1. Synergetic effect of Intrathecal Baclofen and Deep Brain Stimulation in treating Dystonia51
Authors
Yasser Awaad, MD, MSc, FAAN, FAAP 1&2 & Tamer Rizk, MD, FRCPCH3
1Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI, USA.
2 Department of Pediatric Neurology, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia.
3 Pediatric Neurology Department, Altakhassusi Hospital, Suleiman Al-Habiib Medical Group, Riyadh, Saudi Arabia
Table 1. Clinical Characteristics of the 2 Pts before & 12 months after DBS
Introduction
Dystonia is a syndrome of sustained muscular contractions of opposing muscles with various etiologies.
The currently available symptomatic treatment strategies are quite effective for some of the various types of dystonia.
They help in decreasing involuntary movements, correcting abnormal posture, preventing contractures, reducing pain, and improving function and quality of life.
Intrathecal baclofen (ITB) and deep brain stimulation (DBS) were proved to be fairly effective in controlling dystonia when used separately.
Patient
DYT1 Status
Movement Score
Disability Score
Baseline
12 Mo 1 +
59.5
43.5 14 10 2
61.0
30.0 19 12
Dystonia score mean (±SD)
46.3 ± 21.3
21.0 ± 14.1
11.6 ± 5.5
6.5 ± 4.9
P Value
(P < 0.001)
Table 2. . Score of Health-Related Quality of Life, Cognition,
and Mood before and 12 Months after Surgery
Variable Value
Before Surgery
12 M after Surgery
P Value
General health
48 ± 23
62 ± 28
0.04
Physical role
55 ± 41
60 ± 37
0.68
Social functioning
55 ± 38
59 ± 38
0.81
Vitality
40 ± 24
50 ± 24
0.07
MMSE score*
29 ± 1
0.33
Physical functioning
40 ± 29
61 ± 30
0.007
Emotional Role
59 ± 48
77 ± 37
0.18
Pain
38 ± 33
57 ± 35
0.12
Mental Health
53 ± 21
65 ± 22
0.10
Beck Depression Inventory**
10 ± 8
8 ± 8
0.15
Purpose
We are reporting a synergetic effect of ITB and DBS when used simultaneously in two cases of primary generalized dystonia with excellent control of dystonia.
Methods
Our first case, a young boy with primary dystonia with strong family history of dystonia.
ITB pump showed % control of his dystonia, he reached very high doses of baclofen (ranged between 1200 mcg mcg/day).
DBS was done 14 months after ITB pump implantation, achieving good control of his dystonia.
His dystonia became dependent on both treatment modalities: “ITB and DBS” with improvement of his dystonia up to 90 %.
The second case, a nine years old girl, with severe dystonia . Again with strong family history
ITB pump was implanted and showed great improvement.
DBS was done 18 months after ITB pump implantation. markedly improved her movement and disability scores.
- Scores for the Medical Outcomes Study 36-item Short-Form General Health Survey (SF-36) can range from 0 (worst) to 100 (best).
*Scores for the Mini-Mental State Examination (MMSE) can range from 0 to 30. A score of less than 24 indicates cognitive impairment.
**Scores for the Beck Depression Inventory can range from 0 to 63, with a score of 20 or more indicating moderate-to-severe depression.
Results
The dystonia movement score improved from a mean (±SD) of 46.3 ± 21.3 before surgery to 21.0 ± 14.1 at 12 months (P < 0.001).
The disability score improved from 11.6 ± 5.5 before surgery to 6.5 ± 4.9 at 12 months (P < 0.001).
General health and physical functioning were significantly improved at month 12; there were no significant changes in measures of mood and cognition.
At the three-month evaluation, dystonia movement scores were significantly better with neurostimulation than without neurostimulation (24.6 ± 17.7 vs ± 12.3, P <0.001).
The mean postoperative BFM score was 25.7±21.4. Stimulation of GPi was associated with better outcomes compared to stimulation of VLp (P =0.0001).
Conclusions
In some dystonic patients, especially those with generalized primary dystonia, ITB and DBS can act simultaneously as augmentation therapy. This can reach up to 90% improvement regarding daily activity, hygiene, posture and other parameters of daily living activities.
Our patients showed great improvement in their dystonia scale, with marked improvement in their activities of daily living; self care, using utensils, walking Wheel chair use; this rendered them from totally dependent on others to reach a level of independency.
For our knowledge, these are the first reported cases in which both modalities were tried and successfully showed great improvement and control of their dystonia. The simultaneous use of ITB and DBS in patients who showed partial improvement to either device may be considered as an augmentation treatment. More patients are needed to be treated with both modalities to reach a final conclusion.
Based on the excellent result that was achieved using both ITB and DBS, the use of both devices ITB and DBS could be tried in patients in order to achieve higher degree of control of their dystonia.