Aim We performed a meta‐analysis with individual participant data of deep brain stimulation (DBS) for dystonia in children and young people. Method Three databases (PubMed, Embase, and Web of Science) were queried from January 1999 to August 2017 with no language restrictions to identify case studies and cohort studies reporting on pediatric patients (age ≤21y) with dystonia. The primary outcomes were changes in Burke‐Fahn‐Marsden (BFM) or Barry‐Albright Dystonia Scale scores. A mixed‐effects regression was used to identify associations between clinical covariates and outcomes. Results Of 2509 citations reviewed, 72 articles (321 children) were eligible. At last follow‐up (median 12mo, 25th centile=9.0; 75th centile=32.2), 277 (86.3%) patients showed improvement in dystonia, while 66.1 percent showed clinically significant (>20%) BFM Dystonia Rating Scale‐motor improvement. On multivariable hierarchical regression, older age at dystonia onset, inherited dystonia without nervous system pathology and idiopathic dystonia (vs inherited with nervous system pathology or acquired dystonia), and truncal involvement indicated a better outcome (p<0.05). Interpretation The data suggest that DBS is effective and should be considered in selected children with inherited or idiopathic dystonia. What this paper adds Deep brain stimulation is effective in selected children with inherited or idiopathic dystonia.

Although chronic vagus nerve stimulation (VNS) is an established treatment for medically-intractable childhood epilepsy, there is considerable heterogeneity in seizure response and little data are available to pre-operatively identify patients who may benefit from treatment. Since the therapeutic effect of VNS may be mediated by afferent projections to the thalamus, we tested the hypothesis that intrinsic thalamocortical connectivity is associated with seizure response following chronic VNS in children with epilepsy. Twenty-one children (ages 5–21 years) with medically-intractable epilepsy underwent resting-state fMRI prior to implantation of VNS. Ten received sedation, while 11 did not. Whole brain connectivity to thalamic regions of interest was performed. Multivariate generalized linear models were used to correlate resting-state data with seizure outcomes, while adjusting for age and sedation status. A supervised support vector machine (SVM) algorithm was used to classify response to chronic VNS on the basis of intrinsic connectivity. Of the 21 subjects, 11 (52%) had 50% or greater improvement in seizure control after VNS. Enhanced connectivity of the thalami to the anterior cingulate cortex (ACC) and left insula was associated with greater VNS efficacy. Within our test cohort, SVM correctly classified response to chronic VNS with 86% accuracy. In an external cohort of 8 children, the predictive model correctly classified the seizure response with 88% accuracy. We find that enhanced intrinsic connectivity within thalamocortical circuitry is associated with seizure response following VNS. These results encourage the study of intrinsic connectivity to inform neural network-based, personalized treatment decisions for children with intractable epilepsy.

The current study provides a comprehensive reference on the usage of social media in epilepsy. The number of online users interested in epilepsy is likely the highest among all neurological conditions. Surgery, as a method of treating refractory epilepsy, however, could be underrepresented on social media.

OBJECTIVEGilles de la Tourette syndrome (GTS) is a disorder characterized by motor and vocal tics. Although by definition the onset of GTS is before age 18 years, clinical trials of deep brain stimulation (DBS) have been conducted only in adults. Using individual participant data (IPD) meta-analysis methodology, the current study investigated the safety and efficacy of DBS as a treatment for GTS in children and youth.METHODSA systematic review with no date or language restrictions was performed according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement. Three electronic databases were searched: PubMed, EMBASE, and Web of Science. From 843 articles screened, the IPD of 58 children and youth (ages 12–21 years) extracted from 21 articles were collected and analyzed. A mixed-effects univariable analysis followed by multivariable hierarchical regression was performed using change in the Yale Global Tic Severity Scale (YGTSS) score as the primary outcome and reported measures of comorbidities as secondary outcomes.RESULTSThe authors’ results showed an average improvement of 57.5% ± 24.6% across studies on the YGTSS. They also found that comorbid depression and stimulation pulse width each correlated negatively with outcome (p < 0.05). In patients with less severe GTS, greater improvements were evident following thalamic stimulation. More than one-quarter (n = 16, 27.6%) of participants experienced side effects, the majority of which were minor.CONCLUSIONSDBS in the pediatric population may be an effective option with a moderate safety profile for treatment of GTS in carefully selected children and youth. Large, prospective studies with long-term follow-up are necessary to understand how DBS influences tic symptoms and may alter the natural course of GTS in children.

Dystonia, one of the most common childhood movement disorders, is often medically refractory and can lead to profound impacts on the child and their caretakers' quality of life. Limited efficacy of pharmacological treatments has fueled enthusiasm for innovative neurosurgical approaches, notably deep brain stimulation (DBS) as a treatment for refractory dystonia. Areas covered: DBS is increasingly applied to successfully treat childhood dystonia. While generally safe and effective, results vary widely depending on underlying dystonia etiology. The current work synthesizes and highlights advances in research pertaining to the use of DBS for childhood dystonia. The efficacy of DBS for children and youth with dystonia is discussed, with analysis divided among etiological subtypes. The role of DBS as a lifesaving treatment for status dystonicus is also reviewed. Expert commentary: When carefully selected, certain children and youth with dystonia experience significant symptomatic improvement after DBS. Beyond dystonic symptoms, DBS can improve quality of life and reduce caretaker burden.

OBJECTIVEAlthough hemispherectomy is an effective treatment for children with intractable hemispheric epilepsy syndromes, as many as 40% of patients eventually develop seizure recurrence. The causes of seizure recurrence in these patients are incompletely understood. The authors sought to evaluate the efficacy of hemispherectomy at their center and determine whether contralateral MRI abnormalities can predict seizure recurrence.METHODSA retrospective review of consecutive hemispherectomies performed at Miami Children’s Hospital between January 2000 and June 2014 was performed. Time-to-event analysis was performed. The “event” was defined as any seizures following resective epilepsy surgery (not including seizures in the first postoperative week and auras). Several preoperative variables were analyzed to determine their suitability to predict seizure recurrence following surgery.RESULTSSixty-nine patients (44 boys) with a mean age of 8.2 ± 5.9 years (range 0.1–20.8 years) underwent 72 hemispherectomies; 67 of these were functional hemispherectomies, while another 5 were completion of a previous functional hemispherectomy (2 completions of functional hemispherectomies, 3 anatomical hemispherectomies). The duration of epilepsy was 5.8 ± 5.5 years with 66 cases (91.7%) having daily seizures. Etiology included stroke (n = 28), malformation of cortical development (n = 11), hemimegalencephaly (n = 11), encephalitis (n = 13), and other (n = 7). Engel class I outcome was achieved in 59 (86%) and 56 (81%) patients at 1 and 2 years of follow-up, respectively. The mean time to seizure recurrence was 33.5 ± 31.1 months. In univariate analyses, the absence of contralateral abnormalities on MRI (HR 4.09, 95% CI 1.41–11.89, p = 0.009) was associated with a longer duration of seizure freedom. The presence of contralateral MRI abnormalities was associated with contralateral ictal seizures on preoperative scalp EEG (p = 0.002). Fifteen patients experienced 20 complications (20/72, 27.8%), including the development of hydrocephalus necessitating CSF diversion in 9 cases (13%), hygroma in 1, hemispheric edema in 1, aseptic meningitis in 2, postoperative hemorrhage in 2, infection in 2, ischemic stroke in 2, and blood transfusion–contracted hepatitis C in 1 case.CONCLUSIONSPatients with bihemispheric abnormalities, as evidenced by contralateral MRI abnormalities, have a higher risk of earlier seizure recurrence following functional hemispherectomy.

The reported success of ETV/CPC for infantile hydrocephalus is higher in sub-Saharan Africa than developed nations. Large long-term prospective multi-center observational studies addressing patient-important outcomes are required to further evaluate the efficacy and safety of this re-emerging procedure.

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