Table 1
Baseline and post-DBS UHDRS assessments
| Pre-DBS | 6 mo. | 12 mo. | 24 mo. | 36 mo. | 48 mo. | |
|---|---|---|---|---|---|---|
| Motor assessment | 80 | 70 | 75 | 39 | 57 | 58 |
| Chorea subscore | 19 | 15 | 10 | 4 | 6 | 6 |
| Behavioral assessment (severity/frequency) | 3/3 | 2/1 | 7/8 | 0/0 | 0/0 | 0/0 |
| Independence scale | 50 | 50 | NA | 10 | 10 | 10 |
| Functional capacity | 4 | 1 | NA | 1 | 1 | 1 |
| Verbal fluency raw score | NA | 6 | NA | 6 | 2 | 2 |
| Functional assessment | 5 | 1 | 1 | 1 | 1 | 1 |
Figure 1
Microscopic Analysis at the Level of the Tip of the Deep Brain Stimulation Electrode. (A) The tip of the deep brain stimulation (DBS) lead was placed in the internal globus pallidus (GPi) and was confirmed by postmortem analysis. Progressive atrophy of the GPi resulted in the lead being much closer to the internal capsule (∼1.3 mm) than would be predicted by microelectrode mapping and macrostimulation. (B) The tip of a DBS lead placed in the GPi confirmed using hematoxylin and eosin. Some gliosis and hemosiderin deposits are also seen. (C) Gliosis surrounding the tip of the DBS lead as demonstrated by glial fibrillary acidic protein immunohistochemistry. (D) A rim of meningothelial cells near the lead defect as seen by immunohistochemistry using epithelial membrane antigen.
Figure 2
Fusion of Magnetic Resonance and Computed Tomography Images Showing the Lead Locations at the Time of Deep Brain Stimulation Surgery. The deep brain stimulation lead was placed within the internal globus pallidus and was intended to be >2–3 mm away from the internal capsule. The physiology was confirmed by postoperative imaging.
Table 2
Reported Cases Using DBS in the Setting of Huntington’s Disease
| Study | Patients, n | Target (Bilateral) | Follow-up, months | UHDRS Chorea subscore improvement |
|---|---|---|---|---|
| Present study | 1 | GPi | 48 | 68% |
| Zittel et al.19 | 3 | GPi | 12–36 | 40%–58% |
| Wojtecki et al.28 | 6 | GPi and GPe | 6 | 60% |
| Gonzalez et al.10 | 7 | GPi | 36 | 58% |
| Gruber et al.15 | 1 | GPi STN | 48 | 50% |
| Cislaghi et al.22 | 1 | GPi | 48 | 67% |
| Lopez-Sendon Moreno et al.20 | 1 | GPi | 60 | 56% |
| Huys et al.11 | 1 | GPi | 12 | NA |
| Velez-Lago et al.5 | 2 | GPi | 12 | 73% |
| Spielberger et al.21 | 1 | GPi | 48 | 75% |
| Garcia-Ruiz et al.13 | 1 | GPi | 12 | NA |
| Kang et al.23 | 2 | GPi | 24 | 50% and 63% |
| Groiss et al.18 | 1 | GPi | NA | NA |
| Ligot et al.27 | 5 | GPe | 12–19 | NA |
| Biolsi et al.14 | 1 | GPi | 48 | 21% |
| Fasano et al.17 | 1 | GPi | 12 | 77% |
| Hebb et al.12 | 1 | GPi | 12 | 50% |
| Fawcett et al.16 | 1 | GPi | 4 | 56% |
| Moro et al.25 | 1 | GPi | 8 | 64–76% |