Clinical phenotypes associated with outcomes following deep brain stimulation for childhood dystonia

2019 ◽  
Vol 24 (4) ◽  
pp. 442-450 ◽  
Author(s):  
Jetan H. Badhiwala ◽  
Brij Karmur ◽  
Lior M. Elkaim ◽  
Naif M. Alotaibi ◽  
Benjamin R. Morgan ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Latent Variables ◽  
Rating Scale ◽  
Significant Heterogeneity ◽  
Secondary Dystonia ◽  
Primary Dystonia ◽  
Relative Change ◽  
Deep Brain

OBJECTIVEAlthough deep brain stimulation (DBS) is an accepted treatment for childhood dystonia, there is significant heterogeneity in treatment response and few data are available to identify ideal surgical candidates.METHODSData were derived from a systematic review and individual patient data meta-analysis of DBS for dystonia in children that was previously published. Outcomes were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale for movement (BFMDRS-M) and for disability (BFMDRS-D). The authors used partial least squares, bootstrapping, and permutation statistics to extract patterns of contributions of specific preoperative characteristics to relationship with distinct outcomes, in all patients and in patients with primary and secondary dystonia separately.RESULTSOf 301 children undergoing DBS for dystonia, 167 had primary dystonia, 125 secondary dystonia, and 9 myoclonus dystonia. Three dissociable preoperative phenotypes (latent variables) were identified and associated with the following: 1) BFMDRS-M at last follow-up; 2) relative change in BFMDRS-M score; and 3) relative change in BFMDRS-D score. The phenotype of patients with secondary dystonia, with a high BFMDRS-M score and truncal involvement, undergoing DBS at a younger age, was associated with a worse postoperative BFMDRS-M score. Children with primary dystonia involving the trunk had greater improvement in BFMDRS-M and -D scores. Those with primary dystonia of shorter duration and proportion of life with disease, undergoing globus pallidus DBS, had greater improvements in BFMDRS-D scores at long-term follow-up.CONCLUSIONSIn a comprehensive, data-driven, multivariate analysis of DBS for childhood dystonia, the authors identified novel and dissociable patient phenotypes associated with distinct outcomes. The findings of this report may inform surgical candidacy for DBS.

Primary Dystonia Is More Responsive than Secondary Dystonia to Pallidal Interventions: Outcome after Pallidotomy or Pallidal Deep Brain Stimulation

Neurosurgery ◽  
2004 ◽  
Vol 54 (3) ◽  
pp. 613-621 ◽  
Author(s):  
Hazem A. Eltahawy ◽  
Jean Saint-Cyr ◽  
Nir Giladi ◽  
Anthony E. Lang ◽  
Andres M. Lozano
Keyword(s):  
Magnetic Resonance Imaging ◽  
Deep Brain Stimulation ◽  
Magnetic Resonance ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Resonance Imaging ◽  
Secondary Dystonia ◽  
Primary Dystonia ◽  
Pallidal Deep Brain Stimulation ◽  
Deep Brain

Abstract OBJECTIVE The response of patients with dystonia to pallidal procedures is not well understood. In this study, we assessed the postoperative outcome of patients with primary and secondary dystonia undergoing pallidotomy or pallidal deep brain stimulation. METHODS Fifteen patients with dystonia had pallidal surgery (lesions or deep brain stimulation). These included nine patients with primary dystonia (generalized and cervical dystonias) and six with secondary dystonia (generalized, segmental, and hemidystonias). There were nine male patients and six female patients. The mean age at onset was 21 years for primary dystonia and 18 years for secondary dystonia. The primary outcome measure was a Global Outcome Scale score for dystonia at 6 months after surgery. Other outcome measures were the Burke-Fahn-Marsden Dystonia Rating Scale and Toronto Western Spasmodic Torticollis Rating Scale scores. RESULTS The mean Global Outcome Scale score at 6 months for patients with primary dystonia was 3 (improvement in both movement disorder and function). In contrast, patients with secondary dystonia had a mean score of 0.83 (mild or no improvement in movement disorder with no functional improvement). All patients with primary dystonia had normal brains by magnetic resonance imaging, whereas five of six patients with secondary dystonia had basal ganglia abnormalities on their magnetic resonance imaging scans. CONCLUSION This study indicates that primary dystonia responds much better than secondary dystonia to pallidal procedures. We could not distinguish a difference in efficacy between pallidotomy and pallidal deep brain stimulation. The presence of basal ganglia abnormalities on the preoperative magnetic resonance imaging scan is an indicator of a lesser response to pallidal interventions for dystonia.

Deep brain stimulation in children: experience and technical pearls

2011 ◽  
Vol 8 (6) ◽  
pp. 566-574 ◽  
Author(s):  
Ellen L. Air ◽  
Jill L. Ostrem ◽  
Terence D. Sanger ◽  
Philip A. Starr
Keyword(s):  
Deep Brain Stimulation ◽  
Surgical Technique ◽  
Brain Stimulation ◽  
Rating Scales ◽  
Rating Scale ◽  
Glutaric Aciduria Type 1 ◽  
Secondary Dystonia ◽  
Guided Surgery ◽  
Primary Dystonia ◽  
Deep Brain

Object Deep brain stimulation (DBS) is an established technique for the treatment of several movement disorders in adults. However, the technical approach, complications, and results of DBS in children have not been well documented. Methods A database of DBS implantations performed at a single institution, prospectively established in 1998, was reviewed for patients who received DBS prior to the age of 18. Diagnoses, surgical technique, and complications were noted. Outcomes were assessed using standard rating scales of neurological function. Results Of 815 patients undergoing DBS implantation over a 12-year period, 31 were children (mean age at surgery 13.2 years old, range 4–17 years old). Diagnoses included the following: DYT1 primary dystonia (autosomal dominant, Tor1AΔGAG mutation, 10 cases), non-DYT1 primary dystonia (3 cases), secondary dystonia (11 cases), neurodegeneration with brain iron accumulation (NBIA, 3 cases), levodopa-responsive parkinsonism (2 cases), Lesch-Nyhan disease (1 case), and glutaric aciduria Type 1 (1 case). Six children ages 15–17 years old underwent awake microelectrode-guided surgery. For 25 children operated under general anesthesia, the surgical technique evolved from microelectrode-guided surgery to image-guided surgeries using real-time intraoperative MR imaging or CT for lead location confirmation. Complications included 5 hardware infections, all in children younger than 10 years old. At 1 year after implantation, patients with DYT1 dystonia had a mean improvement in the Burke-Fahn-Marsden Dystonia Rating Scale movement subscore of 75%, while those with secondary dystonia had only small improvements. Outcomes in the 3 children with NBIA were disappointing. Conclusions Results of DBS in children with primary and secondary dystonias were similar to those in adults, with excellent results for DYT1 dystonia in children without fixed orthopedic deformity and much more modest results in secondary dystonia. In contrast to reported experience in adults with NBIA, these results in children with NBIA were poor. Infection risk was highest in the youngest patients.

Pallidal Deep Brain Stimulation for Primary Dystonia in Children

Neurosurgery ◽  
2011 ◽  
Vol 68 (3) ◽  
pp. 738-743 ◽  
Author(s):  
Abilash Haridas ◽  
Michele Tagliati ◽  
Irene Osborn ◽  
Ioannis Isaias ◽  
Yakov Gologorsky ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Clinical Improvement ◽  
Brain Stimulation ◽  
Functional Disability ◽  
Rating Scale ◽  
Retrospective Chart Review ◽  
Consecutive Series ◽  
Internal Globus Pallidus ◽  
Deep Brain

Abstract BACKGROUND: Deep brain stimulation (DBS) at the internal globus pallidus (GPi) has replaced ablative procedures for the treatment of primary generalized dystonia (PGD) because it is adjustable, reversible, and yields robust clinical improvement that appears to be long lasting. OBJECTIVE: To describe the long-term responses to pallidal DBS of a consecutive series of 22 pediatric patients with PGD. METHODS: Retrospective chart review of 22 consecutive PGD patients, ≤21 years of age treated by one DBS team over an 8-year period. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to evaluate symptom severity and functional disability, pre- and post-operatively. Adverse events and medication changes were also noted. RESULTS: The median follow-up was 2 years (range, 1-8 years). All 22 patients reached 1-year follow-up; 14 reached 2 years, and 11 reached 3 years. The BFMDRS motor subscores were improved 84%, 93%, and 94% (median) at these time points. These motor responses were matched by equivalent improvements in function, and the response to DBS resulted in significant reductions in oral and intrathecal medication requirements after 12 and 24 months of stimulation. There were no hemorrhages or neurological complications related to surgery and no adverse effects from stimulation. Significant hardware-related complications were noted, in particular, infection (14%), which delayed clinical improvement. CONCLUSION: Pallidal DBS is a safe and effective treatment for PGD in patients <21 years of age. The improvement appears durable. Improvement in device design should reduce hardware-related complications over time.

Bilateral Deep Brain Stimulation for Cervical Dystonia: Long-term Outcome in a Series of 10 Patients

Neurosurgery ◽  
2010 ◽  
Vol 67 (4) ◽  
pp. 957-963 ◽  
Author(s):  
Francesco Cacciola ◽  
Jibril Osman Farah ◽  
Paul R Eldridge ◽  
Patricia Byrne ◽  
Telekath K Varma
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Cervical Dystonia ◽  
Rating Scale ◽  
Spasmodic Torticollis ◽  
Long Term Outcome ◽  
Stimulation Parameters ◽  
Deep Brain

Abstract BACKGROUND: Bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) was shown to be effective in cervical dystonia refractory to medical treatment in several small short-term and 1 long-term follow-up series. Optimal stimulation parameters and their repercussions on the cost/benefit ratio still need to be established. OBJECTIVE: To report our long-term outcome with bilateral GPi deep brain stimulation in cervical dystonia. METHODS: The Toronto Western Spasmodic Torticollis Rating Scale was evaluated in 10 consecutive patients preoperatively and at last follow-up. The relationship of improvement in postural severity and pain was analyzed and stimulation parameters noted and compared with those in a similar series in the literature. RESULTS: The mean (standard deviation) follow-up was 37.6 (16.9) months. Improvement in the total Toronto Western Spasmodic Torticollis Rating Scale score as evaluated at latest follow-up was 68.1% (95% confidence interval: 51.5-84.6). In 4 patients, there was dissociation between posture severity and pain improvement. Prevalently bipolar stimulation settings and high pulse widths and amplitudes led to excellent results at the expense of battery life. CONCLUSION: Improvement in all 3 subscale scores of the Toronto Western Spasmodic Torticollis Rating Scale with bilateral GPi deep brain stimulation seems to be the rule. Refinement of stimulation parameters might have a significant impact on the cost/benefit ratio of the treatment. The dissociation of improvement in posture severity and pain provides tangible evidence of the complex nature of cervical dystonia and offers interesting insight into the complex functional organization of the GPi.

scholarly journals Unilateral thalamic and pallidal deep brain stimulation for idiopathic hemidystonia: results of individual and combined stimulations. Case report

2017 ◽  
Vol 43 (1) ◽  
pp. E2 ◽  
Author(s):  
Victor Goulenko ◽  
Paulo Luiz da Costa Cruz ◽  
Paulo Niemeyer Filho
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Multiple Target ◽  
Motor Pathways ◽  
Severity Rating ◽  
Pallidal Stimulation ◽  
Pallidal Deep Brain Stimulation ◽  
Deep Brain

Pallidal stimulation has been the usual surgical treatment for dystonia in the last decades. The continuous investigation of the physiopathology and the motor pathways involved leads to the search for complementary targets to improve results. The authors present the case of a 37-year-old woman who had suffered from idiopathic hemidystonia with hyperkinetic and hypokinetic movements for 11 years, and who was treated with deep brain stimulation. A brief literature review is also provided. The globus pallidus internus and the ventral intermediate/ventral oral posterior complex of the thalamus were stimulated separately and simultaneously for 3 months and compared using the Burke-Fahn-Marsden Dystonia Rating Scale and the Global Dystonia Severity Rating Scale, with a 3.5-year follow-up. The synergism of multiple-target stimulation resulted in a complete improvement of the mixed dystonic symptoms.

scholarly journals Longitudinal follow-up with VIM thalamic deep brain stimulation for dystonic or essential tremor

Neurology ◽  
2020 ◽  
Vol 94 (10) ◽  
pp. e1073-e1084 ◽  
Author(s):  
Takashi Tsuboi ◽  
Zakia Jabarkheel ◽  
Pamela R. Zeilman ◽  
Matthew J. Barabas ◽  
Kelly D. Foote ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Essential Tremor ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Statistical Significance ◽  
Ventral Intermediate Nucleus ◽  
Deep Brain

ObjectiveTo assess longitudinal tremor outcomes with ventral intermediate nucleus deep brain stimulation (VIM DBS) in patients with dystonic tremor (DT) and to compare with DBS outcomes in essential tremor (ET).MethodsWe retrospectively investigated VIM DBS outcomes for 163 patients followed at our center diagnosed with either DT or ET. The Fahn-Tolosa-Marin tremor rating scale (TRS) was used to assess change in tremor and activities of daily living (ADL) at 6 months, 1 year, 2–3 years, 4–5 years, and ≥6 years after surgery.ResultsTwenty-six patients with DT and 97 patients with ET were analyzed. Compared to preoperative baseline, there were significant improvements in TRS motor up to 4–5 years (52.2%; p = 0.032) but this did not reach statistical significance at ≥6 years (46.0%, p = 0.063) in DT, which was comparable to the outcomes in ET. While the improvements in the upper extremity tremor, head tremor, and axial tremor were also comparable between DT and ET throughout the follow-up, the ADL improvements in DT were lost at 2–3 years follow-up.ConclusionOverall, tremor control with VIM DBS in DT and ET was comparable and remained sustained at long term likely related to intervention at the final common node in the pathologic tremor network. However, the long-term ADL improvements in DT were not sustained, possibly due to inadequate control of concomitant dystonia symptoms. These findings from a large cohort of DT indicate that VIM targeting is reasonable if the tremor is considerably more disabling than the dystonic features.Classification of evidenceThis study provides Class IV evidence that VIM DBS improves tremor in patients with DT or ET.

The Benefit of Subthalamic Deep Brain Stimulation for Pain in Parkinson Disease: A 2-Year Follow-up Study

Neurosurgery ◽  
2011 ◽  
Vol 70 (1) ◽  
pp. 18-24 ◽  
Author(s):  
Han-Joon Kim ◽  
Beom S. Jeon ◽  
Jee-Young Lee ◽  
Sun Ha Paek ◽  
Dong Gyu Kim
Keyword(s):  
Deep Brain Stimulation ◽  
Beneficial Effect ◽  
Parkinson Disease ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Motor Disorder ◽  
Motor Symptoms ◽  
Stn Dbs ◽  
Deep Brain

Abstract BACKGROUND Pain is a well-recognized feature of Parkinson disease (PD), which is primarily a motor disorder. In a previous study, we showed that subthalamic deep brain stimulation (STN DBS) improves pain as well as motor symptoms 3 months after surgery in PD patients. OBJECTIVE To determine whether there is a long-term beneficial effect of STN DBS on pain in PD. METHODS We studied 21 patients with PD who underwent STN DBS. Motor symptoms were assessed using the Unified Parkinson's Disease Rating Scale and Hoehn and Yahr staging. Pain was evaluated by asking patients about the quality and severity of pain in each body part. Evaluations were performed at baseline and at 3 and 24 months after surgery. RESULTS At baseline, 18 of the 21 patients (86%) experienced pain. After surgery, most of the pain reported at baseline had improved or disappeared at 3 months and improved further at 24 months. The benefit of STN DBS for pain evaluated at 24 months was comparable to that with medication at baseline. At 24 months, 9 patients (43%) reported new pain that was not present at baseline. Most of the new pain was musculoskeletal in quality. Despite the development of new pain, the mean pain score at follow-up was lower than at baseline. CONCLUSION STN DBS improves pain in PD, and this beneficial effect persists, being observed after a prolonged follow-up of 24 months. In addition, in many of the PD patients new, mainly musculoskeletal pain developed on longer follow-up.

BILATERAL SUBTHALAMIC DEEP BRAIN STIMULATION IN A PATIENT WITH PARKINSON'S DISEASE WHO HAD PREVIOUSLY UNDERGONE THALAMOTOMY AND AUTOLOGOUS ADRENAL GRAFTING IN THE CAUDATE NUCLEUS

Neurosurgery ◽  
2006 ◽  
Vol 59 (5) ◽  
pp. E1140-E1140 ◽  
Author(s):  
Francesco Vergani ◽  
Andrea Landi ◽  
Angelo Antonini ◽  
Erik P. Sganzerla
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Disease Rating ◽  
Noticeable Reduction ◽  
Surgical Treatments ◽  
Deep Brain

Abstract OBJECTIVE Subthalamic (Stn) deep brain stimulation (DBS) is a valid surgical therapy for the treatment of severe Parkinson's disease. In recent years, StnDBS has been proposed for patients who previously received other surgical treatments, such as thalamotomy and pallidotomy. Nonetheless, there is no consensus about the indications of DBS in patients who previously underwent surgery. To the best of our knowledge this is the first reported case of a patient treated with DBS after previous thalamotomy and adrenal grafting. CLINICAL PRESENTATION A 62-year-old man with a long history (more than 30 yr) of Parkinson's disease received unilateral thalamotomy and autologous adrenal graft on two independent occasions. Thalamotomy led to a significant improvement, although limited to the control of contralateral tremor. The autologous adrenal graft was of no benefit. For the subsequent occurrence of L-dopa related dyskinesias and severe “off” periods, the patient was referred to our center for StnDBS. INTERVENTION The patient underwent bilateral StnDBS, obtaining a satisfactory improvement of rigidity and bradykinesia on both sides. The 1-year follow-up evaluation showed a 46% improvement in the Unified Parkinson's Disease Rating Scale motor section, along with a noticeable reduction in antiparkinsonian therapy (81%). CONCLUSION This case is consistent with previous reports from the literature, suggesting that StnDBS is feasible and safe, even in patients who previously received other surgical treatments for Parkinson's disease, such as thalamotomy or cell grafting.

scholarly journals Deep brain stimulation in children and young adults with secondary dystonia: the Children's Hospital Los Angeles experience

2013 ◽  
Vol 35 (5) ◽  
pp. E7 ◽  
Author(s):  
Joffre E. Olaya ◽  
Eisha Christian ◽  
Diana Ferman ◽  
Quyen Luc ◽  
Mark D. Krieger ◽  
...  
Keyword(s):  
Young Adults ◽  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Secondary Dystonia ◽  
Benefit Evaluation ◽  
Generalized Dystonia ◽  
Children And Young Adults ◽  
The Mean ◽  
Deep Brain

Background Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. It can be classified as primary or secondary. There is no cure for dystonia and the goal of treatment is to provide a better quality of life for the patient. Surgical intervention is considered for patients in whom an adequate trial of medical treatment has failed. Deep brain stimulation (DBS), specifically of the globus pallidus interna (GPi), has been shown to be extremely effective in primary generalized dystonia. There is much less evidence for the use of DBS in patients with secondary dystonia. However, given the large number of patients with secondary dystonia, the significant burden on the patients and their families, and the potential for DBS to improve their functional status and comfort level, it is important to continue to investigate the use of DBS in the realm of secondary dystonia. Object The objective of this study is to review a series of cases involving patients with secondary dystonia who have been treated with pallidal DBS. Methods A retrospective review of 9 patients with secondary dystonia who received treatment with DBS between February 2011 and February 2013 was performed. Preoperative and postoperative videos were scored using the Barry-Albright Dystonia Scale (BADS) and Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) by a neurologist specializing in movement disorders. In addition, the patients' families completed a subjective questionnaire to assess the perceived benefit of DBS. Results The average age at DBS unit implantation was 15.1 years (range 6–20 years). The average time to follow-up for the BADS evaluation from battery implantation was 3.8 months (median 3 months). The average time to follow-up for the subjective benefit evaluation was 10.6 months (median 9.5 months). The mean BADS scores improved by 9% from 26.5 to 24 (p = 0.04), and the mean BFMDRS scores improved by 9.3% (p = 0.055). Of note, even in patients with minimal functional improvement, there seemed to be decreased contractures and spasms leading to improved comfort. There were no complications such as infections or hematoma in this case series. In the subjective benefit evaluation, 3 patients' families reported “good” benefit, 4 reported “minimal” benefit, and 1 reported no benefit. Conclusions These early results of GPi stimulation in a series of 9 patients suggest that DBS is useful in the treatment of secondary generalized dystonia in children and young adults. Objective improvements in BADS and BFMDRS scores are demonstrated in some patients with generalized secondary dystonia but not in others. Larger follow-up studies of DBS for secondary dystonia, focusing on patient age, history, etiology, and patterns of dystonia, are needed to learn which patients will respond best to DBS.

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