UNASSIGNED: To characterise the ophthalmic indications for, and ophthalmic efficacy of, transverse sinus stenting in adults with medically refractory idiopathic intracranial hypertension.
UNASSIGNED: A retrospective cohort study was undertaken on a single-author database of 226 successive patients with confirmed idiopathic intracranial hypertension (IIH). A total of 32 patients were identified who received a transverse sinus stent for medically refractory disease. This which was defined as visual threat and/or intolerance of maximal medical therapy. Patients with medically refractory disease proceeded to stenting, if found to have a significant transverse sinus stenosis gradient at catheter venography. Visual threat was quantified via the degree of papilledema on optical coherence tomography of the retinal nerve fibre layer, and via the visual field mean deviation. CSF opening pressure at lumbar puncture and cerebral venous sinus pressure measurements from catheter venography were correlated with the ophthalmic data, noting also intolerance of maximal medical therapy. Complications of stenting were fully assessed.
UNASSIGNED: Medically refractory IIH was found in 18% of the total cohort of IIH patients. 90% of those with medically refractory disease had a significant transverse sinus stenosis pressure gradient, and 80% proceeded to stenting. The intervention eliminated papilledema in 96% of stented patients, and allowed 81% to cease acetazolamide. The need for a further procedure was low at 6%, and the safety profile was favourable.
UNASSIGNED: Medically refractory disease in IIH is common (18%), and nearly always associated with a significant transverse sinus stenosis pressure gradient (90%). Endovascular stenting of the stenosis deserves wider uptake as a highly effective, safe, and usually definitive treatment. It safeguards vision by eliminating papilledema (96%), and allows most patients to cease acetazolamide (81%). By analogy with glaucoma, if acetazolamide is the prostaglandin of IIH and CSF diversion the emergency glaucoma filter, stenting is the minimally invasive glaucoma surgery.

UNASSIGNED: Many patients with cluster headache (CH) are inadequately controlled by current treatment options. Non-invasive vagus nerve stimulation (nVNS) is reported to be effective in the management of CH though some studies suggest that it is ineffective.
UNASSIGNED: To assess the safety and efficacy of nVNS in chronic cluster headache (CCH) patients.
UNASSIGNED: We prospectively analysed data from 40 patients with refractory CCH in this open-label, observational study. Patients were seen in tertiary headache clinics at the National Hospital for Neurology and Neurosurgery and trained to use nVNS as preventative therapy. Patients were reivewed at one month and then three-monthly from onset. The primary endpoint was number of patients achieving ≥50% reduction in attack frequency at 3  months. A meta-analysis of all published studies evaluating the efficacy of nVNS in CCH was also conducted. We searched MEDLINE and EMBASE for all studies investigating the use of nVNS as a preventive or adjunctive treatment for CCH with five or more participants. Combined mean difference and responder proportions with 95% confidence intervals (CI) were calculated from the included studies.
UNASSIGNED: 17/40 patients (43%) achieved ≥50% reduction in attack frequency at 3  months. There was a significant reduction in monthly attack frequency from a baseline of 124 (±67) attacks to 79 (±63) attacks in month 3 (mean difference 44.7; 95% CI 25.1 to 64.3; p < 0.001). In month 3, there was also a 1.2-point reduction in average severity from a baseline Verbal Rating Scale of 8/10 (95% CI 0.5 to 1.9; p = 0.001). Four studies, along with the present study, were deemed eligible for meta-analysis, which showed a responder proportion of 0.35 (95% CI 0.07 to 0.69, n = 137) and a mean reduction in headache frequency of 35.3 attacks per month (95% CI 11.0 to 59.6, n = 108), from a baseline of 105 (±22.7) attacks per month.
UNASSIGNED: This study highlights the potential benefit of nVNS in CCH, with significant reductions in headache frequency and severity. To better characterise the effect, randomised sham-controlled trials are needed to confirm the beneficial response of VNS reported in some, but not all, open-label studies.

Pediatric stereoelectroencephalography (SEEG) has been increasingly performed in the United States, with published literature being limited primarily to large single-center case series. The purpose of this study was to evaluate the experience of pediatric epilepsy centers, where the technique has been adopted in the last several years, via a multicenter case series studying patient demographics, outcomes, and complications.
A retrospective cohort methodology was used based on the STROBE criteria. ANOVA was used to evaluate for significant differences between the means of continuous variables among centers. Dichotomous outcomes were assessed between centers using a univariate and multivariate logistic regression.
A total of 170 SEEG insertion procedures were included in the study from 6 different level 4 pediatric epilepsy centers. The mean patient age at time of SEEG insertion was 12.3 ± 4.7 years. There was no significant difference between the mean age at the time of SEEG insertion between centers (p = 0.3). The mean number of SEEG trajectories per patient was 11.3 ± 3.6, with significant variation between centers (p < 0.001). Epileptogenic loci were identified in 84.7% of cases (144/170). Patients in 140 cases (140/170, 82.4%) underwent a follow-up surgical intervention, with 47.1% (66/140) being seizure free at a mean follow-up of 30.6 months. An overall postoperative hemorrhage rate of 5.3% (9/170) was noted, with patients in 4 of these cases (4/170, 2.4%) experiencing a symptomatic hemorrhage and patients in 3 of these cases (3/170, 1.8%) requiring operative evacuation of the hemorrhage. There were no mortalities or long-term complications.
As the first multicenter case series in pediatric SEEG, this study has aided in establishing normative practice patterns in the application of a novel surgical technique, provided a framework for anticipated outcomes that is generalizable and useful for patient selection, and allowed for discussion of what is an acceptable complication rate relative to the experiences of multiple institutions.

OBJECTIVE: Although 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.
METHODS: A 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.
RESULTS: Sixty-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.
CONCLUSIONS: Patients with bihemispheric abnormalities, as evidenced by contralateral MRI abnormalities, have a higher risk of earlier seizure recurrence following functional hemispherectomy.
BACKGROUND: EVD = external ventricular drain; MCD = malformation of cortical development; MEG = magnetoencephalography; PVWM = periventricular white matter; TTE = time-to-event; VPS = ventriculoperitoneal shunt.

Epilepsy is characterized as recurrent seizures, and it is one of the most prevalent disorders of the human nervous system. A large and diverse profile of different syndromes and conditions can cause perturbations in neural networks that are associated with epilepsy. Advances in neuroimaging and electrophysiological monitoring have enhanced our ability to localize the neuropathological lesions that alter the neural networks giving rise to epilepsy, whereas advances in surgical management have resulted in excellent seizure control in many patients following resections. Histopathologic study using a variety of special stains, molecular analysis, and functional studies of these resected tissues has facilitated the neuropathological characterization of these lesions. Here, we review the neuropathology of common structural lesions that cause epilepsy and are amenable to neurosurgical resection, such as hippocampal sclerosis, focal cortical dysplasia, and its associated principal lesions, including long-term epilepsy-associated tumors, as well as other malformations of cortical development and Rasmussen encephalitis.

Objectives: Standard management strategies for lowering intracranial pressure (ICP) in traumatic brain injury has been well-studied, but the use of lesser known interventions for ICP in subarachnoid hemorrhage (SAH) remains elusive. Searches were performed in PubMed and EBSCO Host to identify best available evidence for evaluation and management of medically refractory ICP in SAH. The role of standard management strategies such as head elevation, hyperventilation, mannitol and hypertonic saline as well as lesser known management such as sodium bicarbonate, indomethacin, tromethamine, decompressive craniectomy, decompressive laparotomy, hypothermia, and barbiturate coma are reviewed. We also included dose concentrations, dose frequency, infusion volume, and infusion rate for these lesser known strategies. Nonetheless, there is still a gap in the evidence to recommend optimal dosing, timing and its role in the improvement of outcomes but early diagnosis and appropriate management reduce adverse outcomes.

OBJECTIVE: Procedures to treat medically refractory trigeminal neuralgia (MRTN) include stereotactic radiosurgery (SRS) and microvascular decompression (MVD). The aim of this study was to compare outcomes of SRS versus MVD in the treatment of MRTN, with a subgroup focus on those being treated for the first time.
METHODS: Search strategy was performed using the PRISMA guidelines for article identification, screening, eligibility and inclusion. Relevant articles were identified from six electronic databases from their inception to June 2017. These articles were screened against established criteria for inclusion into this study. Meta-analysis was conducted by pooling results with odds ratios and subgroup analysis.
RESULTS: From 13 relevant studies identified, 683 patients treated by SRS were compared with 670 patients treated by MVD for MRTN. Overall, the significant findings were that compared to MVD, SRS was associated with lower rates of short-term (OR = 0.16; 95%CI = 0.11-0.22; p < .001) and long-term pain freedom (OR = 0.31; 95%CI = 0.22-0.44; p < .001), fewer postoperative complications (OR = 0.06; 95%CI = 0.02-0.16; p < .001), more facial numbness and dysesthesia (OR = 1.64; 95%CI = 1.08-2.49; p = .02), and more pain recurrence (OR = 2.28; 95%CI = 1.32-3.93; p = .003). These trends were all reflected in MRTN patients being treated for the first time.
CONCLUSIONS: Both SRS and MVD alleviate pain in MRTN patients. MVD results in superior rates of short- and long-term pain relief, facial numbness and dysesthesia control, and less recurrence amongst those in whom pain freedom was achieved, at the cost of greater postoperative complications when compared to SRS. Although no significant difference was found in terms of the need for retreatment surgery, there was a trend towards less procedures favoring MVD. First treatment by either technique represents the overall trends reported.

UNASSIGNED: Epilepsy is a chronic neurological disorder frequently requiring lifelong treatment. In 70% of epilepsy patients, seizures are well controlled by antiepileptic medications. About 30% of epilepsy patients remain refractory to medical treatments and may need surgical interventions for better seizure control. Unfortunately and not infrequently, surgical intervention is not feasible due to various reasons such as multiple seizure foci, not resectable focus because of eloquent cortex location, or inability to tolerate surgery due to ongoing concomitant medical conditions. Neurostimulation devices have provided possible seizure control for refractory epilepsy patients who are not candidates for surgical intervention. Among them, vagal nerve stimulation (VNS) has been the oldest, in use since 1997. VNS was followed by responsive nerve stimulation (RNS) after obtaining FDA approval in 2013. Deep brain stimulation (DBS) has not yet met approval in the USA, but has been in clinical practice in Europe since 2010. Neurostimulation devices vary in how they are inserted and their mechanisms of action. VNS has been easily accepted by patients since it is placed extracranially. By contrast, DBS and RNS require invasive procedures for intracranial implantation. As use of these devices will continue to increase in the foreseeable future, we aimed to contribute to the foundation for new research to expand on current knowledge and practice by reviewing the current status of the literature.

The decision to operate on the frontal sinus is based on persistent symptoms that have been refractory to appropriate medical therapy with associated radiographic evidence of disease by computed tomography. There is currently no evidence to support operating on radiographically negative frontal sinuses, regardless of the availability of technology or site of service options. There are many surgical procedures as well as a variety of different technologies available for the treatment of symptomatic, medically refractory frontal sinus disease. Balloon catheter dilation can be performed safely in an office setting with outcomes comparable to those in traditional operating room settings.

OBJECTIVE: Up to 14% of children with epilepsy continue to experience seizures despite having appropriate medical therapy and develop medically refractory epilepsy (MRE). Assessing clinical outcomes and therapeutic efficacy in children with MRE undergoing palliative epilepsy surgery has been challenging because of the lack of a quantitative instrument capable of estimating the clinical status of these patients. The ideal instrument would at once consider seizure control, neurodevelopment, caregiver burden, and quality of life. The purpose of this study was to develop and pilot the Pediatric Refractory Epilepsy Questionnaire (PREQ), a quantitative instrument to assess the severity and individual burden of epilepsy in children with MRE undergoing palliative epilepsy treatments.
METHODS: The caregivers of 25 patients with MRE completed the PREQ and the Quality of Life in Childhood Epilepsy (QOLCE) measure and participated in a semistructured interview. Medical records of the patients were reviewed, an Early Childhood Epilepsy Severity Scale (E-CHESS) score was calculated, and a Global Assessment of Severity of Epilepsy (GASE) score was obtained for each patient.
RESULTS: The initial PREQ was modified based on the analysis of responses, association with previously validated scales, comments from caregivers, and expertise of the PREQ panelists. Pediatric Refractory Epilepsy Questionnaire subscale scores were calculated based on clinical paradigm and compared with independent measures of seizure severity and quality of life. Significant correlations were observed between the seizure severity subscale and the GASE score (r=0.55) and between the mood subscale and the well-being score (r=0.61) on the QOLCE. Significant correlations were also observed between the caregiver rating of seizure severity and the GASE score (r=0.53), the social activity score (r=0.57), and the behavior score (r=0.43) on the QOLCE. Correlations between the caregiver rating of quality of life and the quality of life score (r=0.58) and the number of AEDs used (r=0.45) were also significant.
CONCLUSIONS: This pilot study is an initial, critical step in the development of the PREQ. The significant correlations between the PREQ subscales and the external epilepsy severity and quality of life measures lend preliminary support to our hypothesis that the PREQ is assessing the severity of epilepsy along with other important domains, such as mood, neurodevelopment, and quality of life. A larger prospective study of this modified PREQ is currently underway to further develop the PREQ.