Subthalamic Nuclei Stimulation in Patients With Pantothenate Kinase-Associated Neurodegeneration (PKAN)
adolescent; subthalamic nucleus; follow up; scoring system; human; article; female; male; adult; middle aged; dystonia; case report; treatment outcome; brain depth stimulation; Deep brain stimulation; neurologic disease assessment; Burke Fahn Marsden Dystonia Rating Scale movement rating scale; fluency disorder; globus pallidus internus; neurodegeneration with brain iron accumulation/th [Therapy]; neurologic examination; pantothenate kinase-associated neurodegeneration; subthalamic nuclei; tone and motor problems; IND; surgical intervention; subthalamic nuclei stimulation
Introduction: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive genetic disease that leads to extrapyramidal symptoms, such as dystonia, ataxia, dysarthria, and involuntary movements. Treatment of PKAN with deep brain stimulation (DBS) has been reported, but mainly focuses on targeting the globus pallidus internus (GPi). Subthalamic nuclei (STN) may also be a potential target for treatment of PKAN. Methods: In this study, we reviewed three patients with PKAN (two with typical PKAN and one with atypical PKAN) treated by bilateral STN stimulation and present a review of the literature. All patients received neurological evaluation using the Burke-Fahn-Marsden Dystonia Rating Scale-movement (BFMDRS) scoring system before and after surgery. Patients were then subject to regular clinical follow-ups (ranging from 22 to 44 months). Results: The mean stimulation amplitude, pulse width and frequency was 2.65 +/- 0.45 V, 91.7 +/- 21.9 mus, and 146.7 +/- 12.5 Hz, respectively. BFMDRS scores were improved in all patients after surgery, ranging from 41.6 to 73.1%. Improvements of appendicular symptoms ranged from 46.2 to 94.1%, and improvements of axial symptoms ranged from 27.3 to 33.3%. No side effects were reported in patients 1 and 2; whereas patient 3 exhibited a mild decline in verbal fluency one year after surgery. Conclusion: STN stimulation could serve as a candidate DBS target in the treatment of PKAN, especially for patients with prominent appendicular symptoms. Copyright © 2017 International Neuromodulation Society
Liu Z; Liu Y; Yang Y; Wang L; Dou W; Guo J; Wang Y; Guo Y; Wan X; Ma W; Wang R
Neuromodulation
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/ner.12549" target="_blank" rel="noreferrer noopener">10.1111/ner.12549</a>
Deep brain stimulation for dystonia: A meta-analysis
meta-analysis; Neurosciences & Neurology; dystonia; movement disorders; follow-up; scale; cervical dystonia; deep brain stimulation; electric stimulation therapy; globus-pallidus internus; hemidystonia; meige-syndrome; primary generalized dystonia; Research & Experimental Medicine; severe tardive; spasmodic torticollis; stereotaxic techniques; thalamic-stimulation; tone and motor problems; IND; surgical intervention; deep brain stimulation; BFMDRS
Objective. To use a meta-analysis on all reported cases of deep brain stimulation (DBS) for dystonia to determine which factors significantly influence outcome. The Burke-Fahn-Marsden (BFM) movement scale, the most reported measure, was chosen as the primary outcome measure for this analysis. Methods. A MEDLINE search identified 137 patients who underwent DBS for dystonia in 24 studies that had individual BFM scores. Individual patient data, including age at onset of dystonia, age at surgery, gender, distribution of dystonia, etiology of dystonia, presence of associated features, abnormality of preoperative imaging, prior stereotactic surgeries, nucleus stimulated, type of anesthesia used, use of physiologic monitoring, type of imaging used for localization, stimulation parameters used, time of response to stimulation, and timing of outcome assessment were entered into an SPSS database for statistical analysis. Results. The mean BFM percentage change (improvement in postoperative score from baseline) was 51.8% (range -34% to 100%). Significantly better outcomes were achieved with stimulation of the globus pallidus internus (GPi) than with stimulation of the posterior portion of the ventral lateral (VLp) nucleus of the thalamus (p = 0.0001). The etiology of the dystonia also had a significant effect on outcomes. Statistically significant improvements in outcomes were seen for all etiologic categories, except encephalitis. Dystonia due to birth injury and encephalitis had significantly worse outcomes when compared to other etiologies. However, there were no significant differences in the outcomes of patients who were DYT1 (DYT1 is the gene associated with the disorder Dystonia Musculorum Deformans) gene positive, DYT1 gene negative, or had pantothenate kinase-associated neurodegeneration (PKAN), tardive dyskinesia, and idiopathic and posttraumatic dystonias. Longer duration of dystonia symptoms correlated negatively with surgical outcome. A regression model using the three variables-stimulation site, etiology of dystonia, and duration of dystonia symptoms-explained 51% of the variance in outcomes. Conclusion. Deep brain stimulation of the GPi provides significant improvement in BFM scores in a variety of dystonic conditions.
Holloway K L; Baron M S; Brown R; Cifu D X; Carne W; Ramakrishnan V
Neuromodulation
2006
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1525-1403.2006.00067.x" target="_blank" rel="noreferrer noopener">10.1111/j.1525-1403.2006.00067.x</a>
Neurophysiological Underpinnings of Electronic Analgesic Neuromodulation for Dummies
Analgesic; Neuromodulation
Electronic neuromodulation can be a safe and effective treatment for intractable pain. Unfortunately, many physicians and other healthcare providers know nothing of neuromodulatory techniques. There is little opportunity to learn about them in medical school or during general medical training. Nearly all of the literature about neuromodulation is aimed at specialists who already have a detailed knowledge of the field. This article reviews the pathophysiology of chronic pain from the point of view of a primary care practitioner, with the aim of providing a rationale for the appropriate use of electronic neurostimulators in patients with chronic pain. In order to understand advanced pain management, it is important to first understand that pain management is not about treating pain, but about “reducing hypersensitivity.” Specifically, advanced pain management techniques are aimed at the pathophysiological processes of hyperalgesia, allodynia, neurogenic inflammation, and neural remodeling. Some approaches to electronic analgesic neuromodulation are summarized.
2006-05
Brookoff D
Pain Medicine
2006
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1111/j.1526-4637.2006.00126.x" target="_blank" rel="noreferrer">10.1111/j.1526-4637.2006.00126.x</a>
Historical Developments In Children's Deep Brain Stimulation
Brain Depth Stimulation; Dystonia; Pediatrics; Adult; Basal Ganglion; Central Nervous System; Child; Clinical Feature; Clinical Outcome; Clinical Study; Degenerative Disease; Dystonia/su [surgery]; Dystonic Disorder/su [surgery]; Globus Pallidus; Human; Medical History; Myoclonus; Myoclonus Dystonia/su [surgery]; Nerve Cell Network; Nerve Conduction; Neuromodulation; Palliative Therapy; Priority Journal; Review; Side Effect; Subthalamic Nucleus; Surgery; Symptom; Thalamus; Thalamus Nucleus
Background Heterogeneous by the underlying pathobiology and clinical presentation, childhood onset dystonia is most frequently progressive, with related disability and limitations in functions of daily living. Consequently, there is an obvious need for efficient symptomatic therapies. Methods and Results Following lesional surgery to basal ganglia (BG) and thalamus, deep brain stimulation (DBS) is a more conservative and adjustable intervention to and validated for internal segment of the globus pallidus (GPi), highly efficient in treating isolated "primary" dystonia and associated symptoms such as subcortical myoclonus. The role of DBS in acquired, neurometabolic and degenerative disorders with dystonia deserves further exploration to confirm as an efficient and lasting therapy. However, the pathobiological background with distribution of the sequellae over the central nervous system and related clinical features, will limit DBS efficacy in these conditions. Cumulative arguments propose DBS in severe life threatening dystonic conditions called status dystonicus as first line therapy, irrespective of the underlying cause. There are no currently available validated selection criteria for DBS in pediatric dystonia. Concurrent targets such as subthalamic nucleus (STN) and several motor nuclei of the thalamus are under exploration and only little information is available in children. DBS programming in paediatric population was adopted from experience in adults. The choice of neuromodulatory DBS parameters could influence not only the initial therapeutic outcome of dystonic symptoms but also its maintenance over time and potentially the occurrence of DBS related side effects. Conclusion DBS allows efficient symptomatic treatment of severe dystonia in children and advances pathophysiological knowledge about local and distributed abnormal neural activity over the motor cortical-subcortical networks in dystonia and other movement disorders. Copyright © 2016 The Authors
Cif L; Coubes P
European Journal Of Paediatric Neurology
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
10.1016/j.ejpn.2016.08.010