Pallidal Beta Activity Is Linked to Stimulation-Induced Slowness in Dystonia.

BACKGROUND: Pallidal deep brain stimulation (DBS) effectively alleviates symptoms in dystonia patients, but may induce movement slowness as a side-effect. In Parkinson's disease, hypokinetic symptoms have been associated with increased beta oscillations (13-30 Hz). We hypothesize that this pattern is symptom-specific, thus accompanying DBS-induced slowness in dystonia. METHODS: In 6 dystonia patients, pallidal rest recordings with a sensing-enabled DBS device were performed and tapping speed was assessed using marker-less pose estimation over 5 time points following cessation of DBS. RESULTS: After cessation of pallidal stimulation, movement speed increased over time (P < 0.01). A linear mixed-effects model revealed that pallidal beta activity explained 77% of the variance in movement speed across patients (P = 0.01). CONCLUSIONS: The association between beta oscillations and slowness across disease entities provides further evidence for symptom-specific oscillatory patterns in the motor circuit. Our findings might help DBS therapy improvements, as DBS-devices able to adapt to beta oscillations are already commercially available. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

American Cancer Society Head and Neck Cancer Survivorship Care Guideline.

Answer questions and earn CME/CNE The American Cancer Society Head and Neck Cancer Survivorship Care Guideline was developed to assist primary care clinicians and other health practitioners with the care of head and neck cancer survivors, including monitoring for recurrence, screening for second primary cancers, assessment and management of long-term and late effects, health promotion, and care coordination. A systematic review of the literature was conducted using PubMed through April 2015, and a multidisciplinary expert workgroup with expertise in primary care, dentistry, surgical oncology, medical oncology, radiation oncology, clinical psychology, speech-language pathology, physical medicine and rehabilitation, the patient perspective, and nursing was assembled. While the guideline is based on a systematic review of the current literature, most evidence is not sufficient to warrant a strong recommendation. Therefore, recommendations should be viewed as consensus-based management strategies for assisting patients with physical and psychosocial effects of head and neck cancer and its treatment. CA Cancer J Clin 2016;66:203-239. © 2016 American Cancer Society.

Deep brain stimulation in Lesch-Nyhan syndrome: a systematic review.

Given the good results of deep brain stimulation (DBS) in the treatment of movement disorders, DBS was initially tried to treat Lesch-Nyhan syndrome (LNS) with the aim to alleviate LNS-related dystonia. Some cases have reported clinical results of DBS in LNS thus far. This systematic review was conducted to comprehensively summarize cases of LNS treated with DBS and evaluate the efficacy and safety of DBS in LNS. Eight publications covering 12 LNS patients were included in this review. DBS improved dystonia of the LNS to varying degrees. All the included cases achieved partial or complete control of self-injurious behavior (SIB). Overall, DBS is a promising treatment for both motor and behavior disorders of LNS patients, but the results reported thus far have varied widely, especially for motor outcomes. The ultimate clinical benefits in LNS patients were still unpredictable. DBS-related complications were rather common, which raised questions about the safety of the procedure in LNS. More research is needed to further clarify the safety and effectiveness of this treatment.

Complications of Deep Brain Stimulation for Movement Disorders: Literature Review and Personal Experience.

The contemporary technique of deep brain stimulation (DBS) is very effective for management of movement disorders-including Parkinson's disease, generalized dystonia, and tremors-and has also been successfully applied for novel indications (e.g., intractable epilepsy and chronic pain). As a result, growing numbers of DBS procedures have been performed worldwide; correspondingly, the incidence of associated morbidity has also increased. All complications of DBS can be divided into those associated with (1) the surgical procedure, (2) the device itself, and (3) the applied electrical stimulation. On the basis of an analysis of the available literature and the personal experience of the author, it may be concluded that implantation of a DBS device is a relatively safe procedure accompanied by very low risks of major morbidity or a permanent neurological deficit. Nevertheless, awareness of the possible complications and application of appropriate preventive measures for their avoidance are very important for providing safe and effective treatment.

Long-Term Outcome of Subthalamic Deep Brain Stimulation for Generalized Isolated Dystonia.

OBJECTIVES: Few studies have focused on subthalamic nucleus deep brain stimulation for refractory isolated dystonia, and the long-term outcomes are unclear. In this study, we evaluated the efficacy of subthalamic stimulation for generalized isolated dystonia for more than five years and explored the factors predicting clinical outcomes. MATERIALS AND METHODS: A total of 16 patients with generalized isolated dystonia underwent a two-phase procedure for stimulation system implantation. After implanting the leads, we performed a test stimulation and observed the stimulation response. The severity of dystonia was assessed using a blinded rating of the Burke-Fahn-Marsden Dystonia Rating Scale based on videos recorded at scheduled times. RESULTS: The mean follow-up time was 7.4 ± 2.2 years (5-12.5 years). The severity of dystonia improved significantly one year after surgery. The movement score decreased from 49.3 (40.9) points at baseline to 26.5 (43.5) points (-44.6%) at six months, 12.0 (22.5) points (-66.8%) at one year, 11.25 (17.6) points (-72.7%) at three years, and 12.5 (21.0) points (-72.6%) at the last follow-up. The improvement in motor symptoms resulted in a corresponding improvement in activities of daily living. Greater long-term outcomes were correlated with early stimulation responses, lower baseline movement scores, and female sex. When analyzed comprehensively, only the baseline movement score had meaningful predictive value for the outcome. CONCLUSIONS: Our results indicate that subthalamic stimulation is effective and durable in treating generalized isolated dystonia. The subthalamic nucleus may be an alternative target for the treatment of refractory dystonia. Patients with less severe motor symptoms may benefit more from this treatment.

Clinical and Psychosocial Factors Considered When Deciding Whether to Offer Deep Brain Stimulation for Childhood Dystonia.

INTRODUCTION: Childhood dystonia is often nonresponsive to medications, and refractory cases are increasingly being treated with deep brain stimulation (DBS). However, many have noted that there is little consensus about when DBS should be offered, and there has been little examination of clinicians' decision-making process when determining whether to offer DBS for childhood dystonia. OBJECTIVES: This study aimed to identify and examine the factors considered by pediatric movement disorder specialists before offering DBS. MATERIALS AND METHODS: Semistructured interviews (N = 29) with pediatric dystonia clinicians were conducted, transcribed, and coded. Using thematic content analysis, nine central themes were identified when clinicians were asked about key factors, clinical factors, and psychosocial factors considered before offering pediatric DBS. RESULTS: Clinicians identified nine main factors. Five of these were classified primarily as clinical factors: early intervention and younger age (raised by 86% of respondents), disease progression and symptom severity (83%), etiology and genetic status (79%), clinicians' perceived risks and benefits of DBS for the patient (79%), and exhaustion of other treatment options (55%). The remaining four were classified primarily as psychosocial factors: social and family support (raised by 97% of respondents), patient and caregiver expectations about outcomes and understanding of DBS treatment (90%), impact of dystonia on quality of life (69%), and financial resources and access to care (31%). CONCLUSIONS: Candidacy determinations, in this context, are complicated by an interrelation of clinical and psychosocial factors that contribute to the decision. There is potential for bias when considering family support and quality of life. Uncertainty of outcomes related to the etiology of dystonia makes candidacy judgments challenging. More systematic examination of the characteristics and criteria used to identify pediatric patients with dystonia who can significantly benefit from DBS is necessary to develop clear guidelines and promote the well-being of these children.

Deep brain stimulation for chorea-acanthocytosis: a systematic review.

Deep brain stimulation (DBS) is a reversible treatment for chorea-acanthocytosis (ChAc). Its safety and efficacy remain elusive due to the low prevalence of ChAc. We aimed to investigate the safety and efficacy of DBS for ChAc by systematically reviewing literature through PubMed and EMBASE. Inclusion criteria were reports on the efficacy or safety of DBS for ChAc and English language articles, and exclusion criteria were other movement disorders, non-human subjects, and studies without original data. Most studies were published as case reports, and we therefore pooled these cases in one cohort. Twenty studies with 34 patients were included. The mean age of symptom onset was 29.3 years (range, 17-48). The median follow-up was 12 months (range, 2-84). Twenty-nine patients underwent GPi-DBS, two received STN-DBS, and one underwent Vop-DBS. Electrodes were implanted into the ventralis oralis complex of the thalamus and the pallidal in two patients. Symptoms seemed to be easier relieved in chorea (88.5%) and dystonia (76.9%) but dysarthria of most patients (85.7%) was no response after DBS. The Unified Huntington's Disease Rating Scale-Motor Score was used to assess the efficacy of DBS in 25 patients; the mean score decreased from 43.2 to 22.3 and the median improvement rate was 46.7%. Of 24 patients with data on adverse events, complications occurred in 9 patients (37.5%; mostly transient and mild events). DBS is a promising treatment for ChAc with satisfactory efficacy and safety based on the review. Pallidal and thalamic DBS have been applied in ChAc; GPi-DBS seems to be more widely used.

Bilateral Pallidotomy for Dystonia: A Systematic Review.

Stereotactic lesioning of the bilateral globus pallidus (GPi) was one of the first surgical treatments for medication-refractory dystonia but has largely been abandoned in clinical practice after the introduction of deep brain stimulation (DBS). However, some patients with dystonia are not eligible for DBS. Therefore, we reviewed the efficacy, safety, and sustainability of bilateral pallidotomy by conducting a systematic review of individual patient data (IPD). Guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and IPD were followed. In May 2020, Medline, Embase, Web of Science, and Cochrane Library were searched for studies reporting on outcome of bilateral pallidotomy for dystonia. If available, IPD were collected. In this systematic review, 100 patients from 33 articles were evaluated. Adverse events were reported in 20 patients (20%), of which 8 were permanent (8%). Pre-and postoperative Burke-Fahn-Marsden Dystonia Rating Movement Scale scores were available for 53 patients. A clinically relevant improvement (>20%) of this score was found in 42 of 53 patients (79%). Twenty-five patients with status dystonicus (SD) were described. In all but 2 the SD resolved after bilateral pallidotomy. Seven patients experienced a relapse of SD. Median-reported follow-up was 12 months (n = 83; range: 2-180 months). Based on the current literature, bilateral pallidotomy is an effective and relatively safe procedure for certain types of dystonia, particularly in medication-refractory SD. Although due to publication bias the underreporting of negative outcomes is very likely, bilateral pallidotomy is a reasonable alternative to DBS in selected dystonia patients. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Arching deep brain stimulation in dystonia types.

Although medical treatment including botulinum toxic injection is the first-line treatment for dystonia, response is insufficient in many patients. In these patients, deep brain stimulation (DBS) can provide significant clinical improvement. Mounting evidence indicates that DBS is an effective and safe treatment for dystonia, especially for idiopathic and inherited isolated generalized/segmental dystonia, including DYT-TOR1A. Other inherited dystonia and acquired dystonia also respond to DBS to varying degrees. For Meige syndrome (craniofacial dystonia), other focal dystonia, and some rare inherited dystonia, further evidences are still needed to evaluate the role of DBS. Because short disease duration at DBS surgery and absence of fixed musculoskeletal deformity are associated with better outcome, DBS should be considered as early as possible when indicated after careful evaluation including genetic work-up. This review will focus on the factors to be considered in DBS for patients with dystonia and the outcome of DBS in the different types of dystonia.

Efficacy and safety of general anesthesia deep brain stimulation for dystonia: an individual patient data meta-analysis of 341 cases.

OBJECTIVE: The efficacy and safety of deep brain stimulation (DBS) under general anesthesia for the treatment of dystonia have not yet been confirmed with high level of evidence. This meta-analysis with pooled individual patient data aims to assess the clinical outcomes and identify the potential prognostic factors of dystonia patients who underwent general anesthesia DBS. METHODS: We searched PubMed, Web of Science, and Embase for articles describing patients with dystonia who underwent asleep DBS and had individual Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores. The relative improvement in BFMDRS scores was considered the primary outcome. Pearson correlation analyses and multivariate linear regression analysis were conducted to explore the prognostic factors. RESULTS: A total of 34 studies involving 341 patients were included. The mean postoperative improvement in BFMDRS-M (BFMDRS movement subscale) and BFMDRS-D (BFMDRS disability subscale) scores were 58.6±36.2% and 48.5±38.7% at the last follow-up visit, respectively, with a mean follow-up time of 22.4±27.6 months. Age at surgery and disease duration showed a negative correlation with the percent improvement of BFMDRS-M (%) at the last visit (r=-0.134, P=0.013; r=-0.165, P=0.006). In the stepwise multivariate regression, only disease duration remained a relevant factor. Additionally, the adverse events were acceptable. CONCLUSION: General anesthesia DBS is a safe, effective, and feasible option for dystonia patients in the long term. Shorter disease duration predicts better clinical outcomes.

Bilateral Pallidal Stimulation with Directional Leads for Primary Focal Lingual Dystonia.

There have been limited studies regarding stereotactic and functional neurosurgery for lingual dystonia. Here, we report a patient with primary lingual dystonia who showed significant improvement after bilateral deep brain stimulation (DBS). A 42-year-old woman presented with a 5- to 6-year history of tongue protrusion; however, she lacked a significant medical or medication history before onset. She presented with gradually worsening symptoms and was diagnosed with idiopathic lingual dystonia. Her tongue was injected with botulinum toxin on 6 occasions; however, it had a limited effect. Oral medications were ineffective. She underwent DBS since her involuntary tongue movements were causing nocturnal breathing problems. Directional leads were bilaterally inserted into the internal segment of the globus pallidus (GPi). The directional part of each lead was inserted at the GPi bottom on both sides. The posteromedial contacts were used to deliver stimulation. After 1.5 years, the patient's Burke-Fahn-Marsden dystonia rating scale score improved from 9 to 1.5 and 2 to 1 for movement and disability, respectively. This case demonstrated the effectiveness of bilateral GPi-DBS. Placing the directional part of the lead in the GPi bottom could improve the stimulation effects.

Subthalamic Nucleus Deep Brain Stimulation Lessens Acquired Dystonia: Report of Two Patients and Systematic Review of Published Cases.

INTRODUCTION: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an efficient treatment of primary dystonia. Few studies have reported the effect of STN-DBS on secondary or acquired dystonia. METHODS: We reported 2 patients with acquired dystonia treated by subthalamic DBS and followed up for 24 months, besides providing a systematic review and meta-analysis of published series. RESULTS/CONCLUSIONS: Both patients had thalamic vascular or autoimmune lesions within the ventral and the pulvinar nuclei. A reduction of 67.2% on the Burke-Fahn-Marsden Dystonia Rating Scale and 90% improvement in disability scores were shown in the first patient, while the second patient showed a lower reduction in both dystonia symptoms (28.6%) and disability scores (44%). Both patients had a significant mean improvement in the quality of life (62.5% in the first and 57.9% in the second) and were free of drugs postoperatively. A systematic review showed a mean follow-up of 13 months in 19 patients, including our 2 patients. The review showed a significant Burke-Fahn-Marsden Dystonia Scale (BFMDRS) score median reduction of 19 points (52.4%; confidence interval [CI]: 11.0-25.0) and a significant median reduction of 6 points in disability scores (44.5%; 95% CI: 4.0-14.0), thereby improving quality of life. Age at surgery was inversely correlated with postoperative improvement (r = 0.63; p = 0.039). Hemidystonia had a nonsignificant better improvement than generalized dystonia (55.3 vs. 43.5%; p = 0.4433). No association between etiology and postoperative improvement and no serious complications were found. Although few data reported so far, subthalamic DBS is likely efficient for acquired dystonia.

Asleep Deep Brain Stimulation in Patients With Isolated Dystonia: Stereotactic Accuracy, Efficacy, and Safety.

OBJECTIVES: Lead placement for deep brain stimulation (DBS) is routinely performed using neuroimaging or microelectrode recording (MER). Recent studies have demonstrated that DBS under general anesthesia using an imaging-guided target technique ("asleep" DBS) can be performed accurately and effectively with lower surgery complication rates than the MER-guided target method under local anesthesia ("awake" DBS). This suggests that asleep DBS may be a more acceptable method. However, there is limited direct evidence focused on isolated dystonia using this method. Therefore, this study aimed to investigate the clinical outcomes and targeting accuracy in patients with dystonia who underwent asleep DBS. MATERIALS AND METHODS: We examined 56 patients (112 leads) with isolated dystonia who underwent asleep DBS targeting in the globus pallidus internus (GPi) and subthalamic nucleus (STN). The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores were assessed preoperatively and at 12-month follow-up (12 m-FU). The lead accuracy was evaluated by comparing the coordinates of the preoperative plan with those of the final electrode implantation location. Other measures analyzed included stimulation parameters and adverse events (AEs). RESULTS: For both GPi and STN cohorts, mean BFMDRS motor scores were significantly lower at 12 m-FU (8.9 ± 10.9 and 4.6 ± 5.7 points) than at baseline (22.6 ± 16.4 and 16.1 ± 14.1 points, p < 0.001). The mean difference between the planned target and the distal contact of the leads was 1.33 ± 0.54 mm for the right brain electrodes and 1.50 ± 0.57 mm for the left, determined by Euclidian distance. No perioperative complications or AEs related to the device were observed during the complete follow-up. However, AEs associated with stimulation occurred in 12 and 6 patients in the GPi and STN groups, respectively. CONCLUSIONS: Asleep DBS may be an accurate, effective, and safe method for treating patients with isolated dystonia regardless of the stimulation target.

Pallidal Deep Brain Stimulation Reduces Sensorimotor Cortex Activation in Focal/Segmental Dystonia.

BACKGROUND: Although deep brain stimulation of the globus pallidus internus (GPi-DBS) is an established treatment for many forms of dystonia, including generalized as well as focal forms, its effects on brain (dys-)function remain to be elucidated, particularly for focal and segmental dystonia. Clinical response to GPi-DBS typically comes with some delay and lasts up to several days, sometimes even weeks, once stimulation is discontinued. OBJECTIVE: This study investigated how neural activity during rest and motor activation is affected by GPi-DBS while excluding the potential confound of altered feedback as a result of therapy-induced differences in dystonic muscle contractions. METHODS: Two groups of patients with focal or segmental dystonia were included in the study: 6 patients with GPi-DBS and 8 without DBS (control group). All 14 patients had cervical dystonia. Using H215 O PET, regional cerebral blood flow was measured at rest and during a motor task performed with a nondystonic hand. RESULTS: In patients with GPi-DBS (stimulation ON and OFF), activity at rest was reduced in a prefrontal network, and during the motor task, sensorimotor cortex activity was lower than in patients without DBS. Within-group contrasts (tapping > rest) showed less extensive task-induced motor network activation in GPi-DBS patients than in non-DBS controls. Reduced sensorimotor activation amounted to a significant group-by-task interaction only in the stimulation ON state. CONCLUSIONS: These findings support previous observations in generalized dystonia that suggested that GPi-DBS normalizes dystonia-associated sensorimotor and prefrontal hyperactivity, indicating similar mechanisms in generalized and focal or segmental dystonia. Evidence is provided that these effects extend into the OFF state, which was not previously demonstrated by neuroimaging. © 2020 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.

Deep brain stimulation for cerebral palsy: where are we now?

Cerebral palsy (CP) is a complex disorder and children frequently have multiple impairments. Dystonia is a particularly frustrating impairment that interferes with rehabilitation and function and is difficult to treat. Of the available treatments, deep brain stimulation (DBS) has emerged as an option with the potential for large effect size in a subgroup of children. While brain stimulation has been used in CP for more than 40 years, modern devices and targeting methods are improving both the safety and efficacy of the procedure. Successful use of DBS depends on appropriate selection of patients, identification of effective neuroanatomical targets in each patient, careful neurosurgical procedure, and detailed follow-up evaluation and programming. The use of functional neurosurgery for neuromodulation in CP remains a technology in its infancy, but improving experience and knowledge are likely to make this one of the safest and most effective interventions for children with moderate-to-severe motor disorders. This review summarizes the current procedures for patient and target selection, and surgical implantation of DBS electrodes for CP. The history of DBS and future directions when used in secondary dystonia are also examined. WHAT THIS PAPER ADDS: Selection of candidates for deep brain stimulation (DBS) requires understanding of dystonia in cerebral palsy . DBS could become a first-line treatment option in some children.

ESTIMULACIÓN CEREBRAL PROFUNDA PARA LA PARÁLISIS CEREBRAL: ¿DÓNDE ESTAMOS AHORA?: La parálisis cerebral (PC) es un trastorno complejo y los niños con frecuencia tienen discapacidades múltiples. La distonía es un deterioro particularmente frustrante que interfiere con la rehabilitación y la función y es difícil de tratar. De los tratamientos disponibles, la estimulación cerebral profunda (DBS, por sus siglas en inglés - deep brain stimulation) ha surgido como una opción con el potencial de un gran tamaño de efecto en un subgrupo de niños. Si bien la estimulación cerebral se ha utilizado en PC durante más de 40 años, los dispositivos modernos y los métodos de detección están mejorando tanto la seguridad como la eficacia del procedimiento. El uso exitoso de la DBS depende de la selección apropiada de los pacientes, la identificación de objetivos neuroanatómicos efectivos en cada paciente, el procedimiento neuroquirúrgico cuidadoso y la evaluación y programación de seguimiento detalladas. El uso de la neurocirugía funcional para la neuromodulación en la PC sigue siendo una tecnología en su infancia, pero es probable que la mejora de la experiencia y los conocimientos hagan de esta una de las intervenciones más seguras y efectivas para los niños con trastornos motores de moderados a graves. Esta revisión resume los procedimientos actuales para la selección de pacientes y objetivos, y la implantación quirúrgica de electrodos DBS para PC. La historia de DBS y las direcciones futuras cuando se utilizan en la distonía secundaria también se examinan.

ESTIMULAÇÃO CEREBRAL PROFUNDA PARA PARALISIA CEREBRAL: ONDE ESTAMOS AGORA?: Paralisia cerebral (PC) é uma desordem complexa e crianças frequentemente apresentam múltiplas deficiências. A distonia é uma deficiência particularmente frustrante que interfere com a reabilitação e função, e é difícil de tratar. Dentre dos tratamentos disponíveis, a estimulação cerebral profunda (ECP) emergiu como uma opção com potencial de grande tamanho de efeito em um subgrupo de crianças. Embora a estimulação cerebral seja usada em PC há mais de 40 anos, dispositivos modernos e métodos de identificação de áreas alvo tem melhorado tanto a segurança quanto a eficácia do procedimento. O uso bem sucedido da ECP depende da seleção apropriada dos pacientes, identificação dos alvos neuroanatômicos efetivos para cada paciente, procedimento neurocirúrgico cuidadoso, e avaliação e programação detalhadas no acompanhamento. O uso de neurocirurgia funcional para neuromodulação em PC ainda é uma tecnologia iniciante, mas a crescente experiência e conhecimento provavelmente farão dela uma das mais seguras e efetivas intervenções para crianças com transtornos motores de moderados a severos. Esta revisão sintetiza os procedimentos atuais para seleção de pacientes e alvos, e a implantação cirúrgica de eletrodos de ECP para PC. A história da ECP e direções futuras, quando usados em distonia secundária, também são examinados.

Subthalamic Nucleus Deep Brain Stimulation in Post-Infarct Dystonia.

Dystonia secondary to cerebral infarcts presents months to years after the initial insult, is usually unilateral and causes significant morbidity. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is established as the most frequent target in the management of the dystonic symptoms. We report our experience with subthalamic nucleus (STN) DBS in 3 patients with post-infarct dystonia, in whom GPi DBS was not confidently possible due to the presence of striatal infarcts. Two patients had unilateral STN DBS implantation, whereas the third patient had bilateral STN DBS implantation for bilateral dystonic symptoms. Prospectively collected preoperative and postoperative functional assessment data including imaging, medication and neuropsychology evaluations were analyzed with regard to symptom improvement. Median follow-up period was 38.3 months (range 26-43 months). All patients had clinically valuable improvements in dystonic symptoms and pain control despite variable improvements in the Burke-Fahn-Marsden dystonia rating scores. In our series, we have demonstrated that STN DBS could be an alternative in the management of post-infarct dystonia in patients with abnormal striatal anatomy which precludes GPi DBS. A multidisciplinary team-based approach is essential for patient selection and management.

Pediatric Deep Brain Stimulation for Dystonia: Current State and Ethical Considerations.

Dystonia is a movement disorder that can have a debilitating impact on motor functions and quality of life. There are 250,000 cases in the United States, most with childhood onset. Due to the limited effectiveness and side effects of available treatments, pediatric deep brain stimulation (pDBS) has emerged as an intervention for refractory dystonia. However, there is limited clinical and neuroethics research in this area of clinical practice. This paper examines whether it is ethically justified to offer pDBS to children with refractory dystonia. Given the favorable risk-benefit profile, it is concluded that offering pDBS is ethically justified for certain etiologies of dystonia, but it is less clear for others. In addition, various ethical and policy concerns are discussed, which need to be addressed to optimize the practice of offering pDBS for dystonia. Strategies are proposed to help address these concerns as pDBS continues to expand.

Essential tremor: a nuanced approach to the clinical features.

Click here to listen to the Podcast Essential tremor is the most common form of tremor in humans. Given neurologists' high exposure to this condition, and its seemingly straightforward phenotype, it might seem easy to diagnose. However, 30%-50 % of patients labelled as having 'essential tremor' have other diagnoses, mostly Parkinson's disease and dystonia. The tremor of essential tremor is neither non-descript nor featureless but is multifaceted and highly patterned. This review focuses on its clinical features, beginning with a discussion of tremors and then briefly discussing its additional motor features, and presents several aids to help distinguish essential tremor from Parkinson's disease and dystonia. Careful attention to certain clinical nuances will aid the diagnosis and care of patients with essential tremor.

Hyperbaric oxygen for late sequelae of carbon monoxide poisoning enhances neurological recovery: case report.

Neuropsychiatric sequelae have been reported in 15%-45% of survivors of carbon monoxide (CO) poisoning. Hyperbaric oxygen (HBO2) therapy reduces the incidence of cognitive and neurological a dysfunction. The efficacy of providing HBO2 beyond the first one to two days after initial insult is unknown. However, some evidence exists for the benefit of this treatment. We report on treating a patient 14 months after CO injury, who responded with markedly improved neurologic status. A 27-year-old scholar was found comatose due to CO poisoning (carboxyhemoglobin = 31.7%). He received five acute HBO2 treatments. After discharge, he developed chorea, Parkinsonism, dystonia, memory loss, slowed processing speed and verbal fluency, leaving him disabled. After the patient reached a clinical plateau, HBO2 was tried again at 90 minutes at 2.4 ATA plus air breaks. Neuropsychological testing was performed at baseline and after each 20 HBO2 cycles, five of which were performed during the period from 14-22 months after CO exposure. After the first 20 treatments, Parkinsonism and dystonia improved. After 40 sessions, further improvements were seen on mental speed, verbal fluency, and fine motor movements. The outcome following 100 treatments was that the patient regained independence, including the ability to drive and to become gainfully employed. Our case calls into question the concept that HBO2 therapy has no role during the chronic phase of CO brain injury. Randomized clinical trials should be considered to evaluate the therapeutic efficacy of HBO2 in patients with neurological sequelae following CO injury.