Table. 1
Clinical Data on Four Patients with ET
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | |
|---|---|---|---|---|
| Age (years) | 79 | 70 | 52 | 38 |
| Gender | Male | Male | Male | Female |
| Age of tremor onset (years) | 35 | 6 | 12 | 16 |
| Tremor duration (years) | 44 | 64 | 40 | 22 |
| Family history of ET | Yes (Brother, Sister) | Yes (Sister) | Yes (Grandfather, Nephew) | Yes (Father, Grandmother) |
| Current ET medications | Pro 10 mg/d, Top 50 mg/d | None | Gab 1600 mg/d, Top 100 mg/d | Pro 80 mg/d |
| Mean Archimedes spiral score (range = 0–31) | 2.5 | 2.0 | 3.0 | 3.0 |
| International Co-operative Ataxia Rating Scale (range = 0–1001) | 24 | 13 | 27 | 37 |
| Posture/Gait (0–34) | 9 | 4 | 3 | 10 |
| Kinetic Functions (0–52) | 15 | 9 | 24 | 27 |
| Speech (0–8) | 0 | 0 | 0 | 0 |
| Oculomotor (0–6) | 0 | 0 | 0 | 0 |
| Activities-specific Balance Confidence Scale (ABC, range = 01–100) | 48.8 | 78.8 | 78.4 | 90.9 |
| Berg Balance Scale (range = 01–56) | 42 | 54 | 54 | 53 |
| POMA (range = 01–28) | 18 | 22 | 25 | 21 |
| Number of tandem missteps (range = 0–101) | 10 | 4 | 1 | 5 |
| GAITRite Data2 | ||||
| Velocity (m/s) | 0.79 [1.17] | 0.90 [1.17] | 1.18 [1.28] | 1.16 [1.28] |
| Cadence (steps/min) | 85.2 [102.0] | 100.1 [102.0] | 129.6 [125.1] | 87.5 [125.1] |
| Step length (m) | 0.55 [0.69] | 0.52 [0.69] | 0.60 [0.65] | 0.80 [0.65] |
| Double support time (%) | 28.7 [26.3] | 40.1 [26.3] | 27.5 | 32.4 |
| Step time difference (s) | 0.07 [0.03] | 0.04 [0.03] | 0.05 | 0.05 |
| Swing time CV (%) | 9.4 [4.5] | 6.7 [4.5] | 11.8 | 6.7 |