Table 2.
Overview on cases with dystonia and stable symptom relief after DBS discontinuation in the literature.
| AUTHOR | CLINICAL CHARACTERISTICS | CLINICAL COURSE | COMMENT | ||
|---|---|---|---|---|---|
| Goto et al. 2004 (3) | Idiopathic cervical dystonia, bilateral GPi DBS, Male, 53y at disease onset, DBS at 54y |
Improvement with DBS: TWSTRS 25 → 5 After 22m: bilateral off and stable symptom improvement >12m |
Continued pharmacotherapy, short disease duration |
||
| Hebb et al. 2007 (4) | Meige syndrome, bilateral GPi DBS, Female, 48y at disease onset, sympt. progression, DBS at 58y |
Improvement with DBS: GDRS 47 → 10 After 4y: bilateral off and stable symptom improvement at GDRS 20 for >12m |
Sequential DBS implant (8 months between left and right electrode) and sequential turnoff | ||
| Stavrinou et al. 2012 (12) | Acquired segmental dystonia, bilateral GPi DBS, Male, 23y at disease onset, DBS at 29y |
Improvement with DBS: BFM 34 → 26 After 27m: bilateral off and stable symptom improvement >6m |
Young age, acquired dystonia | ||
| Cheung et al. 2013 (2) | 2 cases: #1: inherited generalized dystonia (DYT1), bilateral GPi DBS Male, 11y at disease onset, DBS at 15y #2: generalized idiopathic dystonia, bilateral GPi DBS Male, 11y at disease onset, DBS at 18y |
#1: Improvement with DBS: BFM 64 → 0 After 5y: Unilateral off for 3m; after 2 more weeks mild symptom recurrence #2: Improvement with DBS: BFM 66 → 1 After 19m: Unilateral off since 7m and bilaterally for 2m; after 2 more weeks symptom recurrence |
Young age, short disease duration | ||
| Ruge et al. 2014 (11) | 3 cases: #1: inherited generalized dystonia (DYT1), bilateral GPi DBS Male, 8y at disease onset, DBS at 9.5y #2: inherited generalized dystonia (DYT1), bilateral GPi DBS Male, 6y at disease onset, DBS at 13y #3: inherited generalized dystonia (DYT1), bilateral GPi DBS Female, 9y at disease onset, DBS at 10.5y |
#1: Improvement with DBS: BFM 89 → 5.5 After 4.5y: bilateral off and stable symptom improvement at BFM 14 >14d #2: Improvement with DBS: BFM 69 → 0 After 9y: bilateral off and stable symptom improvement at BFM 13 >12m #3: Improvement with DBS: BFM 60 → 5 After 5y: bilateral off and stable symptom improvement at BFM 5.5 >14d |
Young age, short disease duration |
||
| Wolf et al. 2021 | Acquired generalized dystonia, bilateral Vim DBS followed by bilateral GPi DBS; Male, disease onset in childhood, DBS at 25y |
Improvement with DBS: BFM 80.5 → 67 After 76m: bilateral off and stable symptom improvement >47m |
Young age, acquired dystonia, switch from bilateral Vim to bilateral GPi DBS |
||
BFM = Burke Fahn Marsden scale; TWSTRS TSS = Toronto Western Spasmodic Torticollis Rating Scale Total Severity Score; GDRS = Global Dystonia Rating Scale; y = years; m = months; d = days.