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Status dystonicus is the most severe form of dystonia with life-threatening complications if not treated promptly. We present consensus recommendations for the initial management of acutely worsening dystonia (including pre-status dystonicus and status dystonicus), as well as refractory status dystonicus in children. This guideline provides a stepwise approach to assessment, triage, interdisciplinary treatment, and monitoring of status dystonicus. The clinical pathways aim to: (1) facilitate timely recognition/triage of worsening dystonia, (2) standardize supportive and dystonia-directed therapies, (3) provide structure for interdisciplinary cooperation, (4) integrate advances in genomics and neuromodulation, (5) enable multicenter quality improvement and research, and (6) improve outcomes. © 2024 International Parkinson and Movement Disorder Society.
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OBJECTIVE: To deconstruct the epileptogenic networks of patients with drug-resistant epilepsy (DRE) using source functional connectivity (FC) analysis; unveil the FC biomarkers of the epileptogenic zone (EZ); and develop machine learning (ML) models to estimate the EZ using brief interictal electroencephalography (EEG) data. METHODS: We analyzed scalp EEG from 50 patients with DRE who had surgery. We reconstructed the activity (electrical source imaging [ESI]) of virtual sensors (VSs) across the whole cortex and computed FC separately for epileptiform and non-epileptiform EEG epochs (with or without spikes). In patients with good outcome (Engel 1a), four cortical regions were defined: EZ (resection) and three non-epileptogenic zones (NEZs) in the same and opposite hemispheres. Region-specific FC features in six frequency bands and three spatial ranges (long, short, inner) were compared between regions (Wilcoxon sign-rank). We developed ML classifiers to identify the VSs in the EZ using VS-specific FC features. Cross-validation was performed using good outcome data. Performance was compared with poor outcomes and interictal spike localization. RESULTS: FC differed between EZ and NEZs (p < .05) during non-epileptiform and epileptiform epochs, showing higher FC in the EZ than its homotopic contralateral NEZ. During epileptiform epochs, the NEZ in the epileptogenic hemisphere showed higher FC than its contralateral NEZ. In good outcome patients, the ML classifiers reached 75% accuracy to the resection (91% sensitivity; 74% specificity; distance from EZ: 38 mm) using epileptiform epochs (gamma and beta frequency bands) and 62% accuracy using broadband non-epileptiform epochs, both outperforming spike localization (accuracy = 47%; p < .05; distance from EZ: 57 mm). Lower performance was seen in poor outcomes. SIGNIFICANCE: We present an FC approach to extract EZ biomarkers from brief EEG data. Increased FC in various frequencies characterized the EZ during epileptiform and non-epileptiform epochs. FC-based ML models identified the resection better in good than poor outcome patients, demonstrating their potential for presurgical use in pediatric DRE.
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Epilepsia Refractaria , Electroencefalografía , Humanos , Niño , Electroencefalografía/métodos , Epilepsia Refractaria/cirugía , Imagen por Resonancia Magnética , BiomarcadoresRESUMEN
Epilepsy is increasingly considered a disorder of brain networks. Studying these networks with functional connectivity can help identify hubs that facilitate the spread of epileptiform activity. Surgical resection of these hubs may lead patients who suffer from drug-resistant epilepsy to seizure freedom. Here, we aim to map non-invasively epileptogenic networks, through the virtual implantation of sensors estimated with electric and magnetic source imaging, in patients with drug-resistant epilepsy. We hypothesize that highly connected hubs identified non-invasively with source imaging can predict the epileptogenic zone and the surgical outcome better than spikes localized with conventional source localization methods (dipoles). We retrospectively analysed simultaneous high-density electroencephalography (EEG) and magnetoencephalography data recorded from 37 children and young adults with drug-resistant epilepsy who underwent neurosurgery. Using source imaging, we estimated virtual sensors at locations where intracranial EEG contacts were placed. On data with and without spikes, we computed undirected functional connectivity between sensors/contacts using amplitude envelope correlation and phase locking value for physiologically relevant frequency bands. From each functional connectivity matrix, we generated an undirected network containing the strongest connections within sensors/contacts using the minimum spanning tree. For each sensor/contact, we computed graph centrality measures. We compared functional connectivity and their derived graph centrality of sensors/contacts inside resection for good (n = 22, ILAE I) and poor (n = 15, ILAE II-VI) outcome patients, tested their ability to predict the epileptogenic zone in good-outcome patients, examined the association between highly connected hubs removal and surgical outcome and performed leave-one-out cross-validation to support their prognostic value. We also compared the predictive values of functional connectivity with those of dipoles. Finally, we tested the reliability of virtual sensor measures via Spearman's correlation with intracranial EEG at population- and patient-level. We observed higher functional connectivity inside than outside resection (P < 0.05, Wilcoxon signed-rank test) for good-outcome patients, on data with and without spikes across different bands for intracranial EEG and electric/magnetic source imaging and few differences for poor-outcome patients. These functional connectivity measures were predictive of both the epileptogenic zone and outcome (positive and negative predictive values ≥55%, validated using leave-one-out cross-validation) outperforming dipoles on spikes. Significant correlations were found between source imaging and intracranial EEG measures (0.4 ≤ rho ≤ 0.9, P < 0.05). Our findings suggest that virtual implantation of sensors through source imaging can non-invasively identify highly connected hubs in patients with drug-resistant epilepsy, even in the absence of frank epileptiform activity. Surgical resection of these hubs predicts outcome better than dipoles.
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Epilepsia Refractaria , Epilepsia , Niño , Adulto Joven , Humanos , Estudios Retrospectivos , Reproducibilidad de los Resultados , Encéfalo , Electroencefalografía/métodos , Resultado del Tratamiento , Mapeo Encefálico , Imagen por Resonancia MagnéticaRESUMEN
Neurosurgical intervention is the best available treatment for selected patients with drug resistant epilepsy. For these patients, surgical planning requires biomarkers that delineate the epileptogenic zone, the brain area that is indispensable for the generation of seizures. Interictal spikes recorded with electrophysiological techniques are considered key biomarkers of epilepsy. Yet, they lack specificity, mostly because they propagate across brain areas forming networks. Understanding the relationship between interictal spike propagation and functional connections among the involved brain areas may help develop novel biomarkers that can delineate the epileptogenic zone with high precision. Here, we reveal the relationship between spike propagation and effective connectivity among onset and areas of spread and assess the prognostic value of resecting these areas. We analysed intracranial EEG data from 43 children with drug resistant epilepsy who underwent invasive monitoring for neurosurgical planning. Using electric source imaging, we mapped spike propagation in the source domain and identified three zones: onset, early-spread and late-spread. For each zone, we calculated the overlap and distance from surgical resection. We then estimated a virtual sensor for each zone and the direction of information flow among them via Granger causality. Finally, we compared the prognostic value of resecting these zones, the clinically-defined seizure onset zone and the spike onset on intracranial EEG channels by estimating their overlap with resection. We observed a spike propagation in source space for 37 patients with a median duration of 95 ms (interquartile range: 34-206), a spatial displacement of 14 cm (7.5-22 cm) and a velocity of 0.5 m/s (0.3-0.8 m/s). In patients with good surgical outcome (25 patients, Engel I), the onset had higher overlap with resection [96% (40-100%)] than early-spread [86% (34-100%), P = 0.01] and late-spread [59% (12-100%), P = 0.002], and it was also closer to resection than late-spread [5 mm versus 9 mm, P = 0.007]. We found an information flow from onset to early-spread in 66% of patients with good outcomes, and from early-spread to onset in 50% of patients with poor outcome. Finally, resection of spike onset, but not area of spike spread or the seizure onset zone, predicted outcome with positive predictive value of 79% and negative predictive value of 56% (P = 0.04). Spatiotemporal mapping of spike propagation reveals information flow from onset to areas of spread in epilepsy brain. Surgical resection of the spike onset disrupts the epileptogenic network and may render patients with drug resistant epilepsy seizure-free without having to wait for a seizure to occur during intracranial monitoring.
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Epilepsia Refractaria , Epilepsia , Niño , Humanos , Epilepsia Refractaria/diagnóstico por imagen , Epilepsia Refractaria/cirugía , Electroencefalografía/métodos , Epilepsia/cirugía , Convulsiones , Resultado del TratamientoRESUMEN
INTRODUCTION: Literature lacks studies investigating the cortical generation of sleep spindles in drug-resistant epilepsy (DRE) and how they evolve after resection of the epileptogenic zone (EZ). Here, we examined sleep EEGs of children with focal DRE who became seizure-free after focal epilepsy surgery, and aimed to investigate the changes in the spindle generation before and after the surgery using low-density scalp EEG and electrical source imaging (ESI). METHODS: We analyzed N2-sleep EEGs from 19 children with DRE before and after surgery. We identified slow (8-12 Hz) and fast spindles (13-16 Hz), computed their spectral features and cortical generators through ESI and computed their distance from the EZ and irritative zone (IZ). We performed two-way ANOVA testing the effect of spindle type (slow vs. fast) and surgical phase (pre-surgery vs. post-surgery) on each feature. RESULTS: Power, frequency and cortical activation of slow spindles increased after surgery (p < 0.005), while this was not seen for fast spindles. Before surgery, the cortical generators of slow spindles were closer to the EZ (57.3 vs. 66.2 mm, p = 0.007) and IZ (41.3 vs. 55.5 mm, p = 0.02) than fast spindle generators. CONCLUSIONS: Our data indicate alterations in the EEG slow spindles after resective epilepsy surgery. Fast spindle generation on the contrary did not change after surgery. Although the study is limited by its retrospective nature, lack of healthy controls, and reduced cortical spatial sampling, our findings suggest a spatial relationship between the slow spindles and the epileptogenic generators.
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Epilepsia Refractaria , Epilepsias Parciales , Epilepsia , Niño , Humanos , Estudios Retrospectivos , Epilepsia/diagnóstico por imagen , Epilepsia/cirugía , Epilepsia Refractaria/diagnóstico por imagen , Epilepsia Refractaria/cirugía , Sueño/fisiología , Electroencefalografía/métodosRESUMEN
OBJECTIVE: Intrathecal baclofen (ITB) pumps are commonly used in pediatric patients with cerebral palsy (CP) and medically refractory spasticity. However, catheter malfunction and associated risk factors are not well understood. The aim of this study was to examine potential risk factors for spinal catheter malfunction and characterize postoperative follow-up to understand the clinical consequences. METHODS: Patients who received ITB pump replacement or revision at Boston Children's Hospital between 2010 and 2023 were retrospectively reviewed. The spinal catheter revision cohort (SCRC) included patients whose spinal catheter was occluded requiring lumbar catheter revision. The second cohort included abdominal pump replacements only (APRC). Between-group comparisons and multivariable regression identified factors associated with catheter revision and postoperative outcomes. RESULTS: Forty-one (33.6%) patients underwent spinal catheter revision and were compared with 81 patients (66.4%) who underwent abdominal pump replacement only. Younger age at surgery and an elevated preoperative lower-extremity modified Ashworth scale grade were associated with spinal catheter revision (p < 0.05). Catheter model type, tip location, and history of spinal fusion were not associated with obstruction. Postoperatively, SCRC patients experienced a higher rate of infection (17.1%) relative to APRC patients (0%) within 30 days from their ITB pump replacement procedure (p < 0.05) and greater likelihood of subsequent ITB system removal compared with the APRC (24.4% vs 7.4%, p < 0.05). Although not differing preoperatively, SCRC patients had lower postoperative ITB doses when compared with the APRC group (median dose 143 vs 350 µg/day, p < 0.05) at hospital discharge and remained statistically different at the 6-month and 1-year follow-ups (p < 0.05). There were no postoperative differences in baclofen overdose, withdrawal, or median number of hospital readmissions within 30 days. Overall, 31.7% of spinal catheter revisions were unanticipated by the clinical team at time of surgery. CONCLUSIONS: Younger age at surgery and increased preoperative lower-extremity tone may be risk factors for catheter obstruction, resulting in a higher rate of postoperative infection and subsequent ITB pump removal compared with pump replacement alone. Spinal catheter occlusion can complicate revision or replacement procedures, especially when unanticipated. Routine clinical assessment may be inadequate for diagnosing insidious catheter malfunction. Catheter occlusion deserves further study, and routine assessment of catheter patency may be warranted to prevent suboptimal tone therapy.
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Baclofeno , Parálisis Cerebral , Bombas de Infusión Implantables , Relajantes Musculares Centrales , Humanos , Baclofeno/administración & dosificación , Baclofeno/efectos adversos , Masculino , Femenino , Niño , Bombas de Infusión Implantables/efectos adversos , Factores de Riesgo , Relajantes Musculares Centrales/administración & dosificación , Estudios Retrospectivos , Adolescente , Parálisis Cerebral/cirugía , Parálisis Cerebral/complicaciones , Preescolar , Espasticidad Muscular/tratamiento farmacológico , Espasticidad Muscular/etiología , Espasticidad Muscular/cirugía , Reoperación/métodos , Inyecciones Espinales/métodos , Resultado del Tratamiento , Complicaciones Posoperatorias/etiología , Falla de Equipo , Estudios de CohortesRESUMEN
OBJECTIVE: Minimally invasive magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been proposed as an alternative to open epilepsy surgery, to address concerns regarding the risk of open surgery. Our primary hypothesis was that seizure freedom at 1 year after MRgLITT is noninferior to open surgery in children with drug-resistant epilepsy (DRE). The secondary hypothesis was that MRgLITT has fewer complications and shorter hospitalization than surgery. The primary objective was to compare seizure outcome of MRgLITT to open surgery in children with DRE. The secondary objective was to compare complications and length of hospitalization of the two treatments. METHODS: This retrospective multicenter cohort study included children with DRE treated with MRgLITT or open surgery with 1-year follow-up. Exclusion criteria were corpus callosotomy, neurostimulation, multilobar or hemispheric surgery, and lesion with maximal dimension > 60 mm. MRgLITT patients were propensity matched to open surgery patients. The primary outcome was seizure freedom at 1 year posttreatment. The difference in seizure freedom was compared using noninferiority test, with noninferiority margin of -10%. The secondary outcomes were complications and length of hospitalization. RESULTS: One hundred eighty-five MRgLITT patients were matched to 185 open surgery patients. Seizure freedom at 1 year follow-up was observed in 89 of 185 (48.1%) MRgLITT and 114 of 185 (61.6%) open surgery patients (difference = -13.5%, one-sided 97.5% confidence interval = -23.8% to ∞, pNoninferiority = .79). The lower confidence interval boundary of -23.8% was below the prespecified noninferiority margin of -10%. Overall complications were lower in MRgLITT compared to open surgery (10.8% vs. 29.2%, respectively, p < .001). Hospitalization was shorter for MRgLITT than open surgery (3.1 ± 2.9 vs. 7.2 ± 6.1 days, p < .001). SIGNIFICANCE: Seizure outcome of MRgLITT at 1 year posttreatment was inferior to open surgery. However, MRgLITT has the advantage of better safety profile and shorter hospitalization. The findings will help counsel children and parents on the benefits and risks of MRgLITT and contribute to informed decision-making on treatment options.
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Epilepsia Refractaria , Terapia por Láser , Convulsiones , Niño , Humanos , Epilepsia Refractaria/cirugía , Epilepsia Refractaria/terapia , Terapia por Láser/métodos , Imagen por Resonancia Magnética , Estudios Retrospectivos , Convulsiones/prevención & control , Resultado del TratamientoRESUMEN
OBJECTIVE: Lennox-Gastaut syndrome (LGS) is a severe form of epileptic encephalopathy, presenting during the first years of life, and is very resistant to treatment. Once medical therapy has failed, palliative surgeries such as vagus nerve stimulation (VNS) or corpus callosotomy (CC) are considered. Although CC is more effective than VNS as the primary neurosurgical treatment for LGS-associated drop attacks, there are limited data regarding the added value of CC following VNS. This study aimed to assess the effectiveness of CC preceded by VNS. METHODS: This multinational, multicenter retrospective study focuses on LGS children who underwent CC before the age of 18 years, following prior VNS, which failed to achieve satisfactory seizure control. Collected data included epilepsy characteristics, surgical details, epilepsy outcomes, and complications. The primary outcome of this study was a 50% reduction in drop attacks. RESULTS: A total of 127 cases were reviewed (80 males). The median age at epilepsy onset was 6 months (interquartile range [IQR] = 3.12-22.75). The median age at VNS surgery was 7 years (IQR = 4-10), and CC was performed at a median age of 11 years (IQR = 8.76-15). The dominant seizure type was drop attacks (tonic or atonic) in 102 patients. Eighty-six patients underwent a single-stage complete CC, and 41 an anterior callosotomy. Ten patients who did not initially have a complete CC underwent a second surgery for completion of CC due to seizure persistence. Overall, there was at least a 50% reduction in drop attacks and other seizures in 83% and 60%, respectively. Permanent morbidity occurred in 1.5%, with no mortality. SIGNIFICANCE: CC is vital in seizure control in children with LGS in whom VNS has failed. Surgical risks are low. A complete CC has a tendency toward better effectiveness than anterior CC for some seizure types.
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Epilepsia , Síndrome de Lennox-Gastaut , Estimulación del Nervio Vago , Niño , Masculino , Humanos , Lactante , Preescolar , Adolescente , Síndrome de Lennox-Gastaut/cirugía , Estudios Retrospectivos , Cuerpo Calloso/cirugía , Convulsiones/terapia , Síncope , Resultado del Tratamiento , Nervio VagoRESUMEN
BACKGROUND: Epidural analgesia is commonly used for pain control after reconstructive hip surgery, but its use is controversial in the presence of an intrathecal baclofen pump (ITB). The purpose of this retrospective study was to investigate the rate of serious anesthetic and postoperative complications as well as the efficacy of epidural analgesia compared with lumbar plexus blocks (LPBs) for pain management after neuromuscular hip reconstruction in children with cerebral palsy (CP) and ITB. METHODS: Pediatric patients with CP and ITB undergoing hip reconstructive surgery from 2010 to 2019 were retrospectively identified. Patients receiving epidural analgesia were compared with those receiving LPB. Morphine milligram equivalents per kilogram were used as a surrogate measure for pain-related outcomes, as pain scores were reported with wide ranges (eg, 0 to 5/10), making it unfeasible to compare them across the cohort. Postoperative complications were graded using the modified Clavien-Dindo classification. RESULTS: Forty-four patients (26/44, 59% male) underwent surgery at an average age of 10.3 years (SD=3.4 y, range: 4 to 17 y). The majority utilized LPB (28/44, 64%) while the remaining utilized epidural (16/44, 36%). There were no differences in rates of serious complications, including no cases of ITB malfunction, damage, or infection. During the immediate postoperative course, patients who received LPB had higher morphine milligram equivalents per kilogram requirements than patients who received epidural analgesia. CONCLUSIONS: In patients with CP undergoing hip reconstruction surgery with an ITB in situ, epidural anesthesia was associated with improved analgesia compared with LPB analgesia, with a similar risk for adverse outcomes. Epidural catheters placed using image-guided insertion techniques can avoid damage to the ITB catheter while providing effective postoperative pain control without increasing rates of complications in this complex patient population. LEVEL OF EVIDENCE: Level III.
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Analgesia Epidural , Parálisis Cerebral , Baclofeno , Parálisis Cerebral/complicaciones , Parálisis Cerebral/cirugía , Niño , Femenino , Humanos , Plexo Lumbosacro , Masculino , Dolor Postoperatorio/tratamiento farmacológico , Dolor Postoperatorio/etiología , Estudios RetrospectivosRESUMEN
OBJECTIVE: Cathodal direct current stimulation (cDCS) induces long-term depression (LTD)-like reduction of cortical excitability (DCS-LTD), which has been tested in the treatment of epilepsy with modest effects. In part, this may be due to variable cortical neuron orientation relative to the electric field. We tested, in vivo and in vitro, whether DCS-LTD occurs throughout the cortical thickness, and if not, then whether drug-DCS pairing can enhance the uniformity of the cortical response and the cDCS antiepileptic effect. METHODS: cDCS-mediated changes in cortical excitability were measured in vitro in mouse motor cortex (M1) and in human postoperative neocortex, in vivo in mouse somatosensory cortex (S1), and in a mouse kainic acid (KA)-seizure model. Contributions of N-methyl-D-aspartate-type glutamate receptors (NMDARs) to cDCS-mediated plasticity were tested with application of NMDAR blockers (memantine/D-AP5). RESULTS: cDCS reliably induced DCS-LTD in superficial cortical layers, and a long-term potentiation (LTP)-like enhancement (DCS-LTP) was recorded in deep cortical layers. Immunostaining confirmed layer-specific increase of phospho-S6 ribosomal protein in mouse M1. Similar nonuniform cDCS aftereffects on cortical excitability were also found in human neocortex in vitro and in S1 of alert mice in vivo. Application of memantine/D-AP5 either produced a more uniform DCS-LTD throughout the cortical thickness or at least abolished DCS-LTP. Moreover, a combination of memantine and cDCS suppressed KA-induced seizures. INTERPRETATION: cDCS aftereffects are not uniform throughout cortical layers, which may explain the incomplete cDCS clinical efficacy. NMDAR antagonists may augment cDCS efficacy in epilepsy and other disorders where regional depression of cortical excitability is desirable. ANN NEUROL 2020;88:489-502.
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Corteza Cerebral/efectos de los fármacos , Corteza Cerebral/fisiopatología , Antagonistas de Aminoácidos Excitadores/farmacología , Depresión Sináptica a Largo Plazo/fisiología , Estimulación Transcraneal de Corriente Directa/métodos , Animales , Epilepsia/fisiopatología , Humanos , Depresión Sináptica a Largo Plazo/efectos de los fármacos , Masculino , Ratones , Ratones Endogámicos C57BLRESUMEN
BACKGROUND: Children with cerebral palsy often suffer from increased tone, which can be treated with intrathecal baclofen via implanted pump. Additionally, they often require major orthopedic surgery for hip reconstruction; however, the presence of an intrathecal baclofen pump is a relative contraindication to regional anesthesia due to concerns about damaging the intrathecal baclofen pump system. AIMS: (a) To evaluate adverse events related to placing epidural catheters in children with intrathecal baclofen pumps and (b) describe our multidisciplinary approach to the care of these complex patients. METHODS: Children with cerebral palsy and intrathecal baclofen pump in situ who underwent hip reconstruction between 2010 and 2019 and had a perioperative epidural placed were reviewed retrospectively. Charts were assessed for adverse events or intrathecal baclofen complications. Fluoroscopic images were reviewed to evaluate the proximity between epidural and intrathecal baclofen catheters. The process of coordinating multiple services was examined. RESULTS: Sixteen children met the inclusion criteria. There were no major complications following epidural placement. Postoperative pump interrogation was normal for all patients. Fluoroscopy was utilized for 9/16 (56%) epidural procedures. Epidurogram was used to confirm 11/16 catheters (68%). Children with an intrathecal baclofen pump were identified by orthopedic surgeons at the time of surgical booking and referred to the regional anesthesia team for review. The neurosurgical, pain, and regional anesthesia teams determined the appropriateness and safety of approaching the neuraxis. Pain and/or regional anesthesiologists with competency in spine fluoroscopy were scheduled on the day of surgery for fluoroscopically guided epidural placement. Postoperatively, catheters were managed by the acute pain team. Intrathecal baclofen pumps were interrogated by the baclofen pump team prior to patient discharge. DISCUSSION: In this case series, not only was epidural placement feasible but also there were no observed complications. This work highlights the importance of a multidisciplinary approach to complex regional anesthetic techniques, as well as the importance of basic competency in spine fluoroscopy for regional anesthesiologists.
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Parálisis Cerebral , Relajantes Musculares Centrales , Baclofeno , Catéteres , Parálisis Cerebral/complicaciones , Parálisis Cerebral/tratamiento farmacológico , Niño , Humanos , Bombas de Infusión Implantables , Inyecciones Espinales , Relajantes Musculares Centrales/uso terapéutico , Estudios RetrospectivosRESUMEN
BACKGROUND: Spinal administration of medications is challenging in patients with complete posterior spinal fusion. We describe percutaneous image-guided intrathecal port placement for administration of the antisense oligonucleotide nusinersen for children and young adults with spinal muscular atrophy. OBJECTIVE: To describe and present our initial experience with a new technique for administering nusinersen in patients with spinal muscular atrophy and posterior spinal fusion. MATERIALS AND METHODS: We reviewed medical records of 13 patients who received intrathecal ports using DynaCT, biplane fluoroscopy and iGuide from April 2018 through June 2019, and we describe the clinical course over 1 year. RESULTS: Image-guided catheter and port implantation was successful in all cases. Two ports were subsequently removed, one for persistent cerebrospinal fluid leak and one for superficial infection. The other 11 have functioned successfully for a minimum of 23 months. CONCLUSION: We report our experience with image-guided intrathecal port placement in children with complete posterior spine fusion. The implanted port permits dosing in an outpatient setting and avoids the need for multiple future radiologic procedures, and it reduces discomfort, procedural costs and potential risks and sequelae of multiple anesthetics and radiation exposures. Further studies are needed to define the relative risks and benefits of intrathecal ports compared to other approaches such as repeated transforaminal lumbar punctures.
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Atrofia Muscular Espinal , Fusión Vertebral , Catéteres , Niño , Humanos , Inyecciones Espinales , Atrofia Muscular Espinal/diagnóstico por imagen , Atrofia Muscular Espinal/tratamiento farmacológico , OligonucleótidosRESUMEN
PURPOSE OF REVIEW: Deep brain stimulation (DBS) has recently emerged as an important management option in children with medically refractory dystonia. DBS is most commonly used, best studied, and thought to be most efficacious for a select group of childhood or adolescent onset monogenic dystonias (designated with a standard 'DYT' prefix). We review how to clinically recognize these types of dystonia and the relative efficacy of DBS for key monogenic dystonias. RECENT FINDINGS: Though used for dystonia in adults for several years, DBS has only lately been used in children. Recent evidence shows that patients with shorter duration of dystonia often experience greater benefit following DBS. This suggests that early recognition of the appropriate dystonic phenotypes and consideration of DBS in these patients may improve the management of dystonia. SUMMARY: DBS should be considered early in patients who have medically refractory dystonia, especially for the monogenic dystonias that have a high response rate to DBS. It is important to differentiate between these monogenic dystonias and dystonias of other causes to properly prognosticate for these patients and to determine whether DBS is an appropriate management option.
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Estimulación Encefálica Profunda , Trastornos Distónicos/genética , Trastornos Distónicos/terapia , Proteínas Reguladoras de la Apoptosis/genética , Proteínas de Unión al ADN/genética , Marcadores Genéticos , Humanos , Chaperonas Moleculares/genética , Proteínas Nucleares/genética , ATPasa Intercambiadora de Sodio-Potasio/genética , Resultado del TratamientoAsunto(s)
Anestésicos , Terapia por Láser , Pediatría , Niño , Humanos , Rayos Láser , Imagen por Resonancia Magnética , ConvulsionesAsunto(s)
Accidentes por Caídas , Suministros de Energía Eléctrica , Lesiones Oculares/cirugía , Cuerpos Extraños/cirugía , Lesiones Oculares/diagnóstico por imagen , Lesiones Oculares/etiología , Cuerpos Extraños/diagnóstico por imagen , Humanos , Lactante , Masculino , Procedimientos Neuroquirúrgicos , Procedimientos Quirúrgicos OftalmológicosRESUMEN
BACKGROUND: Alzheimer's disease (AD) is a debilitating neurological illness of increasing prevalence. Because many patients are affected and current treatments have limited effectiveness, other therapeutic strategies are urgently needed. OBJECTIVES: Here we provide a review of the neurosurgical approaches that have been attempted or are currently being investigated for the treatment of AD. METHODS: Computerized database searches identified all of the published studies in the English-language literature examining the surgical treatment of AD since 1950. RESULTS: The following 5 categories of neurosurgical treatment were identified: cerebrospinal fluid shunting, intraventricular infusions, tissue grafting, gene therapy, and electrical neural stimulation. CONCLUSIONS: While none of the neurosurgical approaches applied to the treatment of AD have proven effective to date, recent trials involving gene therapy and electrical neural stimulation are showing promising early results. Larger trials investigating these treatments have been proposed or are currently under way.
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Enfermedad de Alzheimer/terapia , Encéfalo/cirugía , Derivaciones del Líquido Cefalorraquídeo , Terapia por Estimulación Eléctrica , Terapia Genética , Enfermedad de Alzheimer/cirugía , Humanos , Resultado del TratamientoRESUMEN
BACKGROUND: Indirect revascularization techniques for posterior circulation moyamoya are infrequently described in the literature given the relative rarity of the disease process, particularly in the best candidates for indirect approaches-children. METHODS: We present a detailed, illustrated video demonstration of the occipital pial synangiosis procedure performed in a 5-year-old child. CONCLUSION: The specific advantages of the pial synangiosis technique-suturing the donor vessel to the pia and a wide arachnoidal opening-can be successfully applied to posterior circulation indirect revascularization. KEY POINTS: ⢠Perioperative hydration and aspirin limit the risk of perioperative stroke. ⢠Pain control and maintained normocapnea limit the risk of perioperative ischemic events. ⢠The posterior auricular artery may be an alternative donor vessel. ⢠The donor vessel is dissected from distal to proximal; forceps protect it during skin incision. ⢠The donor vessel is dissected along a generous length to minimize tension. ⢠Coagulation of the dura is limited as meningeal vessels may serve as synergistic collaterals. ⢠A wide arachnoidal opening facilitates the ingrowth of collateral vessels. ⢠Suturing the donor vessel to the pia ensures it remains well apposed to the brain surface. ⢠The dura is left open to encourage ingrowth of collateral vessels and limit tension on the donor vessel. ⢠EEG monitoring is a crucial adjunct.
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Revascularización Cerebral/métodos , Enfermedad de Moyamoya/cirugía , Piamadre/irrigación sanguínea , Piamadre/cirugía , Preescolar , Circulación Colateral , Electroencefalografía , Humanos , Enfermedad de Moyamoya/complicaciones , Lóbulo Occipital , Accidente Cerebrovascular/prevención & control , Resultado del TratamientoAsunto(s)
Adenosina Desaminasa/deficiencia , Agammaglobulinemia/genética , Péptidos y Proteínas de Señalización Intercelular/deficiencia , Inmunodeficiencia Combinada Grave/genética , Adenosina Desaminasa/sangre , Adenosina Desaminasa/genética , Agammaglobulinemia/sangre , Preescolar , Femenino , Glicosilación , Humanos , Péptidos y Proteínas de Señalización Intercelular/sangre , Péptidos y Proteínas de Señalización Intercelular/genética , Mutación , Inmunodeficiencia Combinada Grave/sangreRESUMEN
PURPOSE: Stereoelectroencephalography (SEEG) is widely performed on individuals with medically refractory epilepsy for whom invasive seizure localization is desired. Despite increasing adoption in many centers across the world, no standardized electrode naming convention exists, generating confusion among both clinical and research teams. METHODS: We have developed a novel nomenclature, named the Standardized Electrode Nomenclature for SEEG Applications system. Concise, unique, informative, and unambiguous labels provide information about entry point, deep targets, and relationships between electrodes. Inter-rater agreement was evaluated by comparing original electrode names from 10 randomly sampled cases (including 136 electrodes) with those prospectively assigned by four additional blinded raters. RESULTS: The Standardized Electrode Nomenclature for SEEG Application system was prospectively implemented in 40 consecutive patients undergoing SEEG monitoring at our institution, creating unique electrode names in all cases, and facilitating implantation design, SEEG recording and mapping interpretation, and treatment planning among neurosurgeons, neurologists, and neurophysiologists. The inter-rater percent agreement for electrode names among two neurosurgeons, two epilepsy neurologists, and one neurosurgical fellow was 97.5%. CONCLUSIONS: This standardized naming convention, Standardized Electrode Nomenclature for SEEG Application, provides a simple, concise, reproducible, and informative method for specifying the target(s) and relative position of each SEEG electrode in each patient, allowing for successful sharing of information in both the clinical and research settings. General adoption of this nomenclature could pave the way for improved communication and collaboration between institutions.