Table 1.
Demographic and Clinical Data
| Data | Subject #1 | Subject #2 | Subject #3 | Subject #4 | Subject #5 | Subject #6 | Subject #7 |
| Age at most recent evaluation (years) | 60 | 62 | 71 | 59 | 73 | 58 | 66 |
| Gender | Male | Male | Female | Male | Female | Male | Male |
| BSP onset (years) | 47 | 47 | 60 | 45 | 65 | 44 | 52 |
| CD onset (years) | 52 | 55 | 60 | 53 | 66 | 45 | 56 |
| AEO | Yes | Yes | Yes | Yes | Yes | Yes | Yes |
| Other dystonia | Lower facial | None | Lower facial | None | None | Laryngeal | Lower facial |
| Time to spread (years) | 5 | 8 | 0 | 8 | 1 | 1 | 4 |
| Duration of BSP (years) | 13 | 15 | 11 | 14 | 7 | 14 | 14 |
| Pattern of spread | BSP→CD | BSP→CD | BSP = CD | BSP→CD | BSP→CD | BSP→CD | BSP→CD |
| TWSTRS: total score | 28.25 | 31.25 | 20 | 30.5 | 30.75 | 16 | 32 |
| TWSTRS: severity score | 17 | 15 | 15 | 13 | 20 | 13 | 14 |
| TWSTRS: disability score | 4 | 9 | 6 | 9 | 6 | 3 | 13 |
| TWSTRS: pain score | 7.25 | 7.25 | 1.5 | 8.5 | 4.75 | 0 | 5 |
| TWSTRS: anterocollis score | 3 | 3 | 1 | 2 | 2 | 1 | 2 |
| Cervical EMG | Tonic | Tonic | Tonic | Na | Tonic | Tonic | Na |
| Relief from sensory tricks | None | None | None | Limited | None | None | Limited |
| Non-physiological head and/or appendicular tremor | No | No | No | No | No | No | No |
| Family history | No | Sister - BSP | No | Sister -BSP | No | Father - segmental craniocervical dystonia | Several 1st & 2nd degree relatives with BSP+/-CD |
Figure 1.
Photographs of Three Subjects with Blepharospasm and Anterocollis
Frontal, lateral, and posterior views of subjects #1 (A–C) and #2 (D–F). Subject #5 before (H) and after ([I] 1 month; [J] 2 months) bilateral GPi DBS.