Table 1.
DYT12 patients with DBS treatment or pallidotomy reported in the literature
Study | Case No. | Age at Symptom Onset (Years) | Age at Surgery (Years) | BFMDRS Motor Score Before/After Surgery | Clinical Notes |
---|---|---|---|---|---|
Pittock et al.8 | 1 | 21 | 23 | Not reported | Unilateral pallidotomy with little effect on dystonia of left arm |
Deutschländer et al.6 | 2 | 21 | 24 |
50/120 No change post‐DBS |
Hypomimia, blepharospasm, torticollis, hypophonia, right hemidystonia, generalized hypokinesia; no effect of bilateral Gpi stimulation; DBS electrodes were removed |
Kamm et al.7 | 3 | 12 | 24 |
55.5/120 Post‐DBS: 49/120 |
Severe generalized dystonia of all four limbs, moderate dysarthria and slight hypophonia, tongue bradykinesia, and involuntary facial grimacing; modest improvement was observed mostly in the craniocervical region and trunk |
Brücke et al. (this study) | 4 | 17 | 22 |
28/120 Post‐DBS: 26/120 |
Generalized bradykinesia, more pronounced on the upper limbs, dystonic posturing of both hands, dysarthrophonia; no effect of bilateral Gpi stimulation |