- Email: [email protected]
Pallidal deep brain stimulation in the treatment of Meige syndrome
PJNNS 39 1–4 neurologia i neurochirurgia polska xxx (2014) xxx–xxx
Available online at www.sciencedirect.com
ScienceDirect journal homepage: http://www.elsevier.com/locate/pjnns 1 2 3
Short communication
Pallidal deep brain stimulation in the treatment of Meige syndrome
4 5
6 7 8 9
Q1
Michał Sobstyl a,*, Mirosław Ząbek a,1, Zbigniew Mossakowski b,1, Artur Zaczyński b,1 a b
Department of Neurosurgery, Postgraduate Medical Center, Warsaw, Poland Department of Neurosurgery, Bródno Mazovia Hospital, Warsaw, Poland
article info
abstract
Article history:
Introduction: Meige syndrome (MS) is characterized by blepharospasm, facial, oromandibu-
Received 17 January 2014
lar, and often cervical dystonia. The medical treatment of this condition is challenging and
Accepted 26 May 2014
unsuccessful over long time. Recent case reports and small clinical series showed that
Available online xxx
bilateral deep brain stimulation (DBS) of globus pallidus pars interna (GPi) improves dystonic
Keywords:
Materials and methods: We report on our experience in using bilateral GPi DBS in 3 cases of
Pallidal stimulation
MS. We present short-term (3 months) follow-up as well long-term (from 8 months to 36
Meige syndrome
months) results.
features of MS validated by Burk–Fahn–Marsden Dystonia Rating Scale (BFMDRS).
Deep brain stimulation
Preoperative and postoperative BFMDRS assessments were performed on each patient. The postoperative BFMDRS scores was done when both stimulators were switched on and compared to baseline scores. Results: Bilateral GPi DBS reduced the BFMDRS total movement score by 66% at short-term follow-up, and by 75% at long-term follow-up when compared to baseline scores. The BFMDRS total disability score was reduced by 34% at short-term follow-up, and by 47% at long-term follow-up when compared to baseline scores. Conclusions: Our results showed that bilateral GPi DBS in MS is effective and safe, if conservative treatment options failed. The benefit is not only observed at short-term 3 months period but is maintained at long-term follow-up ranging from 8 to 36 months. # 2014 Published by Elsevier Urban & Partner Sp. z o.o. on behalf of Polish Neurological Society.
12 10 11 13 14 15
16 17 18
1.
Introduction
Meige syndrome (MS) is an adult-onset segmental dystonia characterized by combination of blepharospasm and oromandibular (and frequently cervical) dystonia [1,2]. MS symptoms
may severely limited one's ability to perform the simplest activities of daily living. Blepharospasm may lead to embarrassment and withdrawal from social situations [1]. Moreover, severe blepharospasm can result in functional blindness. The efficiency of pharmacotherapy in MS is limited. Nowadays, the treatments for MS are botulinum toxin injections administered
* Corresponding author. Tel.: +48 22 32 65 779. E-mail address: [email protected] (M. Sobstyl). 1 Tel.: +48 22 32 65 779. http://dx.doi.org/10.1016/j.pjnns.2014.05.008 0028-3843/# 2014 Published by Elsevier Urban & Partner Sp. z o.o. on behalf of Polish Neurological Society.
Please cite this article in press as: Sobstyl M, et al. Pallidal deep brain stimulation in the treatment of Meige syndrome. Neurol Neurochir Pol (2014), http://dx.doi.org/10.1016/j.pjnns.2014.05.008
19 20 21 22 23 24
PJNNS 39 1–4
2
4 8 10 5 8 15 8
6 5 8 8 10 28 24
6 7 12 6 8 34 36
10 56 3
Eyes
38 2
Eyes
12
Clonazepam 3 mg, baclofen 80 mg, botulinum toxin/ clonazepam 1 mg Trihexyphenidyl 4 mg, clonazepam 2 mg, botulinum toxin/none Baclofen 80 mg, clonazepam 2 mg, botulinum toxin/ clonazepam 1 mg 22 Neck 25
Pharmacological treatment of a segmental dystonia such as MS is very challenging. Orally administered agents have little effect on dystonia, and higher doses needed for effective
1
73 74 75
Discussion
BFMDRS total disability score long-term follow-up
4.
BFMDRS total disability score short term follow-up
72
BFMDRS total disability score baseline
The mean preoperative BFMDRS total movement score was 25 and the mean preoperative BFMDRS disability score was 10. The postoperative short-term (3 months) assessment was performed in all patients. The mean postoperative BFMDRS total motor score was 9 at the short-term follow-up, and 6 at the long-term follow-up. The mean postoperative BFMDRS disability score was 7 at the short-term follow-up, and 5 at the long-term follow-up. Stimulation parameters and mode of stimulation for each patient are presented in Table 2.
BFMDRS total movement score long-term follow-up
63 64 65 66 67 68 69 70 71
Results
BFMDRS total movement score Short-term follow-up
3.
BFMDRS total movement score baseline
62
Long-term follow-up (months)
The study included three patients (2 female and 1 male) with medically refractory MS. Eligibility for the surgery was based on clinically diagnosed MS that remained completely refractory to pharmacotherapy, and poorly responded to botulinum toxin injections. The brain MR imaging in all patients showed no structural abnormalities. All patients had no history of exposure to neuroleptics. All patients were evaluated by a neurologist who had experiences in treatment of movement disorders. Preoperative and postoperative BFMDRS assessments were performed on each patient. All patients underwent bilateral GPi DBS starting from December 2010. The study received an Institutional Review Board Approval. The stereotactic surgery was performed under general anesthesia; GPi was selected as the stereotactic target. Stereotactic GPi planning was performed by means of a neuronavigation device (Stealth Station, Medtronic, Minneapolis) using Framelink 4 software. The depth of the implanted electrode was controlled by fluoroscopy. Skin was sutured immediately after the implantation of the electrodes and a postoperative CT was carried out with merging of preoperative MR images. Thereafter, DBS electrodes were connected to a Medtronic (2 patients) or a St. Jude (1 patient) single channel internal pulse generators (IPG). There were no intraoperative complications. One patient required a repeated surgery due to dislocation of the internal pulse generator. The patients' characteristics and BFMDRS scores are presented in Table 1.
Medications (per day) baseline/ long-term follow-up
36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61
Materials and methods
Duration of illness (years)
2.
Site of onset
35
Age at onset (years)
in the overactive muscles. Deep brain stimulation (DBS) of the globus pallidus pars interna (GPi) remains a treatment option for drug and botulinum toxin refractory MS. A few case reports or case series describe favorable short-term and long-term results of GPi stimulation for MS [1–3]. The main aim of the present study is to show the clinical efficacy of bilateral GPi stimulation in patients with refractory MS. We report on our experience in using bilateral GPi DBS in 3 cases of MS. We present short-term (3 months) and long-term (from 8 months to 36 months) follow-up results.
Patient
25 26 27 28 29 30 31 32 33 34
Table 1 – Clinical characteristics of three patients at baseline, short-term follow-up (3 months), and long-term follow-up (36 months in patient 1, 24 months in patient 2, and 8 months in patient 3). BFMDRS, Burk–Fahn–Marsden Dystonia Rating Scale.
neurologia i neurochirurgia polska xxx (2014) xxx–xxx
Please cite this article in press as: Sobstyl M, et al. Pallidal deep brain stimulation in the treatment of Meige syndrome. Neurol Neurochir Pol (2014), http://dx.doi.org/10.1016/j.pjnns.2014.05.008
PJNNS 39 1–4
3
neurologia i neurochirurgia polska xxx (2014) xxx–xxx
Table 2 – Programming parameters in three patients at long-term follow-up. In patients 1 and 2, a Medtronic DBS equipment was used, in patient 3 St. Jude DBS equipment was used. All patients received single channel internal pulse generators. Patients
76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 112 113 114 115 116 117 118 119 120 121
Long-term follow-up in months
Stimulation parameters at the long-term follow-up GPi right
Stimulation parameters at the long-term follow-up GPi left
1
36
2
24
3
8
Case (+), contact 1 ( ), contact 2 ( ) 120 ms, 130 Hz, 3.1 V Contact 1( ), contact 2 (+) 120 ms, 145 Hz, 3.6 V Case (+), contact 1 ( ), contact 2 ( ) 94 ms, 145 Hz, 2.5 mA
Case (+), contact 0 ( ), contact 1 ( ) 120 ms, 130 Hz, 2.2 V Case (+), contact 1 ( ), contact 2 ( ) 120 ms, 145 Hz, 2.8 V Contact 1 ( ), contact 2 ( ), contact 3 (+) 94 ms, 145 Hz, 3.0 mA
amelioration of dystonic movements are usually not tolerated by patients. Botulinum toxin injections are a treatment of choice for focal dystonia but can also play a role in diminishing the most disabling dystonic symptoms such as blepharospasm or retrocollis in segmental or even generalized dystonia. In the present study, pharmacological treatment proved ineffective, and the response to botulinum toxin injections was poor. Bilateral GPi DBS reduced the BFMDRS total movement score by 75%, and the total disability score by 47% at a long-term follow-up, as compared to baseline scores. Similar pattern of improvement in using GPi DBS was reported by other authors [3–5]. Lyons et al. noticed that in a group of three patients with MS examined at a 12-month follow-up the mean BFM score improved by 83% [3]. In the study by Ostrem et al., 6 patients with MS were followed up to 6 months. At 6 months, patients showed a mean improvement of 72% in the BFMDRS total movement score. The mean BFMDRS disability score showed a 38% improvement [4]. In the largest study (12 patients with MS) involving bilateral GPi DBS, Reese et al. found that the BFMDRS showed a mean improvement of 53% at a long-term follow-up [5]. In our study, the long-term follow-up showed that two patients reduced medical treatment after surgery, and one patient discontinued medication. This observation is in line with reports by others authors who treated patients with MS using bilateral GPi DBS [1–5]. The surgical procedure was well tolerated. One patient required translocation of the internal pulse generator. No other delayed hardware complications occurred. We did not observe other adverse events reported by some authors observing worsening of motor function in previously nondystonic body regions with bilateral GPi stimulation for MS. In report by Capelle et al. the patient developed stimulation-induced micrographia [1]. Ostrem et al. reported in four of six patients' new difficulty with coordination and slowness in motor functions like worsening of handwriting, typing, balance and walking [4]. These difficulties were mild and not evident on objective clinical examination [4]. Berman et al. observed induction of bradykinesia with pallidal deep brain stimulation in patients with MS [6]. These adverse events of GPi DBS on nondystonic body regions deserve further investigation. Not only motor functions are impaired by bilateral GPi DBS but also mood changes leading to even suicide may occur which were reported by Foncke et al. in 2 among 16 individuals after bilateral GPi in dystonic patients [7]. This observation stresses the importance of paying careful attention to mood effects when considering dystonic patients suitable candidates for GPi DBS for MS [8].
Our results contribute to the growing world-wide experience in using GPi DBS in MS. Motor function improvements illustrated by unbiased BFMDRS scores reflect disability improvements and patient's benefits after surgery.
122 123 124 125
5.
126
Conclusions
Bilateral GPi DBS is an effective and relatively safe procedure for intractable MS and reduces BFM motor and functional disability at short as well long-term follow-up.
127 128 129
Conflict of interest
130
None declared.
131
Financial support
132
None declared.
133
Ethics
134
The work described in this article has been carried out in accordance with The Code of Ethics of the World Medical Association (Declaration of Helsinki) for experiments involving humans; Uniform Requirements for manuscripts submitted to Biomedical journals.
135 136 137 138 139
references
140
[1] Capelle HH, Weigel R, Krauss JK. Bilateral pallidal stimulation for blepherospasm-oromandibular dystonia (Meige syndrome). Neurology 2003;60:2017–8. [2] Markaki E, Kefalopoulou Z, Georgiopoulous M, Paschali A, Constantoyannis C. Meige syndrome: a cranial dystonia treated with bilateral pallidal deep brain stimulation. Clin Neurol Neurosurg 2010;112:344–6. [3] Lyons MK, Birch BD, Hillman RA, Boucher OK, Evidente VG. Long-term follow-up of deep brain stimulation for Meige syndrome. Neurosurg Focus 2010;29(August (2)):1–5. [4] Ostrem JL, Marks WJ, Volz MM, Heath SL, Starr Ph. Pallidal deep brain stimulation in patients with cranial-cervical
141 142 143 144 145 146 147 148 149 150 151 152
Please cite this article in press as: Sobstyl M, et al. Pallidal deep brain stimulation in the treatment of Meige syndrome. Neurol Neurochir Pol (2014), http://dx.doi.org/10.1016/j.pjnns.2014.05.008
PJNNS 39 1–4
4 153 154 155 156 157 158 159 160
neurologia i neurochirurgia polska xxx (2014) xxx–xxx
dystonia (Meige syndrome). Mov Disord 2007;22: 1885–91. [5] Reese R, Gruber D, Schoenecker T, Bäzner H, Blahak C, Capelle HH, et al. Long-term clinical outcome in Meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord 2011;26:691–8. [6] Berman BD, Starr PA, Marks WJ, Ostrem JL. Induction of bradykinesia with pallidal deep brain stimulation in patients
with cranial-cervical dystonia. Stereotact Funct Neurosurg 2009;87:37–44. [7] Foncke EM, Schuurman PR, Speelman JD. Suicide after deep brain stimulation of the internal globus pallidus for dystonia. Neurology 2006;66:142–3. [8] Saleh C. How effective is GPi-DBS in the treatment of Meige's syndrome? Parkinsonism Relat Disord 2011;17:669.
161 162 163 164 165 166 167 168 169
Please cite this article in press as: Sobstyl M, et al. Pallidal deep brain stimulation in the treatment of Meige syndrome. Neurol Neurochir Pol (2014), http://dx.doi.org/10.1016/j.pjnns.2014.05.008
Available online at www.sciencedirect.com
ScienceDirect journal homepage: http://www.elsevier.com/locate/pjnns 1 2 3
Short communication
Pallidal deep brain stimulation in the treatment of Meige syndrome
4 5
6 7 8 9
Q1
Michał Sobstyl a,*, Mirosław Ząbek a,1, Zbigniew Mossakowski b,1, Artur Zaczyński b,1 a b
Department of Neurosurgery, Postgraduate Medical Center, Warsaw, Poland Department of Neurosurgery, Bródno Mazovia Hospital, Warsaw, Poland
article info
abstract
Article history:
Introduction: Meige syndrome (MS) is characterized by blepharospasm, facial, oromandibu-
Received 17 January 2014
lar, and often cervical dystonia. The medical treatment of this condition is challenging and
Accepted 26 May 2014
unsuccessful over long time. Recent case reports and small clinical series showed that
Available online xxx
bilateral deep brain stimulation (DBS) of globus pallidus pars interna (GPi) improves dystonic
Keywords:
Materials and methods: We report on our experience in using bilateral GPi DBS in 3 cases of
Pallidal stimulation
MS. We present short-term (3 months) follow-up as well long-term (from 8 months to 36
Meige syndrome
months) results.
features of MS validated by Burk–Fahn–Marsden Dystonia Rating Scale (BFMDRS).
Deep brain stimulation
Preoperative and postoperative BFMDRS assessments were performed on each patient. The postoperative BFMDRS scores was done when both stimulators were switched on and compared to baseline scores. Results: Bilateral GPi DBS reduced the BFMDRS total movement score by 66% at short-term follow-up, and by 75% at long-term follow-up when compared to baseline scores. The BFMDRS total disability score was reduced by 34% at short-term follow-up, and by 47% at long-term follow-up when compared to baseline scores. Conclusions: Our results showed that bilateral GPi DBS in MS is effective and safe, if conservative treatment options failed. The benefit is not only observed at short-term 3 months period but is maintained at long-term follow-up ranging from 8 to 36 months. # 2014 Published by Elsevier Urban & Partner Sp. z o.o. on behalf of Polish Neurological Society.
12 10 11 13 14 15
16 17 18
1.
Introduction
Meige syndrome (MS) is an adult-onset segmental dystonia characterized by combination of blepharospasm and oromandibular (and frequently cervical) dystonia [1,2]. MS symptoms
may severely limited one's ability to perform the simplest activities of daily living. Blepharospasm may lead to embarrassment and withdrawal from social situations [1]. Moreover, severe blepharospasm can result in functional blindness. The efficiency of pharmacotherapy in MS is limited. Nowadays, the treatments for MS are botulinum toxin injections administered
* Corresponding author. Tel.: +48 22 32 65 779. E-mail address: [email protected] (M. Sobstyl). 1 Tel.: +48 22 32 65 779. http://dx.doi.org/10.1016/j.pjnns.2014.05.008 0028-3843/# 2014 Published by Elsevier Urban & Partner Sp. z o.o. on behalf of Polish Neurological Society.
Please cite this article in press as: Sobstyl M, et al. Pallidal deep brain stimulation in the treatment of Meige syndrome. Neurol Neurochir Pol (2014), http://dx.doi.org/10.1016/j.pjnns.2014.05.008
19 20 21 22 23 24
PJNNS 39 1–4
2
4 8 10 5 8 15 8
6 5 8 8 10 28 24
6 7 12 6 8 34 36
10 56 3
Eyes
38 2
Eyes
12
Clonazepam 3 mg, baclofen 80 mg, botulinum toxin/ clonazepam 1 mg Trihexyphenidyl 4 mg, clonazepam 2 mg, botulinum toxin/none Baclofen 80 mg, clonazepam 2 mg, botulinum toxin/ clonazepam 1 mg 22 Neck 25
Pharmacological treatment of a segmental dystonia such as MS is very challenging. Orally administered agents have little effect on dystonia, and higher doses needed for effective
1
73 74 75
Discussion
BFMDRS total disability score long-term follow-up
4.
BFMDRS total disability score short term follow-up
72
BFMDRS total disability score baseline
The mean preoperative BFMDRS total movement score was 25 and the mean preoperative BFMDRS disability score was 10. The postoperative short-term (3 months) assessment was performed in all patients. The mean postoperative BFMDRS total motor score was 9 at the short-term follow-up, and 6 at the long-term follow-up. The mean postoperative BFMDRS disability score was 7 at the short-term follow-up, and 5 at the long-term follow-up. Stimulation parameters and mode of stimulation for each patient are presented in Table 2.
BFMDRS total movement score long-term follow-up
63 64 65 66 67 68 69 70 71
Results
BFMDRS total movement score Short-term follow-up
3.
BFMDRS total movement score baseline
62
Long-term follow-up (months)
The study included three patients (2 female and 1 male) with medically refractory MS. Eligibility for the surgery was based on clinically diagnosed MS that remained completely refractory to pharmacotherapy, and poorly responded to botulinum toxin injections. The brain MR imaging in all patients showed no structural abnormalities. All patients had no history of exposure to neuroleptics. All patients were evaluated by a neurologist who had experiences in treatment of movement disorders. Preoperative and postoperative BFMDRS assessments were performed on each patient. All patients underwent bilateral GPi DBS starting from December 2010. The study received an Institutional Review Board Approval. The stereotactic surgery was performed under general anesthesia; GPi was selected as the stereotactic target. Stereotactic GPi planning was performed by means of a neuronavigation device (Stealth Station, Medtronic, Minneapolis) using Framelink 4 software. The depth of the implanted electrode was controlled by fluoroscopy. Skin was sutured immediately after the implantation of the electrodes and a postoperative CT was carried out with merging of preoperative MR images. Thereafter, DBS electrodes were connected to a Medtronic (2 patients) or a St. Jude (1 patient) single channel internal pulse generators (IPG). There were no intraoperative complications. One patient required a repeated surgery due to dislocation of the internal pulse generator. The patients' characteristics and BFMDRS scores are presented in Table 1.
Medications (per day) baseline/ long-term follow-up
36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61
Materials and methods
Duration of illness (years)
2.
Site of onset
35
Age at onset (years)
in the overactive muscles. Deep brain stimulation (DBS) of the globus pallidus pars interna (GPi) remains a treatment option for drug and botulinum toxin refractory MS. A few case reports or case series describe favorable short-term and long-term results of GPi stimulation for MS [1–3]. The main aim of the present study is to show the clinical efficacy of bilateral GPi stimulation in patients with refractory MS. We report on our experience in using bilateral GPi DBS in 3 cases of MS. We present short-term (3 months) and long-term (from 8 months to 36 months) follow-up results.
Patient
25 26 27 28 29 30 31 32 33 34
Table 1 – Clinical characteristics of three patients at baseline, short-term follow-up (3 months), and long-term follow-up (36 months in patient 1, 24 months in patient 2, and 8 months in patient 3). BFMDRS, Burk–Fahn–Marsden Dystonia Rating Scale.
neurologia i neurochirurgia polska xxx (2014) xxx–xxx
Please cite this article in press as: Sobstyl M, et al. Pallidal deep brain stimulation in the treatment of Meige syndrome. Neurol Neurochir Pol (2014), http://dx.doi.org/10.1016/j.pjnns.2014.05.008
PJNNS 39 1–4
3
neurologia i neurochirurgia polska xxx (2014) xxx–xxx
Table 2 – Programming parameters in three patients at long-term follow-up. In patients 1 and 2, a Medtronic DBS equipment was used, in patient 3 St. Jude DBS equipment was used. All patients received single channel internal pulse generators. Patients
76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 112 113 114 115 116 117 118 119 120 121
Long-term follow-up in months
Stimulation parameters at the long-term follow-up GPi right
Stimulation parameters at the long-term follow-up GPi left
1
36
2
24
3
8
Case (+), contact 1 ( ), contact 2 ( ) 120 ms, 130 Hz, 3.1 V Contact 1( ), contact 2 (+) 120 ms, 145 Hz, 3.6 V Case (+), contact 1 ( ), contact 2 ( ) 94 ms, 145 Hz, 2.5 mA
Case (+), contact 0 ( ), contact 1 ( ) 120 ms, 130 Hz, 2.2 V Case (+), contact 1 ( ), contact 2 ( ) 120 ms, 145 Hz, 2.8 V Contact 1 ( ), contact 2 ( ), contact 3 (+) 94 ms, 145 Hz, 3.0 mA
amelioration of dystonic movements are usually not tolerated by patients. Botulinum toxin injections are a treatment of choice for focal dystonia but can also play a role in diminishing the most disabling dystonic symptoms such as blepharospasm or retrocollis in segmental or even generalized dystonia. In the present study, pharmacological treatment proved ineffective, and the response to botulinum toxin injections was poor. Bilateral GPi DBS reduced the BFMDRS total movement score by 75%, and the total disability score by 47% at a long-term follow-up, as compared to baseline scores. Similar pattern of improvement in using GPi DBS was reported by other authors [3–5]. Lyons et al. noticed that in a group of three patients with MS examined at a 12-month follow-up the mean BFM score improved by 83% [3]. In the study by Ostrem et al., 6 patients with MS were followed up to 6 months. At 6 months, patients showed a mean improvement of 72% in the BFMDRS total movement score. The mean BFMDRS disability score showed a 38% improvement [4]. In the largest study (12 patients with MS) involving bilateral GPi DBS, Reese et al. found that the BFMDRS showed a mean improvement of 53% at a long-term follow-up [5]. In our study, the long-term follow-up showed that two patients reduced medical treatment after surgery, and one patient discontinued medication. This observation is in line with reports by others authors who treated patients with MS using bilateral GPi DBS [1–5]. The surgical procedure was well tolerated. One patient required translocation of the internal pulse generator. No other delayed hardware complications occurred. We did not observe other adverse events reported by some authors observing worsening of motor function in previously nondystonic body regions with bilateral GPi stimulation for MS. In report by Capelle et al. the patient developed stimulation-induced micrographia [1]. Ostrem et al. reported in four of six patients' new difficulty with coordination and slowness in motor functions like worsening of handwriting, typing, balance and walking [4]. These difficulties were mild and not evident on objective clinical examination [4]. Berman et al. observed induction of bradykinesia with pallidal deep brain stimulation in patients with MS [6]. These adverse events of GPi DBS on nondystonic body regions deserve further investigation. Not only motor functions are impaired by bilateral GPi DBS but also mood changes leading to even suicide may occur which were reported by Foncke et al. in 2 among 16 individuals after bilateral GPi in dystonic patients [7]. This observation stresses the importance of paying careful attention to mood effects when considering dystonic patients suitable candidates for GPi DBS for MS [8].
Our results contribute to the growing world-wide experience in using GPi DBS in MS. Motor function improvements illustrated by unbiased BFMDRS scores reflect disability improvements and patient's benefits after surgery.
122 123 124 125
5.
126
Conclusions
Bilateral GPi DBS is an effective and relatively safe procedure for intractable MS and reduces BFM motor and functional disability at short as well long-term follow-up.
127 128 129
Conflict of interest
130
None declared.
131
Financial support
132
None declared.
133
Ethics
134
The work described in this article has been carried out in accordance with The Code of Ethics of the World Medical Association (Declaration of Helsinki) for experiments involving humans; Uniform Requirements for manuscripts submitted to Biomedical journals.
135 136 137 138 139
references
140
[1] Capelle HH, Weigel R, Krauss JK. Bilateral pallidal stimulation for blepherospasm-oromandibular dystonia (Meige syndrome). Neurology 2003;60:2017–8. [2] Markaki E, Kefalopoulou Z, Georgiopoulous M, Paschali A, Constantoyannis C. Meige syndrome: a cranial dystonia treated with bilateral pallidal deep brain stimulation. Clin Neurol Neurosurg 2010;112:344–6. [3] Lyons MK, Birch BD, Hillman RA, Boucher OK, Evidente VG. Long-term follow-up of deep brain stimulation for Meige syndrome. Neurosurg Focus 2010;29(August (2)):1–5. [4] Ostrem JL, Marks WJ, Volz MM, Heath SL, Starr Ph. Pallidal deep brain stimulation in patients with cranial-cervical
141 142 143 144 145 146 147 148 149 150 151 152
Please cite this article in press as: Sobstyl M, et al. Pallidal deep brain stimulation in the treatment of Meige syndrome. Neurol Neurochir Pol (2014), http://dx.doi.org/10.1016/j.pjnns.2014.05.008
PJNNS 39 1–4
4 153 154 155 156 157 158 159 160
neurologia i neurochirurgia polska xxx (2014) xxx–xxx
dystonia (Meige syndrome). Mov Disord 2007;22: 1885–91. [5] Reese R, Gruber D, Schoenecker T, Bäzner H, Blahak C, Capelle HH, et al. Long-term clinical outcome in Meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord 2011;26:691–8. [6] Berman BD, Starr PA, Marks WJ, Ostrem JL. Induction of bradykinesia with pallidal deep brain stimulation in patients
with cranial-cervical dystonia. Stereotact Funct Neurosurg 2009;87:37–44. [7] Foncke EM, Schuurman PR, Speelman JD. Suicide after deep brain stimulation of the internal globus pallidus for dystonia. Neurology 2006;66:142–3. [8] Saleh C. How effective is GPi-DBS in the treatment of Meige's syndrome? Parkinsonism Relat Disord 2011;17:669.
161 162 163 164 165 166 167 168 169
Please cite this article in press as: Sobstyl M, et al. Pallidal deep brain stimulation in the treatment of Meige syndrome. Neurol Neurochir Pol (2014), http://dx.doi.org/10.1016/j.pjnns.2014.05.008