Chronic stimulation of the globus pallidus internus for treatment of non-DYT1 generalized dystonia and choreoathetosis: 2-year follow up

2003 ◽  
Vol 98 (4) ◽  
pp. 785-792 ◽  
Author(s):  
Joachim K. Krauss ◽  
Thomas J. Loher ◽  
Ralf Weigel ◽  
H. Holger Capelle ◽  
Sabine Weber ◽  
...  

Object. The authors studied the long-term efficacy of deep brain stimulation (DBS) of the posteroventral lateral globus pallidus internus up to 2 years postoperatively in patients with primary non-DYT1 generalized dystonia or choreoathetosis. The results are briefly compared with those reported for DBS in DYT1 dystonia (Oppenheim dystonia), which is caused by the DYT1 gene. Methods. Enrollment in this prospective expanded pilot study was limited to adult patients with severely disabling, medically refractory non-DYT1 generalized dystonia or choreoathetosis. Six consecutive patients underwent follow-up examinations at defined intervals of 3 months, 1 year, and 2 years postsurgery. There were five women and one man, and their mean age at surgery was 45.5 years. Formal assessments included both the Burke-Fahn-Marsden dystonia scale and the recently developed Unified Dystonia Rating Scale. Two patients had primary generalized non-DYT1 dystonia, and four suffered from choreoathetosis secondary to infantile cerebral palsy. Bilateral quadripolar DBS electrodes were implanted in all instances, except in one patient with markedly asymmetrical symptoms. There were no adverse events related to surgery. The Burke-Fahn-Marsden scores in the two patients with generalized dystonia improved by 78 and 71% at 3 months, by 82 and 69% at 1 year, and by 78 and 70% at 2 years postoperatively. This was paralleled by marked amelioration of disability scores. The mean improvement in Burke-Fahn-Marsden scores in patients with choreoathetosis was 12% at 3 months, 29% at 1 year, and 23% at 2 years postoperatively, which was not significant. Two of these patients thought that they had achieved marked improvement at 2 years postoperatively, although results of objective evaluations were less impressive. In these two patients there was a minor but stable improvement in disability scores. All patients had an improvement in pain scores at the 2-year follow-up review. Medication was tapered off in both patients with generalized dystonia and reduced in two of the patients with choreoathetosis. All stimulation-induced side effects were reversible on adjustment of the DBS settings. Energy consumption of the batteries was considerably higher than in patients with Parkinson disease. Conclusions. Chronic pallidal DBS is a safe and effective procedure in generalized non-DYT1 dystonia, and it may become the procedure of choice in patients with medically refractory dystonia. Postoperative improvement of choreoathetosis is more modest and varied, and subjective ratings of outcome may exceed objective evaluations.

2004 ◽  
Vol 101 (2) ◽  
pp. 189-194 ◽  
Author(s):  
Philippe Coubes ◽  
Laura Cif ◽  
Hassan El Fertit ◽  
Simone Hemm ◽  
Nathalie Vayssiere ◽  
...  

Object. Primary generalized dystonia (PGD) is a medically refractory disease of the brain causing twisting or spasmodic movements and abnormal postures. In more than 30% of cases it is associated with the autosomal DYT1 mutation. Continuous electrical stimulation of the globus pallidus internus (GPi) has been used successfully in the treatment of PGD. The aim of this study was to examine the long-term efficacy and safety of deep brain stimulation (DBS) in the treatment of PGD in children and adults with and without the DYT1 mutation. Methods. Thirty-one patients with PGD were selected for surgery. Electrodes were bilaterally implanted under stereotactic guidance and connected to neurostimulators that were inserted subcutaneously. Efficacy was evaluated by comparing scores on the clinical and functional Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) before and after implantation. The efficacy of stimulation improved with time. After 2 years, compared with preoperative values, the mean (± standard deviation) clinical and functional BFMDRS scores had improved by 79 ± 19% and 65 ± 33%, respectively. At the 2-year follow-up examination the improvement was comparable in patients with and without the DYT1 mutation in both the functional (p = 0.12) and clinical (p = 0.33) scores. Children displayed greater improvements in the clinical score than adult patients (p = 0.04) at 2 years of follow up. In contrast, there was no significant difference in functional scores between children and adults (p = 0.95). Conclusions. Electrical stimulation of the GPi is an effective, reversible, and adaptable treatment for PGD and should be considered for conditions refractory to pharmaceutical therapies.


2004 ◽  
Vol 101 (2) ◽  
pp. 195-200 ◽  
Author(s):  
Antonella Peppe ◽  
Mariangela Pierantozzi ◽  
Andrea Bassi ◽  
Maria Grazia Altibrandi ◽  
Livia Brusa ◽  
...  

Object. The authors compared the effects of deep brain stimulation (DBS) in the globus pallidus internus (GPi) with those in the subthalamic nucleus (STN) in patients with Parkinson disease (PD) in whom electrodes had been bilaterally implanted in both targets. Methods. Eight of 14 patients with advanced PD in whom electrodes had been implanted bilaterally in both the GPi and STN for DBS were selected on the basis of optimal DBS effects and were studied 2 months postsurgery in offand on-stimulus conditions and after at least 1 month of pharmacological withdrawal. Subcutaneous administration of an apomorphine test dose (0.04 mg/kg) was also performed in both conditions. Compared with the off status, the results showed less reduction in the Unified PD Rating Scale Section III scores during DBS in the GPi (43.1%) than during DBS of the STN (54.5%) or DBS of both the STN and GPi (57.1%). The difference between the effects of DBS in the GPi compared with that in the STN or simultaneous DBS was statistically significant (p < 0.01). In contrast, no statistical difference was found between DBS in the STN and simultaneous DBS in the STN and GPi (p < 0.9). The improvement induced by adding apomorphine administration to DBS was similar in all three stimulus modalities. The abnormal involuntary movements (AIMs) induced by apomorphine were almost abolished by DBS of the GPi, but were not affected by stimulation of the STN. The simultaneous stimulation of STN and GPi produced both antiparkinsonian and anti-AIM effects. Conclusions. The improvement of parkinsonian symptoms during stimulation of the GPi, STN, and both nuclei simultaneously may indicate a similar DBS mechanism for both nuclei in inducing antiparkinsonian effects, although STN is more effective. The antidyskinetic effects produced only by DBS of the GPi, with or without STN, may indicate different mechanisms for the antidyskinetic and antiparkinsonian activity related to DBS of the GPi or an additional mechanism in the GPi. These findings indicate that implantation of double electrodes for DBS should not be proposed as a routine procedure, but could be considered as a possible subsequent choice if electrode implantation for DBS of the STN does not control AIMs.


2004 ◽  
Vol 100 (6) ◽  
pp. 997-1001 ◽  
Author(s):  
Mitsuhiro Ogura ◽  
Naoyuki Nakao ◽  
Ekini Nakai ◽  
Yuji Uematsu ◽  
Toru Itakura

Object. Although chronic electrical stimulation of the globus pallidus (GP) has been shown to ameliorate motor disabilities in Parkinson disease (PD), the underlying mechanism remains to be clarified. In this study the authors explored the mechanism for the effects of deep brain stimulation of the GP by investigating the changes in neurotransmitter levels in the cerebrospinal fluid (CSF) during the stimulation. Methods. Thirty patients received chronic electrical stimulation of the GP internus (GPi). Clinical effects were assessed using the Unified PD Rating Scale (UPDRS) and the Hoehn and Yahr Staging Scale at 1 week before surgery and at 6 and 12 months after surgery. One day after surgery, CSF samples were collected through a ventricular tube before and 1 hour after GPi stimulation. The concentration of neurotransmitters such as γ-aminobutyric acid (GABA), noradrenaline, dopamine, and homovanillic acid (HVA) in the CSF was measured using high-performance liquid chromatography. The treatment was effective for tremors, rigidity, and drug-induced dyskinesia. The concentration of GABA in the CSF increased significantly during stimulation, although there were no significant changes in the level of noradrenaline, dopamine, and HVA. A comparison between an increased rate of GABA concentration and a lower UPDRS score 6 months postimplantation revealed that the increase in the GABA level correlated with the stimulation-induced clinical effects. Conclusions. Stimulation of the GPi substantially benefits patients with PD. The underlying mechanism of the treatment may involve activation of GABAergic afferents in the GP.


1994 ◽  
Vol 80 (5) ◽  
pp. 810-819 ◽  
Author(s):  
Joachim K. Krauss ◽  
Mohsen Mohadjer ◽  
Fritz Nobbe ◽  
Fritz Mundinger

✓ The authors report the long-term results of stereotactic surgery for severe posttraumatic appendicular tremor in 35 patients. The tremors developed after severe head trauma in 33 patients (94%) and after mild to moderate head trauma in two (6%). In all but one, the tremor was most evident during activity. The amplitude of the kinetic tremor was greater than 5 cm in 33 patients (94%) and greater than 12 cm in 19 patients (54%). All were severely incapacitated in their daily living activities due to the tremors. The 35 patients underwent 42 stereotactic operations; five patients were reoperated on the same side and two were treated with a bilateral staged procedure. The contralateral zona incerta was the stereotactic target in 12 patients and was targeted in combination with the base of the ventrolateral (oroventral) thalamus in 23 patients. Long-term postoperative follow-up review was obtained in 32 patients (mean follow-up period 10.5 years). Persistent improvement of tremor was noted in 88%. The tremor was absent or markedly reduced in 65%. Functional disability was assessed and quantified with a modified form of an established rating scale for patients with tremor; it was reduced from a mean value of 57% of maximum disability to 37% over the long term (p < 0.001). Follow-up lesion assessment was obtained in 18 patients by multiplanar magnetic resonance imaging and at autopsy in one patient whose death was unrelated to surgery. As in previous studies, the frequency of persistent side effects was relatively high (38%). These consisted mainly of aggravation of preoperative symptoms. The results are compared to those of a total of 55 patients reported from 1960 to 1992. The occurrence of dystonia and dystonic postures is discussed. Stereotactic surgery is a powerful tool to alleviate posttraumatic tremor and to improve functional disability. However, as there is considerable risk of persistent morbidity in patients after severe head trauma, the operation should be restricted to selected cases with disabling tremor.


2012 ◽  
Vol 116 (5) ◽  
pp. 1144-1152 ◽  
Author(s):  
Laura Cif ◽  
Victoria Gonzalez-Martinez ◽  
Xavier Vasques ◽  
Astrid Corlobé ◽  
Ana Maria Moura ◽  
...  

Object Deep brain stimulation (DBS) is used for treating various types of dystonia. Multiple electrodes could be proposed to improve the therapeutic outcome enabling the targeting of specific neuronal populations not reached by the electrical field generated by the initially implanted electrode. The authors address the question of the feasibility and safety of staged multiple lead implantations in the sensorimotor internal globus pallidus (GPi) in primary generalized dystonia (PGD). Criteria for patient selection, surgical technique, target selection, electrical settings management, and clinical outcome are presented. Methods Sixteen patients (8 harbored the DYT1 gene mutation) presented with PGD and were enrolled in this study. Patients underwent clinical assessment using the Burke-Fahn-Marsden Dystonia Rating Scale preoperatively and during follow-up with DBS. Prior to the addition of electrodes, the authors confirmed, by turning off stimulation, that the patient was still benefiting from DBS and that DBS settings adjustment did not provide further improvement. The second target was defined according to the position of the first electrode, to the residual volume within the sensorimotor GPi, and according to residual symptoms. The second surgery followed the same protocol as the first and the new electrode were inserted using the same bur hole as the first electrode. Results The addition of a new pair of electrodes was followed by significant improvement in the whole population (p = 0.005), as well as in the DYT1-negative subgroup (p = 0.012) but not in the DYT1 subgroup (p = not significant). Nevertheless, some patients did not exhibit significant additional benefit. Seven hardware-related complications occurred during the entire follow-up, 3 prior to it, and 4 after the addition of the second pair of electrodes. Conclusions The addition of a second pair of electrodes in the GPi in patients with PGD with suboptimal or decaying benefit following the first surgery seems to be a safe procedure and is not followed by an increase in surgery-related complications. This staged procedure may provide further clinical improvement in patients with PGD in whom DBS effect is initially incomplete or when disease progression occurs over time. The position of the additional electrode within the GPi is determined by the available volume within the posteroventral GPi and by the distribution of the dystonic symptoms that need to be controlled.


2008 ◽  
Vol 62 (suppl_1) ◽  
pp. ONS217-ONS225 ◽  
Author(s):  
Clement Hamani ◽  
Elena Moro ◽  
Cindy Zadikoff ◽  
Yu-Yan Poon ◽  
Andres M. Lozano

Abstract Objective: Deep brain stimulation of the globus pallidus internus has been used for the treatment of various forms of dystonia, but the factors influencing postoperative outcomes remain unknown. We compared the location of the contacts being used for stimulation (active contacts) in patients with cervical dystonia, generalized dystonia, and Parkinson's disease and correlated the results with clinical outcome. Methods: Postoperative magnetic resonance scans of 13 patients with cervical dystonia, six patients with generalized dystonia, and five patients with Parkinson's disease who underwent globus pallidus internus deep brain stimulation were analyzed. We assessed the location of the active contacts relative to the midcommisural point and in relation to the anteroposterior and mediolateral boundaries of the pallidum. Postoperative outcome was measured with the Toronto Western Spasmodic Torticollis Rating Scale (for cervical dystonia) and the Burke-Fahn-Marsden Dystonia Rating Scale (for generalized dystonia) during the last follow-up. Results: We found that the location of the active contacts relative to the midcom-misural point and the internal boundaries of the pallidum was similar across the groups. In our series, the contacts used for stimulation were clustered in the posterolateral region of the pallidum. Within that region, we found no correlation between the location of the contacts and postoperative outcome. Conclusion: The location of the active contacts used for globus pallidus internus deep brain stimulation was similar in patients with cervical dystonia, generalized dystonia, and Parkinson's disease.


2000 ◽  
Vol 93 (supplement_3) ◽  
pp. 128-135 ◽  
Author(s):  
Ronald F. Young ◽  
Skip Jacques ◽  
Rufus Mark ◽  
Oleg Kopyov ◽  
Brian Copcutt ◽  
...  

Object. The purpose of this study was to investigate the long-term effects of gamma knife thalamotomy for treatment of disabling tremor. Methods. One hundred fifty-eight patients underwent magnetic resonance imaging—guided radiosurgical nucleus ventralis intermedius (VIM) thalamotomy for the treatment of parkinsonian tremor (102 patients), essential tremor (52 patients), or tremor due to stroke, encephalitis, or cerebral trauma (four patients). Preoperative and postoperative blinded assessments were performed by a team of independent examiners skilled in the evolution of movement disorders. A single isocenter exposure with the 4-mm collimator helmet of the Leksell gamma knife unit was used to make the lesions. In patients with Parkinson's disease 88.3% became fully or nearly tremor free, with a mean follow up of 52.5 months. Statistically significant improvements were seen in Unified Parkinson's Disease Rating Scale tremor scores and rigidity scores, and these improvements were maintained in 74 patients followed 4 years or longer. In patients with essential tremor, 92.1% were fully or nearly tremor free postoperatively, but only 88.2% remained tremor free by 4 years or more post-GKS. Statistically significant improvements were seen in the Clinical Rating Scale for tremor in essential tremor patients and these improvements were well maintained in the 17 patients, followed 4 years or longer. Only 50% of patients with tremor of other origins improved significantly. One patient sustained a transient complication and two patients sustained mild permanent side effects from the treatments. Conclusions. Gamma knife VIM thalamotomy provides relief from tremor equivalent to that provided by radiofrequency thalamotomy or deep brain stimulation, but it is safer than either of these alternatives. Long-term follow up indicates that relief of tremor is well maintained. No long-term radiation-induced complications have been observed.


2002 ◽  
Vol 97 (3) ◽  
pp. 598-606 ◽  
Author(s):  
Philip C. Su ◽  
Ham-Min Tseng ◽  
Hon-Man Liu ◽  
Ruoh-Fang Yen ◽  
Horng-Huei Liou

Object. The aim of this study was to determine if subthalamotomy is effective in treating advanced Parkinson disease (PD). Methods. The authors performed microelectrode mapping—guided stereotactic surgery on the subthalamic nucleus in eight patients with PD. Lesioning was performed using radiofrequency heat coagulation and confirmed with magnetic resonance imaging. Three patients who underwent unilateral and four with bilateral subthalamotomy were evaluated for up to 18 months according to the Unified PD Rating Scale (UPDRS). One patient who underwent unilateral subthalamotomy died 6 months postsurgery. At 3 months into the “off” period after surgery, there were significant improvements in contralateral bradykinesia (p < 0.0002), rigidity (p < 0.0001), tremor (p < 0.01), axial motor features (p < 0.02), gait (p < 0.03), postural stability (p < 0.03), total UPDRS scores (p < 0.03), and Schwab and England scores (p < 0.04). The benefits were sustained at 6, 12, and 18 months, except for the improvement in tremor. At 12 months into the “on” period, significant benefits were present for motor fluctuation (p < 0.04), on dyskinesia (p < 0.006), off duration (p < 0.05), total UPDRS score (p < 0.02), and contralateral tremor (p < 0.05). Benefits for motor fluctuation, off duration, and off-period tremor were lost after the 18-month follow-up period. The levodopa requirement was reduced by 66% for the unilateral and 38% for the bilaterally treated group. Bilateral subthalamotomy offered more benefits than did unilateral surgery for various parkinsonian features in both the on and off periods. Three patients suffered hemiballismus, two recovered spontaneously, and one died of aspiration pneumonia after discontinuation of levodopa. Conclusions. These findings indicate that subthalamotomy can ameliorate the cardinal symptoms of PD, reduce the dosage of levodopa, diminish complications of the drug therapy, and improve the quality of life.


2003 ◽  
Vol 98 (2) ◽  
pp. 414-416 ◽  
Author(s):  
Takaomi Taira ◽  
Tomonori Kobayashi ◽  
Tomokatsu Hori

✓ Lesch—Nyhan syndrome (LNS) is an X-linked hereditary disorder caused by a deficiency of hypoxanthine-guanine phosphoribosyltransferase. Patients with this syndrome are characterized by hyperuricemia, self-mutilation, developmental retardation, and movement disorders such as spasticity and dystonia. The authors performed bilateral chronic stimulation of the globus pallidus internus for control of dystonic movements in a 19-year-old man with LNS. His self-mutilating behavior unexpectedly disappeared after chronic stimulation. This is the first case of LNS that has been successfully treated with deep brain stimulation. The findings indicate that neurobehavioral features of this syndrome are either mediated in the basal ganglia pathways or secondary to the dystonia.


2003 ◽  
Vol 99 (1) ◽  
pp. 71-77 ◽  
Author(s):  
Rajesh Pahwa ◽  
Steven B. Wilkinson ◽  
John Overman ◽  
Kelly E. Lyons

Object. Bilateral subthalamic nucleus (STN) stimulation is increasingly used in patients with advanced Parkinson disease (PD). This study was performed to evaluate the long-term efficacy and safety of bilateral STN stimulation in cases of PD. Methods. The authors performed a prospective, open-label study in patients with PD who underwent bilateral STN stimulation. The authors compared motor scores and activities of daily living (ADL) scores based on the Unified PD Rating Scale (UPDRS) obtained before surgery while patients were in the medication-off state with scores obtained at follow-up evaluations of these patients while in the medication-off/stimulator-on state. Data contained in patient diaries were also compared. Thirty-three patients with PD were evaluated 12 months postoperatively and 19 were evaluated at a mean follow-up time of 28 months. A comparison between UPDRS scores obtained in patients in the medication-off/stimulator-on state and those obtained when patients were in the baseline medication-off state showed a 27% improvement in ADL scores and a 28% improvement in motor scores after surgery. There was a 57% reduction in the use of levodopa-equivalent medication doses. The percentage of the waking day that patients were in the medication-on state increased from 38 to 72%. Surgical complications included seizures (three patients), confusion (five patients), hemiballismus (one patient), and visual disturbance (one patient). Stimulation-related adverse effects were mild. Device-related events included nine lead replacements, seven lead revisions, six extension replacements, and 12 implantable pulse generator (IPG) replacements; one IPG was cleaned and one IPG was placed in a pocket because of the presence of a shunt. Conclusions. Bilateral STN simulation is associated with a significant improvement in the motor features of PD. Device-related events were common in the first 20 patients who underwent surgery, often requiring repeated surgeries.


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