Surgery in octogenarians with intracranial meningiomas improves functional outcome at 1 year.

PURPOSE: The population is aging, and age remains an important factor in deciding surgical candidacy for intracranial tumors. The natural history and surgical behavior of meningiomas in octogenarians are not well understood. We evaluated the surgical and functional outcomes, including survival, among octogenarians with intracranial meningiomas in a single institution. METHODS: The Tumor Registry (2004-2021) was used to identify octogenarian patients (ages 80-89) diagnosed with intracranial meningioma. Primary endpoints were 1-year survival and functional outcome measured with mRS postsurgery. Kaplan-Meier, univariable Log-rank tests, and multivariable Cox hazards proportional regression models were used for assessing factors associated with overall survival (OS) in octogenarians with meningiomas who underwent surgery; logistic regression and McNemar's were used to further characterize risk factors affecting functional surgical outcome at 1 year. RESULTS: Thirty octogenarians with intracranial meningioma who underwent surgery were identified. Median age was 82.5 years and 66.6% were female patients. The 1-year median postsurgical survival probability for all octogenarians with meningioma was 86.3% and no intraoperative mortality was observed. Frailty (mFI-5, p = 0.84), tumor grade (p = 0.11), tumor size (p = 0.22), extent of resection (p = 0.35), and Karnofsky scale on admission (p = 0.93) did not significantly affect the survival in octogenarians with meningiomas which were treated surgically. The 1-year postoperative functional status of octogenarian meningioma patients who underwent surgery was significantly improved compared to pre-op mRS (McNemar's chi-squared = 9.6, df = 1, p-value = 0.001946). CONCLUSION: In octogenarians with meningiomas, surgical intervention significantly improves the pre-operative modified Rankin Scale at 1 year postsurgery in this cohort.

Sustained quality-of-life improvements over 10 years after deep brain stimulation for dystonia.

BACKGROUND: Little is known about the quality of life of people with dystonia and DBS beyond 5 years. The objectives of this study were (1) to examine the long-term quality-of-life outcomes in a large cohort of people with dystonia and DBS, (2) to determine the incidence of stimulation-induced parkinsonism, and (3) to elucidate the potential long-term cognitive impact of DBS in this cohort. METHODS: Fifty-four subjects with dystonia and DBS for more than 5 years were contacted via social media and were offered to complete a quality-of-life survey comparing current-day life and life prior to DBS. The primary study outcomes were the Short Form survey, a parkinsonian symptoms questionnaire, the Telephone Montreal Cognitive Assessment, and the Measurement of Every Day Cognition. RESULTS: Thirty-seven of 54 subjects consented to the study. Average age was 39.7 ± 16.6 years, 16 were female, and 23 were DYT1+. Average time from implantation was 10.5 years. Average total Short Form survey scores improved, from 43.7 pre-DBS to 69.5 current day (P < 0.0005). Mean total self-reported parkinsonian symptom score was 13.8 ± 14.7, with worsening balance and hypophonia the most common. Average Telephone Montreal Cognitive Assessment was 20.1 ± 1.6, with 3 of 29 scores (10.3%) in the impaired range (score of 18 or less). Average total Every Day Cognition score was 1.25 ± 0.35, with 3 subjects (10.3%) scoring in the range of impaired cognition (>1.81). CONCLUSIONS: DBS for dystonia results in long-term quality-of-life improvements that persist on average 10 years or more after surgery. The prevalence of stimulation-induced parkinsonism and cognitive impairment is low. © 2018 International Parkinson and Movement Disorder Society.

Deep Brain Stimulation for Status Dystonicus: A Case Series and Review of the Literature.

BACKGROUND: Status dystonicus (SD) is a rare and potentially life-threatening complication of primary or secondary dystonia, characterized by acute worsening of dystonic movements. There is no consensus regarding optimal treatment, which may be medical and/or surgical. METHODS: We present our experience with pallidal deep brain stimulation (DBS) in 5 DYT1-positive patients with SD and provide a review of the literature to examine optimal management. RESULTS: Of the 5 patients treated with pallidal DBS, all experienced postoperative resolution of their dystonic crisis within a range of 1-21 days. Long-term follow-up resulted in 1 patient returning to preoperative baseline, 3 patients improving from baseline, and 1 patient making a complete recovery. Of the 28 SD patients (including our 5 patients) reported in the literature who were treated with DBS or ablative surgery, 26 experienced cessation of their dystonic crisis with a return to baseline function and, in most cases, clinical improvement. CONCLUSION: DBS is an effective therapeutic modality for the treatment of SD. In addition to the long-term benefits of stimulation, early and aggressive treatment may improve the overall outcome.

Defining a therapeutic target for pallidal deep brain stimulation for dystonia.

OBJECTIVE: To create a data-driven computational model that identifies brain regions most frequently influenced by successful deep brain stimulation (DBS) of the globus pallidus (GP) for advanced, medication-resistant, generalized dystonia. METHODS: We studied a retrospective cohort of 21 DYT1 primary dystonia patients treated for at least 1 year with bilateral pallidal DBS. We first created individual volume of tissue activation (VTA) models utilizing neuroimaging and postoperative stimulation and clinical data. These models were then combined into a standardized probabilistic dystonia stimulation atlas (DSA). Finally, we constructed a candidate target volume from electrodes demonstrating at least 75% improvement in contralateral symptoms, utilizing voxels stimulated by least 75% of these electrodes. RESULTS: Pallidal DBS resulted in a median contralateral hemibody improvement of 90% (mean = 83%, standard deviation [SD] = 20) after 1 year of treatment. Individual VTA models of the 42 active electrodes included in the study demonstrated a mean stimulation volume of 501mm ([SD] = 284). The resulting DSA showed that areas most frequently stimulated were located squarely in the middle of the posterior GP, with a common target volume measuring 153mm(3) . INTERPRETATION: Our results provide a map of the region of influence of therapeutic DBS for dystonia and represent a potential target to refine current methods of surgical planning and stimulation parameters selection. Based on their role in alleviating symptoms, these regions may also provide anatomical and physiological information relevant to disease models of dystonia. Further experimental and clinical studies will be needed to validate their importance.

Proceedings of the Second Annual Deep Brain Stimulation Think Tank: What's in the Pipeline.

The proceedings of the 2nd Annual Deep Brain Stimulation Think Tank summarize the most contemporary clinical, electrophysiological, and computational work on DBS for the treatment of neurological and neuropsychiatric disease and represent the insights of a unique multidisciplinary ensemble of expert neurologists, neurosurgeons, neuropsychologists, psychiatrists, scientists, engineers and members of industry. Presentations and discussions covered a broad range of topics, including advocacy for DBS, improving clinical outcomes, innovations in computational models of DBS, understanding of the neurophysiology of Parkinson's disease (PD) and Tourette syndrome (TS) and evolving sensor and device technologies.

Deep brain stimulation for intractable psychiatric disorders.

Deep brain stimulation (DBS) has virtually replaced ablative neurosurgery for use in medication-refractory movement disorders. DBS is now being studied in severe psychiatric conditions, such as treatment-resistant depression (TRD) and intractable obsessive-compulsive disorder (OCD). Effects of DBS have been reported in ∼100 cases of OCD and ∼50 cases of TRD for seven (five common) anatomic targets. Although these published reports differ with respect to study design and methodology, the overall response rate appears to exceed 50% in OCD for some DBS targets. In TRD, >50% of patients responded during acute and long-term bilateral electrical stimulation in a different target. DBS was generally well tolerated in both OCD and TRD, but some unique, target- and stimulation-specific adverse effects were observed (e.g., hypomania). Further research is needed to test the efficacy and safety of DBS in psychiatric disorders, compare targets, and identify predictors of response.

Factors Associated with Extended Hospitalization in Patients Who Had Adjuvant Middle Meningeal Artery Embolization After Conventional Surgery for Chronic Subdural Hematomas.

BACKGROUND: This study aims to evaluate the length of stay (LOS) in patients who had adjunct middle meningeal artery embolization (MMAE) for chronic subdural hematoma after conventional surgery and determine the factors influencing the LOS in this population. METHODS: A retrospective review of 107 cases with MMAE after conventional surgery between September 2018 and January 2024 was performed. Factors associated with prolonged LOS were identified through univariable and multivariable analyses. RESULTS: The median LOS for MMAE after conventional surgery was 9 days (interquartile range = 6-17), with a 3-day interval between procedures (interquartile range = 2-5). Among 107 patients, 58 stayed ≤ 9 days, while 49 stayed longer. Univariable analysis showed the interval between procedures, type of surgery, MMAE sedation, and the number of complications associated with prolonged LOS. Multivariable analysis confirmed longer intervals between procedures (odds ratio [OR] = 1.52; P < 0.01), ≥2 medical complications (OR = 13.34; P = 0.01), and neurological complications (OR = 5.28; P = 0.05) were independent factors for lengthier hospitalizations. There was a trending association between general anesthesia during MMAE and prolonged LOS (P = 0.07). Subgroup analysis revealed diabetes (OR = 5.25; P = 0.01) and ≥2 medical complications (OR = 5.21; P = 0.03) correlated with a LOS over 20 days, the 75th percentile in our cohort. CONCLUSIONS: The interval between procedures and the number of medical and neurological complications were strongly associated with prolonged LOS in patients who had adjunct MMAE after open surgery. Reducing the interval between the procedures and potentially performing both under 1 anesthetic may decrease the burden on patients and shorten their hospitalizations.

Sustained relief of generalized dystonia despite prolonged interruption of deep brain stimulation.

BACKGROUND: Pallidal deep brain stimulation (DBS) is an established treatment for disabling, medication-refractory generalized dystonia. Patients typically regress to their preoperative baseline when stimulation is discontinued. METHODS: Presented are case reports of 2 dystonia patients. RESULTS: Two patients with primary generalized dystonia (1 with the DYT1 mutation) who were treated successfully with bilateral pallidal DBS for periods of 18 months and 5 years retained motor benefit for several months after inadvertent interruption of stimulation. Stimulation was interrupted unilaterally for 3 and 7 months and bilaterally for 2 days and 2 months, respectively. Symptoms of dystonia returned only partially during the period of therapy interruption and rapidly and completely resolved after resuming stimulation. CONCLUSIONS: We report unexpected and prolonged retention of motor benefits despite transient cessation of pallidal DBS in 2 dystonia patients. Factors that appear to differentiate these individuals are young age, short duration of disease, and chronic DBS therapy with relatively low energy of stimulation.

Targeting Accuracy of the Leksell Vantage Stereotactic System for Deep Brain Stimulation Surgery: A Retrospective Review.

BACKGROUND: Compared with the Leksell G frame, the new Leksell Vantage stereotactic headframe offers many benefits during deep brain stimulation (DBS) procedures. OBJECTIVE: To define the "real-world" targeting accuracy of the Vantage frame for performing DBS lead implants. METHODS: Retrospective review of all patients who underwent DBS using the Vantage frame with our surgeon between July 2021 and May 2022. Target and actual coordinates of implanted DBS leads were determined in BrainLab. RESULTS: Thirty-three electrode trajectories (17 left-sided, 16 right-sided) were included. On the right, actual electrode placement was, on average, 0.95 mm more medial, 1.06 mm more posterior, and 0.55 mm more superior compared with planned trajectories. The vector error was 1.93 ± 0.91 mm. On the left, actual electrode placement was 0.49 mm more lateral, 0.66 mm more posterior, and 0.67 mm more superior than planned trajectories. The vector error was 1.68 ± 0.66 mm. After excluding all electrodes that were repositioned after microelectrode recording and/or test stimulation, right-sided electrodes (n = 10) were 0.88 mm more medial, 0.94 mm more posterior, and 0.76 mm more superior than planned trajectories. The vector error was 1.74 ± 1.01 mm. Left-sided electrodes (n = 14) were 0.49 mm more lateral, 0.63 mm more posterior, and 0.49 mm more superior than planned trajectories. The vector error was 1.62 ± 0.68 mm. CONCLUSION: This study demonstrated the accuracy of the Leksell Vantage frame in comparison with the traditional Leksell G frame.

Complications Related to Deep Brain Stimulation Lead Implantation: A Single-Surgeon Case Series.

BACKGROUND: Deep brain stimulation (DBS) is the mainstay of surgical treatment for movement disorders, yet previous studies have shown widely varying complication rates. Given the elective nature of DBS surgery, minimizing surgical complications is imperative. OBJECTIVE: To evaluate short-term and long-term complications related to DBS lead implantation surgeries performed by an experienced surgeon and provide an updated benchmark comparison for other DBS centers and alternative therapies. METHODS: A retrospective chart review of patients who underwent DBS lead implantation surgery by a single surgeon at our institution between 2012 and 2020 was conducted. Demographic and clinical data including surgical complications were collected. A Kaplan-Meier survival analysis was used to evaluate the cumulative risk of lead revision or removal over time. Associations between patient characteristics and various complications were evaluated. RESULTS: Four hundred fifty-one DBS leads were placed in 255 patients. Thirteen leads and 11 patients required revision. In total, 3.6% (95% CI [1.3%-5.9%]) of patients required revision at 1 year and 4.8% (95% CI [1.9%-7.6%]) at 5 years, with per-lead revision rates of 2.3% (95% CI [0.9%-3.6%]) and 3.3% (95% CI [1.5%-5.1%]), respectively. Less common diagnoses such as Tourette syndrome, post-traumatic tremor, and cluster headache trended toward association with lead revision or removal. CONCLUSION: DBS performed by an experienced surgeon is associated with extremely low complication rates.

Intracranial Metastasis of Extracranial Chondrosarcoma: Systematic Review With Illustrative Case.

BACKGROUND: Cerebral chondrosarcoma metastases are rare and aggressive neoplasms. The rarity of presentation has precluded rigorous analysis of diagnosis, risk factors, treatment, and survival. We analyzed every reported case through exhaustive literature review. We further present the first case with Maffucci syndrome. METHODS: Three databases, PubMed, Embase, and Google Scholar, and crossed references were queried for cerebral chondrosarcoma metastases. Extracted variables included demographics, risk factors, tumor characteristics, interventions, and outcomes. Univariate and multivariate analyses were performed. RESULTS: Fifty-six patients were included from 1,489 literature results. The average age at brain metastasis was 46.6±17.6 years and occurred at a median of 24±2.8 months from primary diagnosis. Primary tumor histology (dedifferentiated 5.0±1.5 months, mesenchymal 24±3.0 months, conventional 41±7.4 months, p<0.05) and grade (low grade 54±16.7 months vs. high-grade 10±6.4 months, p<0.001) correlated with time interval until brain metastasis. A multiple enchondromatosis syndrome occurred in 13.2% of cases. At time of brain metastases diagnosis, extracranial metastases were identified in 76.2% of cases. Median survival after the development of brain metastasis was 2.0±0.78 months with a 1-year survival of 10.0%. On regression analysis, surgery reduced brain metastasis mortality risk and radiation trended towards reduced mortality risk (surgery: hazard ratio [HR] 0.22, 95% confidence interval [CI] 0.064-0.763, p=0.017; radiation: HR 0.31, 95% CI 0.091-1.072, p=0.064). CONCLUSION: We present a systematic review of cerebral chondrosarcoma metastases. Primary tumor histology and grade correlate with time until cerebral metastasis. Following cerebral metastasis, these tumors have poor prognosis and modestly benefit from surgery.

Predictors of extended length of stay related to craniotomy for tumor resection.

Background: Hospital length of stay (LOS) related to craniotomy for tumor resection (CTR) is a marker of neurosurgical quality of care. Limiting LOS benefits both patients and hospitals. This study examined which factors contribute to extended LOS (eLOS) at our academic center. Methods: Retrospective medical record review of 139 consecutive CTRs performed between July 2020 and July 2021. Univariate and multivariable analyses determined which factors were associated with an eLOS (≥8 days). Results: Median LOS was 6 days (IQR 3-9 days). Fifty-one subjects (36.7%) experienced an eLOS. Upon univariate analysis, potentially modifiable factors associated with eLOS included days to occupational therapy (OT), physical therapy (PT), and case management clearance (p < .001); and discharge disposition (p < .001). Multivariable analysis revealed that pre-operative anti-coagulant use (OR 10.74, 95% CI 2.64-43.63, p = .001), Medicare (OR 4.80, 95% CI 1.07-21.52, p = .04), ED admission (OR 26.21, 95% CI 5.17-132.99, p < .001), transfer to another service post-surgery (OR 30.00, 95% CI 1.56-577.35, p = .02), and time to post-operative imaging (OR 2.91, 95% CI 1.27-6.65, p = .01) were associated with eLOS. Extended LOS was not significantly associated with ED visits (p = .45) or unplanned readmissions within 30 days of surgery (p = .35), and both (p = .04; p = .04) were less likely following a short LOS (<5 days). Conclusion: While some factors driving LOS related to CTR are uncontrollable, expedient pre- and post-operative management may reduce LOS without compromising care.

Prevalence and Predictors of Inappropriate Antithrombotic Prescription in Patients Presenting With Traumatic Brain Injury.

BACKGROUND AND OBJECTIVES: A growing proportion of the US population is on antithrombotic therapy (AT), most significantly within the older subpopulation. Decision to use AT is a balance between the intended benefits and known bleeding risk, especially after traumatic brain injury (TBI). Preinjury inappropriate AT offers no benefit for the patient and also increases the risk of intracranial hemorrhage and worse outcome in the setting of TBI. Our objective was to examine the prevalence and predictors of inappropriate AT among patients presenting with TBI to a Level-1 Trauma Center. METHODS: A retrospective chart review was performed on all patients with TBI and preinjury AT who presented to our institution between January 2016 and September 2020. Demographic and clinical data were collected. Appropriateness of AT was determined through established clinical guidelines. Clinical predictors were determined by logistic regression. RESULTS: Of 141 included patients, 41.8% were female (n = 59) and the average age (mean ± SD) was 80.6 ± 9.9. The prescribed antithrombotic agents included aspirin (25.5%, n = 36), clopidogrel (22.7%, n = 32), warfarin (46.8%, n = 66), dabigatran (2.1%, n = 3), rivaroxaban (Janssen) (10.6%, n = 15), and apixaban (Bristol-Myers Squibb Co.) (18.4%, n = 26). The indications for AT were atrial fibrillation (66.7%, n = 94), venous thromboembolism (13.4%, n = 19), cardiac stent (8.5%, n = 12), and myocardial infarction/residual coronary disease (11.3%, n = 16). Inappropriate antithrombotic therapy use varied significantly by antithrombotic indication ( P < .001) with the highest rates seen with venous thromboembolism. Predictive factors also include age ( P = .005) with higher rates younger than 65 years and older than 85 years and female sex ( P = .049). Race and antithrombotic agent were not significant predictors. CONCLUSION: Overall, 1 in 10 patients presenting with TBI were found to be on inappropriate AT. Our study is the first to describe this problem and warrants investigation into possible workflow interventions to prevent post-TBI continuation of inappropriate AT.

Outcome of Chronic Subdural Hematoma Intervention in Patients on Long-Term Antiplatelet Therapy-A Propensity Score Matched Analysis.

BACKGROUND: Patients presenting with chronic subdural hematomas (cSDHs) and on antiplatelet medications for various medical conditions often complicate surgical decision making. OBJECTIVE: To evaluate risks of preprocedural and postprocedural antiplatelet use in patients with cSDHs. METHODS: Patients with cSDH who were treated between January 2006 and February 2022 at a single institution with surgical intervention were identified. A propensity score matching analysis was then performed analyzing length of hospitalization, periprocedural complications, reintervention rate, rebleeding risk, and reintervention rates. RESULTS: Preintervention, 178 patients were on long-term antiplatelet medication and 298 were not on any form of antiplatelet. Sixty matched pairs were included in the propensity score analysis. Postintervention, 88 patients were resumed on antiplatelet medication, whereas 388 patients did not have resumption of antiplatelets. Fifty-five pairs of matched patients were included in the postintervention propensity score analysis. No significant differences were found in length of hospitalization (7.8 ± 4.2 vs 6.8 ± 5.4, P = .25), procedural complications (3.3% vs 6.7%, P = .68), or reintervention during the same admission (3.3% vs 5%, P = 1). No significant differences were seen in recurrence rate (9.1% vs 10.9%, P = 1) or reintervention rate after discharge (7.3% vs 9.1%, P = 1) in the postintervention group. CONCLUSION: Preintervention antiplatelet medications before cSDH treatment do not affect length of hospitalization, periprocedural complications, or reintervention. Resumption of antiplatelet medication after cSDH procedures does not increase the rebleeding risk or reintervention rate.

Effect of long-term anticoagulant therapy on the outcome of chronic subdural hematoma: a propensity score-matched analysis.

OBJECTIVE: Chronic subdural hematomas (cSDHs) are particularly common in older adults who have increased risk of falls and the conditions that require anticoagulants (ACs). In such cases, clinicians are often left with the dilemma of co-managing the cSDH and the ongoing need for ACs. METHODS: Patients who underwent surgical management for cSDH at the authors' institution between January 2006 and June 2022 were identified. Propensity score-matched analysis was used to obtain a balance in patients who were on ACs before the procedure versus those who were not, and in patients who were on ACs postprocedure versus those who were not. Length of hospitalization, periprocedural complications, reintervention rate during the same admission, rebleeding risk, and reintervention rates after discharge were compared. RESULTS: In total, 104 patients were on long-term ACs before the procedure, whereas 372 were not. After matching, 55 pairs were included in the analysis. Postprocedure, 74 patients were started on long-term ACs; the rest were not. A total of 49 patients in each group were then included in the analysis after matching. Comparing the preprocedure AC group with the non-AC group, no significant differences were found in length of hospitalization (8.5 ± 6.7 days vs 8.1 ± 7.7 days, p = 0.75), periprocedural complications (7.3% vs 7.3%, p > 0.99), or reintervention during the same admission (1.8% vs 5.5%, p = 0.31). In the comparison of postprocedure AC and non-AC groups, no significant differences were seen in recurrence rate (8.2% vs 14.3%, p = 0.52), reintervention rate after discharge (4.1% vs 14.3%, p = 0.16), or disability (i.e., mRS ≤ 2; 83.7% vs 89.8%, p = 0.55). CONCLUSIONS: Being treated with long-term ACs before cSDH procedures does not affect length of hospitalization, periprocedural complications, or reintervention during the same admission. Similarly, administration of long-term ACs after a procedure for cSDH does not increase rebleeding risk or reintervention rate. Patients who are on long-term ACs can have similar interventions to those who are not on ACs. In addition, it is safe to restart patients on AC agents in a 7- to 14-day window after admission for cSDH with or without acute/subacute components.

Reduced recurrence of chronic subdural hematomas treated with open surgery followed by middle meningeal artery embolization compared to open surgery alone: a propensity score-matched analysis.

OBJECTIVE: Middle meningeal artery embolization (MMAE) is an emerging endovascular treatment technique with proven promising results for chronic subdural hematomas (cSDHs). MMAE as an adjunct to open surgery is being utilized with the goal of preventing the recurrence of cSDH. However, the efficacy of MMAE following surgical evacuation of cSDH has not been clearly demonstrated. The authors sought to compare the outcomes of open surgery followed by MMAE versus open surgery alone. METHODS: Patients who underwent surgical evacuation alone (open surgery-alone group) or MMAE along with open surgery for cSDH (adjunctive MMAE group) were identified at the authors' institution. Two balanced groups were obtained through propensity score matching. Primary outcomes included recurrence risk and reintervention rate. Secondary outcomes included decrease in hematoma size and modified Rankin Scale (mRS) score at last follow-up. Variables in the two groups were compared by use of the Mann-Whitney U-test, paired-sample t-test, and Fisher's exact test. RESULTS: A total of 345 cases of open surgery alone and 52 cases of open surgery with adjunctive MMAE were identified. After control for subjective confounders, 146 patients treated with open surgery alone and 41 with adjunctive MMAE following open surgery with drain placement were included in the analysis. Before matching, the rebleeding risk and reintervention rate for open surgery trended higher in the open surgery alone than the open surgery plus MMAE group (14.4% vs 7.3%, p = 0.18; and 11.6% vs 4.9%, p = 0.17, respectively). No significant differences were seen in duration of radiographic or clinical follow-ups or decreases in hematoma size and mRS score at last follow-up. After one-to-one nearest neighbor propensity score matching, 26 pairs of cases were compared for outcomes. Rates of recurrence (7.7% vs 30.8%, p = 0.038) and overall reintervention (3.8% vs 23.1%, p = 0.049) after open surgery were found to be significantly lower in the adjunctive MMAE group than the open surgery-alone group. With one-to-many propensity score matching, 76 versus 37 cases were compared for open surgery alone versus adjunctive MMAE following open surgery. Similarly, the adjunctive MMAE group had significantly lower rates of recurrence (5.4% vs 19.7%, p = 0.037) and overall reintervention (2.7% vs 14.5%, p = 0.049). CONCLUSIONS: Adjunctive MMAE following open surgery can lower the recurrence risks and reintervention rates for cSDH.

Pallidal deep brain stimulation for DYT6 dystonia.

BACKGROUND: Mutations of the THAP1 gene were recently shown to underlie DYT6 torsion dystonia. Little is known about the response of this dystonia subtype to deep brain stimulation (DBS) at the internal globus pallidus (GPi). METHODS: Retrospective analysis of the medical records of three DYT6 patients who underwent pallidal DBS by one surgical team. The Burke-Fahn-Marsden Dystonia Rating scale served as the primary outcome measure. Comparison is made to 23 patients with DYT1 dystonia also treated with GPi-DBS by the same team. RESULTS: In contrast with the DYT1 patients who exhibited a robust and sustained clinical response to DBS, the DYT6 patients exhibited more modest gains during the first 2 years of therapy, and some symptom regression between years 2 and 3 despite adjustments to the stimulation parameters and repositioning of one stimulating lead. Microelectrode recordings made during the DBS procedures demonstrated no differences in the firing patterns of GPi neurons from DYT1 and DYT6 patients. DISCUSSION: Discovery of the genetic mutations responsible for the DYT6 phenotype allows for screening and analysis of a new homogeneous group of dystonia patients. DYT6 patients appear to respond less robustly to GPi-DBS than their DYT1 counterparts, most likely reflecting differences in the underlying pathophysiology of these distinct genetic disorders. CONCLUSIONS: While early results of pallidal DBS for DYT6 dystonia are encouraging, further research and additional subjects are needed both to optimise stimulation parameters for this population and to elucidate more accurately their response to surgical treatment.

Improvement of both dystonia and tics with 60 Hz pallidal deep brain stimulation.

Deep brain stimulation has been utilized in both dystonia and in medication refractory Tourette syndrome. We present an interesting case of a patient with a mixture of disabling dystonia and Tourette syndrome whose coexistent dystonia and tics were successfully treated with 60 Hz-stimulation of the globus pallidus region.