Table 1:
Self-reported symptom severity rating, clinical hand grip and eyelid myotonia assessments, and electromyographic (EMG) myotonia testing
| Subjective Symptoms | |||||||
|---|---|---|---|---|---|---|---|
| Variable | Visit | N | Mean | Standard Deviation |
Comparison to Baseline (Difference) |
99% Confidence Interval (Difference) |
p-value |
| Self-reported Stiffness (0-9, 9=worst) | Baseline | 10 | 6 | 1.25 | |||
| Week 2 | 10 | 2.1 | 1.6 | −3.9 | (−5.76, −2.04) | <0.0001 | |
| Week 4 | 10 | 1.4 | 1.58 | −4.6 | (−6.46, −2.74) | <0.0001 | |
| Week 5 | 10 | 6.5 | 2.88 | 0.5 | (−1.36, 2.36) | 0.4627 | |
| Self-reported Weakness (0-9, 9=worst) | Baseline | 10 | 4 | 1.41 | |||
| Week 2 | 10 | 1.5 | 1.27 | −2.5 | (−4.69, −0.31) | 0.0039 | |
| Week 4 | 10 | 1 | 1.49 | −3 | (−5.19, −0.81) | 0.0008 | |
| Week 5 | 10 | 4.5 | 3.14 | 0.5 | (−1.69, 2.69) | 0.5327 | |
| Self-reported Pain (0-9, 9=worst) | Baseline | 10 | 4.6 | 2.5 | |||
| Week 2 | 10 | 1.6 | 1.65 | −3 | (−5.03, −0.97) | 0.0003 | |
| Week 4 | 10 | 0.9 | 1.37 | −3.7 | (−5.73, −1.67) | <0.0001 | |
| Week 5 | 10 | 4.5 | 3.14 | 0.3 | (−1.73, 2.33) | 0.6857 | |
| Clinical Myotonia/EMG Duration | |||||||
|---|---|---|---|---|---|---|---|
| Variable | Visit | N | Mean | Standard Deviation |
Comparison to Baseline (Ratio) |
99% Confidence Interval (Ratio) |
p-value |
| Hand Grip Opening Time (seconds) | Baseline | 10 | 9.27 | 14.68 | |||
| Week 2 | 10 | 4.43 | 9.52 | 0.47 | (0.25, 0.90) | 0.0030 | |
| Week 4 | 10 | 3.54 | 7.17 | 0.42 | (0.23, 0.80) | 0.0009 | |
| Week 5 | 10 | 9.9 | 15.26 | 0.96 | (0.51, 1.80) | 0.8601 | |
| Eyelid Opening Time (seconds) | Baseline | 10 | 10.76 | 11.96 | |||
| Week 2 | 10 | 8.07 | 13.85 | 0.60 | (0.34, 1.06) | 0.0193 | |
| Week 4 | 10 | 3.57 | 4.27 | 0.47 | (0.26, 0.83) | 0.0010 | |
| Week 5 | 10 | 14.33 | 18.8 | 1.12 | (0.63, 1.99) | 0.5869 | |
| Timed Up and Go (seconds) | Baseline | 9 | 11.43 | 4.84 | |||
| Week 2 | 9 | 9.44 | 3.25 | 0.84 | (0.74, 0.96) | 0.0012 | |
| Week 4 | 9 | 8.76 | 2.36 | 0.79 | (0.70, 0.90) | <0.0001 | |
| Week 5 | 9 | 10.83 | 5.01 | 0.93 | (0.82, 1.06) | 0.1683 | |
| EMG Duration ADM (milliseconds) | Baseline | 10 | 108247 | 228651 | |||
| Week 4 | 10 | 16750 | 14749 | 0.40 | (0.09, 1.77) | 0.0760 | |
| EMG Duration TA (microseconds) | Baseline | 10 | 40031 | 32453 | |||
| Week 4 | 10 | 23981 | 38138 | 0.34 | (0.15, 0.78) | 0.0023 | |
| EMG Frequency | |||||||
|---|---|---|---|---|---|---|---|
| Variable | Visit | N | Median | Quartiles (Q1, Q3) |
Comparison to Baseline (Median Difference) |
99% Confidence Interval (Median Difference) |
p-value |
| EMG Frequency ADM (0-15 insertions) | Baseline | 10 | 15 | (13, 15) | |||
| Week 4 | 10 | 12.5 | (10, 15) | −1.5 | (−6, 1) | 0.1250 | |
| EMG Frequency TA (0-15 insertions) | Baseline | 10 | 15 | (15, 15) | |||
| Week 4 | 10 | 12.5 | (13, 15) | 0.0 | (−9,0) | 0.1250 | |
ADB, abductor digiti minimi; TA, tibialis anterior