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P7.17 Gait impairment following subthalamic deep brain stimulation in Parkinson's disease: multi-center prospective trial of parameter adjustments
S94 P7.17 Gait impairment following subthalamic deep brain stimulation in Parkinson’s disease: multi-center prospective trial of parameter adjustments A. Fasano1 , F. Morgante2 , N. Modugno3 , M. Zibetti4 , F. Lena3 , L. Lopiano4 , C. Piano1 , A.R. Bentivoglio1 1 Department of Neuroscience, UCSC, Rome, Italy, 2 Dipartimento di Neuroscienze, Scienze Psichiatriche ed Anestesiologiche, Universit` a di Messina, Messina, Italy, 3 IRCCS Neuromed, Pozzilli (IS), Italy, 4 Dipartimento di Neuroscienze, Universit` a di Torino, Turin, Italy Introduction: The benefit provided by Deep Brain Stimulation of the subthalamic nucleus (STN-DBS) in patients with Parkinson’s disease (PD) is excellent for the appendicular levodopa-responsive signs. However, the outcome of axial motor symptoms it is often disappointing after surgery, causing significant disability as the disease progresses. Objectives: To prospectively assess the validity of a standardized algorithm of stimulating parameters in a consecutive sample of PD patients presenting with gait impairment after STN-DBS. Methods: Eighteen PD patients (age: 61.2±6.0 year, disease duration: 11.9±2.6, follow-up after surgery: 35.6±34.2 months) were selected in four Italian DBS center by means of a chart review. They underwent clinical assessment (including UPDRS and Gait and Fall questionnaire GFQ) at enrollment visit and one month after each one of the reprogramming sessions. The algorithm encompassed: increase of stimulation amplitude (+0.5 Volt bilateral) or dopaminergic drugs (+20% of levodopa equivalent daily dose), activation of a more dorsal contact in case of foot dyskinesias, reduction of stimulating frequency (80 Hz), and reduction by 0.4 volt of the stimulation within the STN contralateral to the less affected leg (improving lower limbs symmetry). Results: Mean GFQ at baseline was 22.2±12.6 and it was significantly reduced at latest visit (13.4±10.0, p = 0.004). UPDRS scores did not change. One-side voltage reduction and 80 Hz-stimulation were the most successful strategies (both employed in 5 cases). In two cases the activation of a dorsal contact (supposed to impact on the pallido-fugal projections) efficiently reduced disabling foot dyskinesias, in four cases medication or stimulation increase improved gait. Finally, two patients did not improve with any of the strategies enclosed in the algorithm. Conclusions: This first prospective trial validating a parameters adjustment algorithm confirms that reprogramming sessions may be useful in the management of gait impairment following STN-DBS.
P7.18 Propriospinal myoclonus related to a thoraco-lumbar syrinx neurophysiological confirmation R. Arunachalam1 , D. Allen1 , A. Pinto2 1 Department of Clinical Neurophysiology, Wessex Neurological Centre, Southampton, United Kingdom, 2 Department of Neurology, Wessex Neurological Centre, Southampton, United Kingdom Introduction: Propriospinal myoclonus is a rare movement disorder comprising myoclonic jerks of spinal origin, with caudal and rostral propagation. Typically the jerks involve the abdominal wall and are positional being worse or precipitated by lying down [1]. Objectives: We report a case of propriospinal myoclonus in a young woman, related to a small thoracic syrinx and associated with scoliosis. Methods: Multi-level surface EMG recordings were performed to assess the propagation pattern. We also recorded video of typical jerks and performed further neurophysiological investigations and MR Imaging. Results: Nerve conduction, EMG, SEPs and EEG studies were all normal, in keeping with the spinal origin of the jerks. Multi-level surface EMG recordings confirmed that jerks arose at the upper lumbar level musculature, with propagation to rostral and caudal segments. Velocity of spinal spread was calculated. Bursts of EMG activity were of long duration and possessed a stereotyped decay pattern. This and the pattern of jerks were analysed. Conclusions: Propriospinal myoclonus can be diagnosed based on clinical and neurophysiological findings. A definitive cause is only found in a minority of patients. We report a case related to a thoracic syrinx and demonstrate the clinical and neurophysiological findings. Reference(s) Roze et al. Propriospinal myoclonus revisited. Neurology 2009; 72: 1301 1309
Poster presentations: Poster session 7. Movement disorders
2
P7.19 Sympathetic skin response and cardiac sympathetic denervation in Parkinson’s disease S.J. Kim1 Department of Neurology, Busan Paik Hospital, Busan, Korea, Republic
1
Introduction: Sympathetic skin response (SSR) is a simple electrophysiologic test used to evaluate the reflex activity of sympathetic sudomotor pathways. Several studies showed SSR is altered in most patients with Parkinson’s disease (PD) and a correlation between SSR values and severity of disease. But most previous studies found no association of SSR values and cardiovascular autonomic function tests. Objectives: To study a correlation of SSR values and the parameter of myocardiac 123 I-Metaiodobenzylguanidine (MIBG) scan which can evaluated cardiac sympathetic denervation objectively. Methods: We investigated SSR and myocardiac 123 I-Metaiodobenzylguanidine (MIBG) scan in 20 patients with idiopathic PD. SSR was elicited by electrical stimulation of the right and left median nerves and simultaneously recorded on the palms of both hands. Cardiac MIBG uptake was assessed Cardiac MIBG uptake was assessed qualitatively on planar studies and the regions of interest were drawn over the heart and the mediastinum to calculate the heart/mediastinum (H/M) ratio. Results: A statistically significant correlation in SSR amplitudes on more affected side and H/M ratio was found, but there were no correlations between SSR latencies on more affected side or parameters of SSR values on less affected side and H/M ratio. Conclusion: These findings suggest that denervation of sympathetic sudomotor system and sympathetic cardiac system in PD patients may occur concurrently.
P7.20 Bilateral, pallidal deep-brain stimulation in primary and non-primary dystonia: a prospective long-term follow-up study L. Romito1 , G. Zorzi2 , M. Ciceri3 , A. Franzini4 , N. Nardocci2 , A. Albanese1 1 Movement Disorder Department, Fondazione IRCCS Istituto Neurologico “Carlo Besta” and Istituto di Neurologia, Universit` a Cattolica del Sacro Cuore, Milan, Italy, 2 Department of Child Neurology, Fondazione IRCCS 3 Istituto Neurologico “Carlo Besta”, Milan, Italy, Movement Disorder Department, Fondazione IRCCS Istituto Neurologico, Milan, Italy, 4 Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milan, Italy Introduction: Deep brain stimulation of globus pallidum internum (GPiDBS) is currently the most effective treatment for advanced, medically refractory dystonia. Objectives: The aim of this study was to examine the long-term efficacy and safety of GPi-DBS in the treatment of primary dystonia (PD) or nonprimary dystonia (N-PD) in children and adults. Methods: We studied a series of sixty-four consecutive patients (28 F/36 M) with severe focal, segmental or generalized dystonia who received a stereotactical bilateral GPi implant for DBS in the period 1998 2009 at “C. Besta” Institute. Among them, 41 patients had a diagnosis of PD and 23 of N-PD. We compared motor outcome at last available FU with their status preoperatively and after 1 year of treatment. Dystonia was assessed with the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). Adverse events and sides effects were recorded. Results: Mean age at implant was 29.4±17.2 years; mean age at disease onset was 16.5±12.8 years. Follow-up (FU) duration was 3.8±2.5 years. At last FU, in the PD group, the mean BFMDRS movement score improved from 53.3±28.3 before surgery to 31.7±28.6 post-operatively (p = 0.007; mean improvement 40.5%); in the N-PD group, the mean BFMDRS movement score improved from 55.0±26.8 before surgery to 34.8±27.2 post-operatively (p = 0.008; mean improvement 36.7%). In the entire series, motor improvement observed at 1 year (42%) was maintained at last FU (39%), but individual slight decay were noted especially in N-PD group. The safety profile was positive. Conclusions: Bilateral GPi-DBS provides sustained motor benefit in the long-term observation, up to about seven years. Some clinical decline and new dystonic symptoms may occur in the long term FU in a subgroup of patients. In general, the outcomes for primary dystonias is more favourable compared to the non-primary or secondary forms. More studies are required to establish the factors predicting the long-term outcome.
P7.18 Propriospinal myoclonus related to a thoraco-lumbar syrinx neurophysiological confirmation R. Arunachalam1 , D. Allen1 , A. Pinto2 1 Department of Clinical Neurophysiology, Wessex Neurological Centre, Southampton, United Kingdom, 2 Department of Neurology, Wessex Neurological Centre, Southampton, United Kingdom Introduction: Propriospinal myoclonus is a rare movement disorder comprising myoclonic jerks of spinal origin, with caudal and rostral propagation. Typically the jerks involve the abdominal wall and are positional being worse or precipitated by lying down [1]. Objectives: We report a case of propriospinal myoclonus in a young woman, related to a small thoracic syrinx and associated with scoliosis. Methods: Multi-level surface EMG recordings were performed to assess the propagation pattern. We also recorded video of typical jerks and performed further neurophysiological investigations and MR Imaging. Results: Nerve conduction, EMG, SEPs and EEG studies were all normal, in keeping with the spinal origin of the jerks. Multi-level surface EMG recordings confirmed that jerks arose at the upper lumbar level musculature, with propagation to rostral and caudal segments. Velocity of spinal spread was calculated. Bursts of EMG activity were of long duration and possessed a stereotyped decay pattern. This and the pattern of jerks were analysed. Conclusions: Propriospinal myoclonus can be diagnosed based on clinical and neurophysiological findings. A definitive cause is only found in a minority of patients. We report a case related to a thoracic syrinx and demonstrate the clinical and neurophysiological findings. Reference(s) Roze et al. Propriospinal myoclonus revisited. Neurology 2009; 72: 1301 1309
Poster presentations: Poster session 7. Movement disorders
2
P7.19 Sympathetic skin response and cardiac sympathetic denervation in Parkinson’s disease S.J. Kim1 Department of Neurology, Busan Paik Hospital, Busan, Korea, Republic
1
Introduction: Sympathetic skin response (SSR) is a simple electrophysiologic test used to evaluate the reflex activity of sympathetic sudomotor pathways. Several studies showed SSR is altered in most patients with Parkinson’s disease (PD) and a correlation between SSR values and severity of disease. But most previous studies found no association of SSR values and cardiovascular autonomic function tests. Objectives: To study a correlation of SSR values and the parameter of myocardiac 123 I-Metaiodobenzylguanidine (MIBG) scan which can evaluated cardiac sympathetic denervation objectively. Methods: We investigated SSR and myocardiac 123 I-Metaiodobenzylguanidine (MIBG) scan in 20 patients with idiopathic PD. SSR was elicited by electrical stimulation of the right and left median nerves and simultaneously recorded on the palms of both hands. Cardiac MIBG uptake was assessed Cardiac MIBG uptake was assessed qualitatively on planar studies and the regions of interest were drawn over the heart and the mediastinum to calculate the heart/mediastinum (H/M) ratio. Results: A statistically significant correlation in SSR amplitudes on more affected side and H/M ratio was found, but there were no correlations between SSR latencies on more affected side or parameters of SSR values on less affected side and H/M ratio. Conclusion: These findings suggest that denervation of sympathetic sudomotor system and sympathetic cardiac system in PD patients may occur concurrently.
P7.20 Bilateral, pallidal deep-brain stimulation in primary and non-primary dystonia: a prospective long-term follow-up study L. Romito1 , G. Zorzi2 , M. Ciceri3 , A. Franzini4 , N. Nardocci2 , A. Albanese1 1 Movement Disorder Department, Fondazione IRCCS Istituto Neurologico “Carlo Besta” and Istituto di Neurologia, Universit` a Cattolica del Sacro Cuore, Milan, Italy, 2 Department of Child Neurology, Fondazione IRCCS 3 Istituto Neurologico “Carlo Besta”, Milan, Italy, Movement Disorder Department, Fondazione IRCCS Istituto Neurologico, Milan, Italy, 4 Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milan, Italy Introduction: Deep brain stimulation of globus pallidum internum (GPiDBS) is currently the most effective treatment for advanced, medically refractory dystonia. Objectives: The aim of this study was to examine the long-term efficacy and safety of GPi-DBS in the treatment of primary dystonia (PD) or nonprimary dystonia (N-PD) in children and adults. Methods: We studied a series of sixty-four consecutive patients (28 F/36 M) with severe focal, segmental or generalized dystonia who received a stereotactical bilateral GPi implant for DBS in the period 1998 2009 at “C. Besta” Institute. Among them, 41 patients had a diagnosis of PD and 23 of N-PD. We compared motor outcome at last available FU with their status preoperatively and after 1 year of treatment. Dystonia was assessed with the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). Adverse events and sides effects were recorded. Results: Mean age at implant was 29.4±17.2 years; mean age at disease onset was 16.5±12.8 years. Follow-up (FU) duration was 3.8±2.5 years. At last FU, in the PD group, the mean BFMDRS movement score improved from 53.3±28.3 before surgery to 31.7±28.6 post-operatively (p = 0.007; mean improvement 40.5%); in the N-PD group, the mean BFMDRS movement score improved from 55.0±26.8 before surgery to 34.8±27.2 post-operatively (p = 0.008; mean improvement 36.7%). In the entire series, motor improvement observed at 1 year (42%) was maintained at last FU (39%), but individual slight decay were noted especially in N-PD group. The safety profile was positive. Conclusions: Bilateral GPi-DBS provides sustained motor benefit in the long-term observation, up to about seven years. Some clinical decline and new dystonic symptoms may occur in the long term FU in a subgroup of patients. In general, the outcomes for primary dystonias is more favourable compared to the non-primary or secondary forms. More studies are required to establish the factors predicting the long-term outcome.