dystonia |
| 31. Experienced limitations in daily life |
| 32. Quality of life |
| 33. Severity of non-motor symptoms |
| 34. Severity of motor symptoms |
| DBS related aspects |
| 35. Offer the option to patients to choose between rechargeable and non-rechargeable IPGs |
| 36. The presence of a neurologist in the operating room when there is awake DBS surgery |
| 37. Document IPG specification in the electronic patient file |
| Follow-up |
| 38. Structured protocolled post-operative follow-up moments at set intervals |
| 39. Assessment of motor symptoms during follow-up |
| 40. Assessment of non-motor symptoms during follow-up |
| 41. Video recording of dystonic motor symptoms during follow-up |
| 42. Document whether the use of pre-DBS botulinum toxin treatment is continued |
| 43. Document whether medication use could be reduced or ceased after DBS implantation |
| 44. Record side effects and complications during the course of DBS treatment |
| 45. In children after 1 year protocolled follow-up |
| 46. In children at least one other protocolled follow-up moment besides the 1 year follow-up |
| 47. In children after 3 years protocolled follow-up |
| 48. In children after 5 years protocolled follow-up |
Table 3
Items in the questionnaires of the first two rounds of the adult DBS Delphi process and their respective consensus percentages.
| STATEMENTS | ROUND 1* | ROUND 2* |
|---|---|---|
| Motor symptoms | ||
| Scoring the severity of dystonic motor symptoms with a (validated) measurement instrument | 100% | |
| Video recording of the dystonic motor symptoms before surgery | 89% | |
| Video recording according to a set protocol | 89% | |
| Motor goals set by the patient he/she hopes to achieve with DBS treatment | 94% | |
| Non-motor symptoms | ||
| Determine severity of non-motor symptoms with a (validated) measurement instrument | 100% | |
| Preoperative assessment of cognitive status by a neuropsychologist | 72% | |
| Standard involvement of a physiotherapist during preoperative screening | 56% |
|
| Standard involvement of an occupational therapist during preoperative screening | 44% |
|
| Standard involvement of a speech therapist during preoperative screening | 33% |
|
| Standard involvement of a physiatrist during preoperative screening | 33% |
|
| Non-motor goals set by the patient he/she hopes to achieve with DBS treatment | 89% | |
| Importance of the following aspects being assessed with a questionnaire: | ||
| Pain | 100% | |
| Fatigue | 56% | 58% |
| Insomnia | 56% | 42% |
| Depression | 100% | |
| Anxiety | 94% | |
| Cognitive functioning | 78% | |
| Quality of life | 100% | |
| Other aspects | ||
| Participation | 76% | |
| Mobility/Range of motion | 61% | 67% |
| Patient satisfaction | 100% | |
| Activities of daily living | 100% | |
| Selection criteria | ||
| Minimum age | 13% | 18% |
| Maximum age | 25% | 27% |
| Minimal number of drug trials | 77% | |
| Psychiatric comorbidity | 88% | |
| Cognitive impairments | 71% | |
| Travel distance to the hospital | 6% | 8% |
| Etiology of dystonia | 81% | |
| Disease duration | 27% | 42% |
| Experienced limitations in daily life | 94% | |
| Quality of life | 89% | |
| Severity of non-motor symptoms | 83% | |
| Severity of motor symptoms | 100% | |
| DBS related aspects | ||
| Offer the option to patients to choose between rechargeable and non-rechargeable IPGs | 71% | |
| Offer the option to patients to choose for sensing and/or steering systems | 15% | 0% |
| The presence of a neurologist in the operating room when there is awake DBS surgery | 80% | |
| Document IPG specification in the electronic patient file | 100% | |
| Follow-up | ||
| Structured protocolled post-operative follow-up moments at set intervals | 94% | |
| Assessment of motor symptoms during follow-up | 100% | |
| Assessment of non-motor symptoms during follow-up | 94% | |
| Video recording of dystonic motor symptoms during follow-up | 89% | |
| New items added during round 2 | ||
| Document whether the use of pre-DBS botulinum toxin treatment is continued after DBS implantation | 100% | |
| Document whether medication use could be reduced or ceased after DBS implantation | 91% | |
| A specific scale to assess the severity of tremor | 91% | |
| Use a brief neuropsychological exam by default to assess cognition preoperatively, use a full neuropsychological exam when indicated | 42% | |
[i] *Note: All items without consensus in bold.
Table 4
Consensus percentages during the first two rounds of the adult DBS Delphi process on motor symptom measurement instruments. Scales validated in literature for: 1Dystonia [19], 2Dystonia [25], 3Dystonia [25], 4Cervical dystonia [2021], 5Cervical dystonia [202122], 6Myoclonus dystonia [2324], myoclonus [24].
| MOTOR MEASUREMENT INSTRUMENTS | ROUND 1* | ROUND 2* |
|---|---|---|
| Generalized dystonia | ||
| BFMDRS1 | 90% | |
| UDRS2 | 38% | 0% |
| GDRS3 | 25% | 17% |
| Cervical dystonia | ||
| BFMDRS | 73% | |
| UDRS | 22% | 0% |
| GDRS | 22% | 0% |
| TWSTRS4 | 100% | |
| Tsui score5 | 89% | |
| Other focal, segmental or multifocal dystonia | ||
| BFMDRS | 91% | |
| UDRS | 50% | 17% |
| GDRS | 50% | 17% |
| Myoclonus dystonia | ||
| BFMDRS | 89% | |
| UMRS6 | 88% | |
[i] *Note: All items without consensus in bold.
Table 5
Items in the questionnaires of the first two rounds of the pediatric DBS Delphi process and their respective consensus percentages.
| STATEMENTS | ROUND 1* | ROUND 2* |
|---|---|---|
| Motor symptoms | ||
| Scoring the severity of dystonic motor symptoms with a (validated) measurement instrument | 100% | |
| Video recording of the dystonic motor symptoms before surgery | 91% | |
| Video recording according to a set protocol | 100% | |
| Motor goals set by the patient he/she hopes to achieve with DBS treatment | 100% | |
| Non-motor symptoms | ||
| Determine severity of non-motor symptoms with a (validated) measurement instrument | 100% | |
| Assessment of cognitive status by a neuropsychologist | 82% | |
| Standard involvement of physiotherapist during preoperative screening | 64%* |
|
| Standard involvement of occupational therapist during preoperative screening | 82% | |
| Standard involvement of speech therapist during preoperative screening | 82% | |
| Standard involvement of physiatrist during preoperative screening | 82% | |
| Non-motor goals set by the patient he/she hopes to achieve with DBS treatment | 91% | |
| Importance of the following aspects being assessed with a questionnaire: | ||
| Pain | 100% | |
| Fatigue | 50% | 50% |
| Insomnia | 80% | |
| Depression | 90% | |
| Anxiety | 90% | |
| Cognitive functioning | 80% | |
| Quality of life | 100% | |
| Other aspects | ||
| Functioning at school | 100% | |
| Mobility/Range of Motion | 64% | 50% |
| Patient/Parent defined goals | 100% | |
| Patient satisfaction | 100% | |
| Parent/caretaker satisfaction | 100% | |
| Activities of daily living | 100% | |
| Selection criteria | ||
| Minimum age | 56% | 56% |
| Minimal number of drug trials | 57% | 50% |
| Psychiatric comorbidity | 90% | |
| Cognitive impairments | 40% | 40% |
| Travel distance to the hospital | 0% | 0% |
| Etiology of dystonia | 80% | |
| Disease duration | 30% | 30% |
| Experienced limitations in daily life | 100% | |
| Quality of life | 91% | |
| Severity of non-motor symptoms | 82% | |
| Severity of motor symptoms | 100% | |
| DBS related aspects | ||
| Offer the option to patients to choose between rechargeable and non-rechargeable IPGs | 71% | |
| Offer the option to patients to choose for sensing and/or steering systems | 17% | 0% |
| The presence of a neurologist in the operating room when there is awake DBS surgery | 75% | |
| Document IPG specification in the electronic patient file | 100% | |
| Follow-up | ||
| Structured protocolled post-operative follow-up moments at set intervals | 100% | |
| Assessment of motor symptoms during follow-up | 91% | |
| Assessment of non-motor symptoms during follow-up | 82% | |
| Video recording of dystonic motor symptoms during follow-up | 100% | |
| New items added during round 2 | ||
| Use a scale to measure severity of spasticity | – | 78% |
| Record treatment goals of both parents and patients | – | 100% |
| Record side effects during DBS treatment | – | 100% |
| Record complications during DBS treatment | – | 100% |
| Document whether the use of pre-DBS botulinum toxin treatment is continued | – | 78% |
| Document whether medication use could be reduced or ceased after DBS implantation | – | 78% |
| Determine motor goals not only on functional level, but also on skill level | – | 89% |
[i] *Note: All items without consensus in bold.
Table 6
Consensus percentages during the first two rounds of the pediatric DBS Delphi process on motor symptom measurement instruments. Scales validated in literature for: 1Tested in dystonia in older children, but beware of age dependency in young children [262728], 2Dystonia in cerebral palsy [2729], 3Inherited or idiopathic dystonia, dystonia and choreoathetosis in dyskinetic cerebral palsy [3031], 4Cervical dystonia in adults [2021], 5Myoclonus dystonia [2324], myoclonus [24].
| MOTOR MEASUREMENT INSTRUMENTS | ROUND 1* | ROUND 2* |
|---|---|---|
| Generalized dystonia | ||
| BFMDRS1 | 100% | |
| BADS2 | 80% | |
| Cerebral palsy | ||
| BFMDRS | 100% | |
| BADS | 80% | |
| DIS3 | 50% | 43% |
| Other focal, segmental or multifocal dystonia | ||
| BFMDRS | 86% | |
| BADS | 60% | 57% |
| TWSTRS4 | 57% | 29% |
| Myoclonus dystonia | ||
| BFMDRS | 83% | |
| BADS | 50% | 40% |
| UMRS5 | 67% | 50% |
[i] *Note: All items without consensus in bold.
Table 7
Consensus percentages on the consensus meeting items of the adult and pediatric Delphi process
| CONSENSUS MEETING STATEMENTS ADULT DBS | AGREEMENT %* |
|---|---|
| 1. The UDRS and GDRS fall outside the scope of the set of agreed outcome measures in generalized dystonia for adults | 100% |
| 2. The UDRS and GDRS fall outside the scope of the set of agreed outcome measures in cervical dystonia for adults | 100% |
| 3. The UDRS and GDRS fall outside the scope of the set of agreed outcome measures in focal, segmental or multifocal dystonia for adults | 100% |
| 4. The UDRS and GDRS fall outside the scope of the set of agreed outcome measures in myoclonus dystonia for adults | 100% |
| 5. No requirement to use a measurement scale for fatigue and sleep problems, but to discuss this during history taking for adults | 78% |
| 6. By default involve a physical therapist in the preoperative screening phase for adults | 65% |
| 7. Involve an occupational therapist when deemed necessary during the preoperative screening for adults | 70% |
| 8. Involve a physiatrist when deemed necessary during the preoperative screening for adults | 70% |
| 9. Mobility/Range of motion is not checked by default during the preoperative screening and follow-up for adults | 68% |
| 10. Concerning selection criteria for adults, to not necessarily take into account minimal age, maximal age, travel distance to the hospital | 100% |
| 11. In adults a protocolled follow-up moment between 3–5 years post-surgery | 75% |
| 12. In adults by default carry out a brief neuropsychological exam, and when indicated a full neuropsychological exam | 93% |
| 13. Record side effects and complications during the course of DBS treatment | 100% |
| Statements regarding both adults and pediatric DBS | |
| 1. Both for adults and children not the option to by default choose sensing/steering electrodes | 100% |
| 2. Use of micro-electrode recording (MER) only when MRI is not possible* | – |
| CONSENSUS MEETING STATEMENTS PEDIATRIC DBS | AGREEMENT % |
| 1. The DIS falls outside the scope of the set of agreed outcome measures in cerebral paresis for children | 100% |
| 2. The BADS and TWSTRS fall outside the scope of the set of agreed outcome measures in focal, segmental or multifocal dystonia for children | 100% |
| 3. The BADS falls outside the scope of the set of agreed outcome measures in myoclonus dystonia for children | 100% |
| 4. Standard use of the UMRS in children with myoclonus dystonia | 93% |
| 5. No requirement to use a measurement scale for fatigue in children, but to discuss this during history taking | 71% |
| 6. Mobility/Range of motion not assessed by default during the preoperative screening and follow-up for children | 68% |
| 7. Concerning selection criteria for children to not necessarily take into account minimal age, number of drug trials, travel distance to the hospital | 94% |
| 8. Concerning selection criteria for children to not necessarily take into account travel distance and disease duration | 100% |
| 9. In children by default a protocolled follow-up moment at 1 year post-surgery | 100% |
| 10. In children by default a protocolled follow-up at 1 and 5 years post-surgery | 62% |
| 11. In children at least one other protocolled follow-up moment besides the 1 year follow-up | 88% |
| 12. In children after 3 years protocolled follow-up | 97% |
| 13. In children after 5 years protocolled follow-up | 82% |
[i] *Note: All items without consensus in bold.