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1.285 AUDIT OF THE ACTUAL PERFORMANCE OF RECHARGEABLE MEDTRONIC IMPLANTABLE PULSE GENERATOR (ACTIVA-RC) FOR DEEP BRAIN STIMULATION (DBS) THERAPY IN DYSTONIA
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Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79
appeared. No DYT-1 mutation or response to L-dopa were noticed. MRI was normal. Pharmacological treatment was ineffective. At qualification to the surgery the patients was unable to walk, needed help in most of daily activities. Global Dystonia Scale (GDS) score was 85. In April 2009 GPi DBS was implanted. At 6 months followup GDS was reduced to 60 points (by 30%). Major reduction of torticollis and dyphagia were noticed. The patients complained of severe dystonic movements in right upper limb and painful dystonia at shins and feet. In September 2010 STN DBS was implanted. At 6 months follow up with STN-DBS (C+1-2.0/90/130) and GPi-DBS (C+1-2.2/190/185), GDS was reduced to 48 points (by 44% and 20%). The patient was independent in most of daily activities, able to walk with walker. Conclusions: Utilization of multiple target for DBS – GPi and STN – might increase positive effect of neuromodulative treatment of GD. 1.284 THE CURATIVE EFFECT EVALUATIONS FOR BOTULINUM TOXIN-A TREATMENT OF SPASMODIC TORTICOLLIS WITH THE GUIDANCE OF EMG S.S. Chen, Z.H. Liang, C.L. Song. First Affiliated Hospital of Dalian Medical University, Dalian, China Objective: To evaluate the curative effect of botulinum toxin A (BTXA) local injection with the guidance of electromyography (EMG) for the treatment of spasmodic torticollis (ST). Methods: Forty-two patients with ST were diagnosed in the Department of Neurology, the First Affiliated Hospital of Dalian Medical University, from January 2003 to December 2010. In terms of the sound and myoelectric activity from EMG with evoked potential equipment from Medelec, Oxford, Britain, the focus would be ascertained and chosen as the injection site. The BTX-A were from Hengli, China. On the basis of Tsui scale, of each patient, T0, the grade before treatment, and T1, the lowest grade during the treatment, were recorded, and the remission rate was calculated as (T0−T1)/T0×100%. The final heal was defined as remission rate 100% with no recurrence for two years at least. Results: 1. T0: 11.19±5.74, T1: 2.5±6, Z=-0.653, Pfts;= 0, indicating that BTXA with EMG interference could ameliorate ST patients’ suffering; 2. Fifteen cases were final heal, accounting for 35.7%. Conclusion: The BTX-A local injection with the guidance of EMG is effective in the treatment of ST, the cure rate of which can be improved. 1.285 AUDIT OF THE ACTUAL PERFORMANCE OF RECHARGEABLE MEDTRONIC IMPLANTABLE PULSE GENERATOR (ACTIVA-RC) FOR DEEP BRAIN STIMULATION (DBS) THERAPY IN DYSTONIA M.J. Naushahi, N. Pavese, S. O’Riordan, P.G. Bain, C.T. Hopkins, D. Nandi. Imperial College Neuromodulation Group, Imperial College London, London, UK Objective: The advertised battery life for the newly developed Activa-RC for DBS therapy is up to nine-years, with daily or weekly recharge options [1]. However, no clinical published data exists with regards to Activa-RC battery life and its recharging frequency requirements. We describe our experience with the first ten ActivaRC implanted for the treatment of generalised non-DYT1 dystonia with bilateral GPi-DBS. Methods: All ten patients underwent successful bilateral GPi-DBS and subsequently the IPG (Kinetra) was cumulatively replaced 57 times in the 7.9±1.5 years (mean ±SD) prior to Activa-RC implantation, owing to high parameter settings. The Activa-RC recharging frequency requirements were audited using a twoweeks diary. Results: Recharging of the stimulator was required once or twice per day and for 80.3 (mean; range: 15–180) minutes per day. Excluding the costs of surgery and/or its complications, the cost
of the hardware devices (57 Kinetras) required for replacements in the preceding 7.9±1.5 years (mean ±SD) amounted to €473,100 (at 2010 prices). During the same period the cost would have been €144,000, if as expected, only one Activa-RC would have been required by each of the ten patients. Conclusion: Activa-RC has the potential to greatly decrease the cost of treating dystonic patients with GPi-DBS but does require more frequent recharging than previously suggested. This has significant life-style implications for the patient and may potentially have an effect on Activa-RC battery life. Reference(s) [1] http://www.medtronic.eu/your-health/parkinsons-disease/device/ our-dbs-therapy-products/activaRC/index.htm
1.286 LONG TERM EFFICACY OF PALLIDAL DEEP BRAIN STIMULATION IN PATIENT WITH DYT 6 DYSTONIA FROM INDIA C. Savant Sankhla1 , M. Sankhe2 , J. Ray3 . 1 Neurology, P D Hinduja National Hospital, 2 Neurosurgery, P D Hinduja Hospital and Medical Research Centre, Mumbai, 3 Neurosciences, S N Pradhan Center for Neurosciences, Kolkata, India Dystonia 6 is caused by mutations in the gene THAP1. Dystonia 6 is inherited as an autosomal dominant trait with reduced sexindependent penetrance. The disease locus, DYT6, was originally assigned to 8p21-q22 by linkage analysis in Amish-Mennonite families Based on the identification of the disease gene, DYT6 is now known to be located in 8p11.21. Few reports of successful treatment with Deep Brain Stimulation surgery are emerging. Fifty year old male with DYT6 dystonia was operated for his generalized dystonia four years ago. The selected target was posteroventero lateral nucleus of Gpi. The benefit of surgery remains excellent at the end of four years. This patient is also first DYT6 mutation reported from India 1.287 SYNERGETIC EFFECT OF INTRATHECAL BACLOFEN AND DEEP BRAIN STIMULATION IN TREATING DYSTONIA Y. Awaad, T. Rizk. KFMC, Riyadh, Saudi Arabia 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, 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. Objective: 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. 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: Two cases with primary dystonia with strong family history of dystonia. ITB pump showed 60–70% control of their dystonia. DBS was done after ITB pump implantation, achieving good control of their dystonia. Their dystonia became dependent on both modalities: with improvement of their dystonia up to 90%. Results: The dystonia movement score improved at 12 months (P < 0.001). The disability score improved at 12 months (P < 0.001). The dystonia movement score improved at 12 months (P < 0.001). The disability score improved at 12 months (P < 0.001). Conclusions: Based on the excellent result that was achieved using both ITB and DBS, both devices ITB and DBS could be tried in patients in order to achieve higher degree of control of their dystonia.
Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79
appeared. No DYT-1 mutation or response to L-dopa were noticed. MRI was normal. Pharmacological treatment was ineffective. At qualification to the surgery the patients was unable to walk, needed help in most of daily activities. Global Dystonia Scale (GDS) score was 85. In April 2009 GPi DBS was implanted. At 6 months followup GDS was reduced to 60 points (by 30%). Major reduction of torticollis and dyphagia were noticed. The patients complained of severe dystonic movements in right upper limb and painful dystonia at shins and feet. In September 2010 STN DBS was implanted. At 6 months follow up with STN-DBS (C+1-2.0/90/130) and GPi-DBS (C+1-2.2/190/185), GDS was reduced to 48 points (by 44% and 20%). The patient was independent in most of daily activities, able to walk with walker. Conclusions: Utilization of multiple target for DBS – GPi and STN – might increase positive effect of neuromodulative treatment of GD. 1.284 THE CURATIVE EFFECT EVALUATIONS FOR BOTULINUM TOXIN-A TREATMENT OF SPASMODIC TORTICOLLIS WITH THE GUIDANCE OF EMG S.S. Chen, Z.H. Liang, C.L. Song. First Affiliated Hospital of Dalian Medical University, Dalian, China Objective: To evaluate the curative effect of botulinum toxin A (BTXA) local injection with the guidance of electromyography (EMG) for the treatment of spasmodic torticollis (ST). Methods: Forty-two patients with ST were diagnosed in the Department of Neurology, the First Affiliated Hospital of Dalian Medical University, from January 2003 to December 2010. In terms of the sound and myoelectric activity from EMG with evoked potential equipment from Medelec, Oxford, Britain, the focus would be ascertained and chosen as the injection site. The BTX-A were from Hengli, China. On the basis of Tsui scale, of each patient, T0, the grade before treatment, and T1, the lowest grade during the treatment, were recorded, and the remission rate was calculated as (T0−T1)/T0×100%. The final heal was defined as remission rate 100% with no recurrence for two years at least. Results: 1. T0: 11.19±5.74, T1: 2.5±6, Z=-0.653, Pfts;= 0, indicating that BTXA with EMG interference could ameliorate ST patients’ suffering; 2. Fifteen cases were final heal, accounting for 35.7%. Conclusion: The BTX-A local injection with the guidance of EMG is effective in the treatment of ST, the cure rate of which can be improved. 1.285 AUDIT OF THE ACTUAL PERFORMANCE OF RECHARGEABLE MEDTRONIC IMPLANTABLE PULSE GENERATOR (ACTIVA-RC) FOR DEEP BRAIN STIMULATION (DBS) THERAPY IN DYSTONIA M.J. Naushahi, N. Pavese, S. O’Riordan, P.G. Bain, C.T. Hopkins, D. Nandi. Imperial College Neuromodulation Group, Imperial College London, London, UK Objective: The advertised battery life for the newly developed Activa-RC for DBS therapy is up to nine-years, with daily or weekly recharge options [1]. However, no clinical published data exists with regards to Activa-RC battery life and its recharging frequency requirements. We describe our experience with the first ten ActivaRC implanted for the treatment of generalised non-DYT1 dystonia with bilateral GPi-DBS. Methods: All ten patients underwent successful bilateral GPi-DBS and subsequently the IPG (Kinetra) was cumulatively replaced 57 times in the 7.9±1.5 years (mean ±SD) prior to Activa-RC implantation, owing to high parameter settings. The Activa-RC recharging frequency requirements were audited using a twoweeks diary. Results: Recharging of the stimulator was required once or twice per day and for 80.3 (mean; range: 15–180) minutes per day. Excluding the costs of surgery and/or its complications, the cost
of the hardware devices (57 Kinetras) required for replacements in the preceding 7.9±1.5 years (mean ±SD) amounted to €473,100 (at 2010 prices). During the same period the cost would have been €144,000, if as expected, only one Activa-RC would have been required by each of the ten patients. Conclusion: Activa-RC has the potential to greatly decrease the cost of treating dystonic patients with GPi-DBS but does require more frequent recharging than previously suggested. This has significant life-style implications for the patient and may potentially have an effect on Activa-RC battery life. Reference(s) [1] http://www.medtronic.eu/your-health/parkinsons-disease/device/ our-dbs-therapy-products/activaRC/index.htm
1.286 LONG TERM EFFICACY OF PALLIDAL DEEP BRAIN STIMULATION IN PATIENT WITH DYT 6 DYSTONIA FROM INDIA C. Savant Sankhla1 , M. Sankhe2 , J. Ray3 . 1 Neurology, P D Hinduja National Hospital, 2 Neurosurgery, P D Hinduja Hospital and Medical Research Centre, Mumbai, 3 Neurosciences, S N Pradhan Center for Neurosciences, Kolkata, India Dystonia 6 is caused by mutations in the gene THAP1. Dystonia 6 is inherited as an autosomal dominant trait with reduced sexindependent penetrance. The disease locus, DYT6, was originally assigned to 8p21-q22 by linkage analysis in Amish-Mennonite families Based on the identification of the disease gene, DYT6 is now known to be located in 8p11.21. Few reports of successful treatment with Deep Brain Stimulation surgery are emerging. Fifty year old male with DYT6 dystonia was operated for his generalized dystonia four years ago. The selected target was posteroventero lateral nucleus of Gpi. The benefit of surgery remains excellent at the end of four years. This patient is also first DYT6 mutation reported from India 1.287 SYNERGETIC EFFECT OF INTRATHECAL BACLOFEN AND DEEP BRAIN STIMULATION IN TREATING DYSTONIA Y. Awaad, T. Rizk. KFMC, Riyadh, Saudi Arabia 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, 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. Objective: 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. 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: Two cases with primary dystonia with strong family history of dystonia. ITB pump showed 60–70% control of their dystonia. DBS was done after ITB pump implantation, achieving good control of their dystonia. Their dystonia became dependent on both modalities: with improvement of their dystonia up to 90%. Results: The dystonia movement score improved at 12 months (P < 0.001). The disability score improved at 12 months (P < 0.001). The dystonia movement score improved at 12 months (P < 0.001). The disability score improved at 12 months (P < 0.001). Conclusions: Based on the excellent result that was achieved using both ITB and DBS, both devices ITB and DBS could be tried in patients in order to achieve higher degree of control of their dystonia.