Video 1
Pre- and post-video representing the symptoms of the patient.
Part I: Symptoms before GPi DBS surgery (video provided by the patient, which was recorded 2 months before GPi surgery). The patient presented with uncontrollable facial expressions and showed increased muscle tone in the face and neck. In addition, the patient can not relex her right leg.
Part II: Sitting and standing symptoms before cerebellar DBS surgery (2 years after GPi DBS).
Part III: Sitting and standing symptoms 6months after cerebellar DBS surgery.
Figure 1
Postoperative CT images fused with preoperative MR images demonstrating the positions of the implanted electrodes in GPi (1A, 1B, 1C). The two red orthogonal vertical lines refer to the Cartesian coordinate system in each view, while the two slashes, with or without red circles, present the trajectories of the implanted leads. In the center of each view, two green or red circles show the planned targets. AC, anterior commissure; PC, posterior commissure; MR, midline reference.
Table 1
The parameters for GPi DBS of this patienta.
| DBS site | Follow up time (months) | Stimulation parameters [Amplitude(V)/Frequency (Hz)/pulse width (ms)] |
|---|---|---|
| GPi | 1 | Left:2.3/140/60 Case(+) 1b (–) Right:2.0/140/60 Case(+) 6 (–) |
| GPi | 2 | Left:2.95/160/60 Case(+) 1 (–) Right:2.75/160/60 Case(+) 6 (–) |
| GPi | 4 | Left:2.45/75/70 Case(+) 1 (–) Right:2.75/75/70 Case(+) 6 (–) |
| GPi | 8 | Left:3.65/130/70 Case(+) 1(–)2(–) Right:3.75/130/70 Case(+) 6 (–) 7(–) |
| GPi | 9 | Left:2.85/70/70 Case(+) 1 (–) Right:3.0/70/70 Case(+) 5 (–) |
| GPi | 15 | Left:3.0/70/70 Case(+) 1 (–) Right:3.25/70/70 Case(+) 5 (–) |
| GPi | 23 | Left:3.15/70/70 Case(+) 1 (–) Right:3.45/70/70 Case(+) 5 (–) |
[i] a GPi DBS, Globus pallidus internus deep brain stimulation.
b For the electrodes from PINS, the contact numbers 1,2,3,4 are on the left and 5,6,7,8 on the right.
Figure 2
Determining the coordinates of the target superior cerebellar peduncles (SCPs) and dentate nuclei (DNs). The yellow arrows are directed to the SCPs, and orange arrows are directed to the DNs. The stereotactic coordinates of the SCP were identified on the sagittal section as the end point of the SCP to the brainstem (z) (2A) in line with the floor of the fourth ventricle on the horizonal section (y) (2B) and 3 mm lateral from the midline of the coronal section (x) (2C). Coordinates of the DN region were directly under the fastigium of the fourth ventricle on the mid-sagittal section (z, y) (2D) and 13 mm lateral from the midline on the T2 horizonal image (2E). The trajectory was planned using SCPs as the primary target. The coronal and sagittal angles were adjusted to attain a trajectory that traversed the origin of the SCP and target the DN. The trajectory was identified in the horizonal (2F) and sagittal sections (2G), and the entry point was 1 cm away from the sigmoid sinuses in the MRI image and at least 1 cm away from the posterior fixation posts and posterior frame bar in the CT image (2H). The 3D trajectory is shown in (2I).
Figure 3
Images showing the surgical procedure as well as the postoperative head images. During the first stage of trial stimulation, a stereotactic frame (Leksell) was installed under local anesthesia, and head computed tomography (CT) scan (Helex Sequence) was performed. Given the location of the target and the suboccipital approach, the head was fixed to the bed with the straight front piece of the Mayfield head rest during prone position for the anterior bar of the frame, and the long posterior fixation posts, which make the stereotactic frame, were placed lower than usual (3A). For the contacts of the leads to traverse both the SCP and DN, the customized DBS lead for research (L303, Beijing Pins Medical Co., Ltd., Beijing, China) was used. The lead featured 4.0 mm spacing between each of the four 3.0 mm electrodes at the distal end; the electrode spread is 24.0 mm with the ventral contact within SCP and the dorsal contact within DN. For the operation, two L303 DBS leads were implanted into the patient in the prone position under general anesthesia according to the planned coordinates and trajectory with the lead connected to the external extension wire for trial stimulation. The bilateral GPi leads were retained during this step of surgery (3E). After 1 week of trial stimulation, we removed the bilateral GPi DBS leads (3F) and connected the SCP DBS leads to the previous implanted pulse generator (G102R, PINS, Beijing Pins Medical Co., Ltd., Beijing, China). A 1.5 T T1- and T2-weighted MRI is allowed for patients with implanted DBS leads according to the manufacturer’s indications. Ventral contacts 1 (or 5 on the left side) and 2 (or 6) were close to the superior cerebellar peduncle (SCP) (3B), and dorsal contacts 3 (or 7) and 4 (or 8) were close to the dentate nuclei (DN) (3C). All four contacts of the left side are shown in (3D). The X-ray image (3E) showed both the new implanted electrodes in the cerebellum and the old electrode in the globus pallidus internus (GPi), while the (3F) showed only the new implanted electrodes in the cerebellum existing after explanted the old electrodes in GPi.
Table 2
Detailed information and reactions of the patient during the testing of each contact at 2-day follow up.a
| Position | Contact | Frequency (Hz) | Pulse width (µs) | Voltage (V) | Affected body parts | Side effects |
|---|---|---|---|---|---|---|
| Right | 1 | 130 | 60 | 2 | con.face | |
| 3 | ip.leg; con.face | pain in con.face | ||||
| Right | 2 | 130 | 60 | 2 | con.face | |
| 3 | ip.leg; con.face | |||||
| 4 | ip.leg; con.face | gaze deviation; lower speech tone | ||||
| 5 | ip.leg; con.face | ip.leaning | ||||
| Right | 3 | 130 | 60 | 3 | con.face | pain in con.face |
| 4 | ip.leg; con.face | Gaze deviation; dizziness; ip.leaning; nystagmus | ||||
| Right | 4 | 130 | 60 | 8 | con.face | Gaze deviation; |
| Left | 5 | 130 | 60 | 1 | ip.leg; con.face; con.arm; con.finger | |
| 2 | con.face | pain in con.face | ||||
| 3 | con.face | pain in con.face | ||||
| Left | 6 | 130 | 60 | 2 | con.face; ip.leg | |
| 3 | con.face; ip.leg | head ip.leaning | ||||
| 4 | con.face; ip.leg | head ip.leaning; pain in con.face | ||||
| Left | 7 | 130 | 60 | 2 | ip.leg | |
| 3 | ip.leg | head ip.leaning | ||||
| 4 | ip.leg | head leaning; dizziness | ||||
| Left | 8 | 130 | 60 | 7 | ip.leg | |
| 8 | ip.leg | ip.leaning |
[i] a Reaction in face was tingle; reaction in limbs was increases in muscle tonus accompanied by immediate relaxation. The first voltage number listed for each contact is the minimum voltage that causes patient’s reaction. Pain in face means the patient cannot tolerate the tingle and yell. ip, ipsilateral; con, contralateral; ip.leaning means the whole body including head and trunk lean to the ipsilateral side; head ip.leaning means only head leans to the ipsilateral side.
Table 3
Pre- and post-operative clinical scores following SCP DBS in a patient with CP.
| Scales | Subscale | Pre-op | 3 months post-surgery | 6 months post-surgery | |
|---|---|---|---|---|---|
| MAS | Upper extremities (right) | 3 | 2 | 1 | |
| Upper extremities (left) | 4 | 2 | 2 | ||
| Lower extremities (right) | 4 | 4 | 4 | ||
| Lower extremities (left) | 4 | 3 | 3 | ||
| BFMDRS | Movement scores | 92 | 66.5 | 59.5 | |
| Disability scores | 21 | 15 | 14 | ||
| VAS | 6 | 1 | 0 | ||
| SF-36 | Physical function | 0 | 15 | 20 | |
| Role, physical | 0 | 75 | 100 | ||
| Role, emotional | 0 | 100 | 100 | ||
| Body pain | 41 | 74 | 84 | ||
| Mental health | 32 | 52 | 52 | ||
| General health | 25 | 55 | 65 | ||
| Social function | 0 | 50 | 50 | ||
| Vitality | 25 | 65 | 70 | ||
| BAI | 9 | 2 | 2 | ||
| BDI | 14 | 6 | 4 | ||
| MoCA | 18 | 24 | 23 | ||
| Voice | Quality | Basal frequency (Hz) | 192.11 | 212.77 | 219.38 |
| Jitter (%) | 1.12 | 0.58 | 0.54 | ||
| Shimmer (dB) | 4.948 | 3.39 | 2.988 | ||
| Noise-harmonic ratio | 0.1822 | 0.114 | 0.0878 | ||
| Breath Sound Index | 0.6 | 2.08 | 2.15 | ||
| Irregularity | 1.72 | 1.4 | 0.81 | ||
| Maximum phonation time (s) | 3.56 | 7.36 | 9.12 | ||
| DSI index | –0.2 | 0.8 | 2.9 | ||
| Articulation | Jaw distance | 550.73 | 535.92 | 508.96 | |
| Tongue distance | 1283.79 | 649.92 | 1783.17 | ||
| Vowel space area (VSA) | 281914 | 278951 | 372958 | ||
| Fluency | DDK rate (syl/s) | 2.82 | 3.17 | 4.38 | |
| Self-value | Function | 2 | 0 | 0 | |
| Physiology | 11 | 4 | 2 | ||
| Emotion | 12 | 4 | 1 |
[i] SCP DBS, superior cerebellar peduncle deep brain stimulation; CP, cerebral palsy; MAS, Modified Ashworth Scale; BFMDRS, Burke-Fahn-Marsden Dystonia Rating Scale; VAS, visual analog scale; SF-36, 36-item Short Form Health Survey; BAI, Beck Anxiety Inventory; BDI, Beck Depression Inventory; MoCA, Montreal Cognitive Assessment.
Figure 4
Pre- and post-images representing the symptoms of the patient. (4A) Photograph recorded before GPi DBS surgery. Pre-(4B) and (4C) and post-operation-(4D) and (4E) (cerebellar DBS) photographs of the patient’s general appearance on standing and sitting. (4F) Vocal spectrum before and after the operation.
Table 4
The list of all the reported simulation parameters and lead model for cerebellar stimulationa.
| Authors | Galanda et al | Galanda et al | Galanda et al | Sokal et al | Taira et al | Brown et al |
|---|---|---|---|---|---|---|
| Year | 1997 | 2003 | 2007 | 2015 | 2019 | 2019 |
| Number of cases | 31 | 3 | 4 | 10 | 1 | 1 |
| Disease | Cerebral palsy | Cerebral palsy | Cerebral palsy | Cerebral palsy | Generalized dystonia | Acquired hemidystonia |
| Target | SCP | Anterior lobe of the cerebellum | Anterior lobe of the cerebellum | Deep region of anterior lobe | SCP and DN | DN |
| Lead model | TESLA LSP 330; Minneapolis, Medtronic | Model 3387, Medtronic | Model 3387, Medtronic | / | Model 3387, Medtronic | Model 3387, Medtronic |
| Contacts | / | / | / | / | 0 to 1–/3+ | 1–2–3+ |
| Frequency (Hz) | 200 or 130 | 185 | 185-200 | 130 | 200 | 130 |
| Pulse width (ms) | / | 210 | 210 | 150-180 | 150 | 60 |
| Voltage (V) | 1~6 | 0.5~4 | 0.5~2.5 | 1.4~2.4 | 8 | 1.2~2.8 |
| Reactions by threshold stimulation | Pronounced pathological posture; dyskinesias; Fear; | Unpleasant fear | Intense feeling of pleasure; increase in muscular tone; unpleasant fear; immediate relaxation and reduction of spasticity. | / | / | Leaning and head bobbing; Vocal impairment; appendicular ataxia; titubation. |
[i] a SCP, Superior cerebellar peduncle; DN, Dentate nucleus.