Video 1
Patient exam at initial consultation. Segment 1A: The patient is seated in a chair. She is seen to have generalized myoclonus, worse with action, and dystonic posturing of the upper extremities, neck, and trunk. Segment 1B: The patient is walking down the hall; we see her ability to walk is limited by generalized myoclonus.
Figure 1
BrainLAB rendering of lead placement. A: Anterior view of lead placement in the bilateral GPi (pink). Subthalamic nuclei (green) and red nuclei (red) are illustrated for reference. B: Posterior view of lead placement. C: Coronal MRI reference imaging, which also highlights the bilateral globus pallidus externa (blue).
Table 1
DBS Settings.
| MONTHS POST-DBS | HEMISPHERE | CONTACTS | AMPLITUDE (mA) | PULSE WIDTH (µs) | FREQUENCY (Hz) |
|---|---|---|---|---|---|
| 1 | Left | C+, 2–, 3–, 4– | 2 | 90 | 130 |
| Right | C+, 5–, 6–, 7– | 2 | 90 | 130 | |
| 31 | Left | C+, 2– | 4.3 | 60 | 130 |
| Right | 3+, 6+, 2–, 4–, 5–, 7– | 4.3 | 60 | 130 |
Video 2
Patient exam 3 weeks post-DBS. Segment 2A: The patient is seated on an exam table. Her myoclonus is improved with various tasks in her upper extremities. Segment 2B: The patient is walking down the hall with a rolling walker. She is noted to have improvement in both her truncal dystonia and generalized myoclonus.
Table 2
Pre- and Post- DBS Scores. 1Comparison between pre-DBS scores and most recent values, 2Unified Myoclonus Rating Scale, 3Burke-Fahn-Marsden Dystonia Scale, 436-Item Short Form Survey (each item scored 0–100).
| SCALE/ITEM | PRE-DBS | POST-DBS (2 mo) | POST-DBS (6 mo) | POST-DBS (16 mo) | % IMPROVEMENT1 |
|---|---|---|---|---|---|
| UMRS2 | |||||
| Patient questionnaire | 26 | 7 | 3 | 3 | 88.5 |
| Patient global assessment | 3 | 2 | 1 | 1 | 66.7 |
| Myoclonus at rest | 70 | 5 | 2 | 2 | 97.1 |
| Stimulus sensitivity | 12 | 0 | 0 | 0 | 100.0 |
| Myoclonus action | 78 | 5 | 2 | 2 | 97.4 |
| Functional tests | 12 | 5 | 4 | 4 | 66.7 |
| Global disability | 3 | 2 | 2 | 2 | 33.3 |
| Negative myoclonus | 1 | 0 | 0 | 0 | 100.0 |
| Negative myoclonus severity | 3 | 0 | 0 | 0 | 100.0 |
| TOTAL (0–369) | 208 | 26 | 14 | 14 | 93.3 |
| BFMDS MOTOR3 | |||||
| Eyes | 4 | 0 | 0 | 0 | 100.0 |
| Mouth | 0 | 0 | 0 | 0 | – |
| Speech & swallowing | 0 | 0 | 0 | 0 | – |
| Neck | 6 | 4 | 0 | 0 | 100.0 |
| RUE | 4 | 1 | 1 | 1 | 75.0 |
| LUE | 6 | 1 | 1 | 1 | 83.3 |
| RLE | 6 | 1 | 3 | 3 | 50.0 |
| LLE | 9 | 1 | 0 | 3 | 66.7 |
| Trunk | 12 | 9 | 2 | 3 | 75.0 |
| TOTAL (0–120) | 47 | 17 | 7 | 11 | 76.6 |
| SF-364 | |||||
| Physical functioning | 5 | 55 | 50.0 | ||
| Role limitations – physical | 0 | 100 | 100.0 | ||
| Role limitations – emotional | 0 | 100 | 100.0 | ||
| Energy/fatigue | 35 | 60 | 25.0 | ||
| Emotional well-being | 24 | 56 | 32.0 | ||
| Social functioning | 12.5 | 100 | 87.5 | ||
| Pain | 22.5 | 90 | 67.5 | ||
| General health | 40 | 75 | 35.0 | ||