Detethering of MRI-Demonstrated Tethered Cord Syndrome.

INTRODUCTION: Recognition of tethered cord syndrome (TCS) in children is important because as the child grows, the spinal cord is stretched, leading to ischemia and subsequent neurological deficits, including bowel or bladder dysfunction, back and leg pain, or lower extremity weakness. Imaging findings raising concerns for tethering include presence of a fatty and/or thickened filum or a conus medullaris located caudal to L2. OBJECTIVES: The objective of this study was to describe the symptomatic presentation of TCS in our institution, detect demographic and diagnostic predictors of signs and symptoms, assess changes in symptoms over time, and examine whether demographic and diagnostic variables affect changes in symptoms over time. METHODS: Using a retrospective chart review from patients who underwent detethering at our institution between April 1, 2015 and March 31, 2019, we report on the signs and symptoms of patients undergoing detethering surgery at presentation and examine possible demographic and diagnostic predictors of those symptoms and changes in symptoms over time. Logistic regression analyses were used to determine whether symptoms at presentation were related to demographic and diagnostic predictors and to assess a change in symptoms over time. RESULTS: A total of 273 patients underwent detethering and were analyzed. Of these, 144 (53%) were <5 years of age, 151 (55%) were male, 233 (85%) had a fatty filum, 179 (66%) had a thickened filum, and 106 (39%) had a low-lying conus. Patients <5 years of age were less likely to have urological, gastrointestinal, and neurological or orthopedic symptoms; patients with thickened fila (i.e., greater than 2 mm in diameter regardless of fat-infiltration) were less likely to have urological symptoms; and patients with low-lying coni were less likely to have gastrointestinal symptoms. Patients exhibiting symptoms at presentation had reduced rates of symptoms at the follow-up, but a reduction in rates of symptoms over time was unrelated to demographic or diagnostic variables. Surprisingly, 123 (45%) patients presented with intractable constipation. CONCLUSION: In the population studied, several presenting symptoms, particularly constipation, were commonly reported. Children <5 years old were less likely to manifest clinically evident neurological/orthopedic, urological, and gastrointestinal symptoms than the older cohorts. Patients were less likely to report symptoms at both their first and second postoperative visit compared to presentation.

Evaluation of pediatric glioma outcomes using intraoperative MRI: a multicenter cohort study.

BACKGROUND: The use of intraoperative MRI (iMRI) during treatment of gliomas may increase extent of resection (EOR), decrease need for early reoperation, and increase progression-free and overall survival, but has not been fully validated, particularly in the pediatric population. OBJECTIVE: To assess the accuracy of iMRI to identify residual tumor in pediatric patients with glioma and determine the effect of iMRI on decisions for resection, complication rates, and other outcomes. METHODS: We retrospectively analyzed a multicenter database of pediatric patients (age ≤ 18 years) who underwent resection of pathologically confirmed gliomas. RESULTS: We identified 314 patients (mean age 9.7 ± 4.6 years) with mean follow-up of 48.3 ± 33.6 months (range 0.03-182.07 months) who underwent surgery with iMRI. There were 201 (64.0%) WHO grade I tumors, 57 (18.2%) grade II, 24 (7.6%) grade III, 9 (2.9%) grade IV, and 23 (7.3%) not classified. Among 280 patients who underwent resection using iMRI, 131 (46.8%) had some residual tumor and underwent additional resection after the first iMRI. Of the 33 tissue specimens sent for pathological analysis after iMRI, 29 (87.9%) showed positive tumor pathology. Gross total resection was identified in 156 patients (55.7%), but this was limited by 69 (24.6%) patients with unknown EOR. CONCLUSIONS: Analysis of the largest multicenter database of pediatric gliomas resected using iMRI demonstrated additional tumor resection in a substantial portion of cases. However, determining the impact of iMRI on EOR and outcomes remains challenging because iMRI use varies among providers nationally. Continued refinement of iMRI techniques for use in pediatric patients with glioma may improve outcomes.

Clinically Significant Visual Deficits after Laser Interstitial Thermal Therapy for Mesiotemporal Epilepsy.

BACKGROUND: Laser interstitial thermal therapy (LITT) has recently gained popularity as a minimally invasive surgical option for the treatment of mesiotemporal epilepsy (mTLE). Similar to traditional open procedures for epilepsy, the most frequent neurological complications of LITT are visual deficits; however, a critical analysis of these injuries is lacking. OBJECTIVES: To evaluate the visual deficits that occur after LITT for mTLE and their etiology. METHOD: We surveyed five academic epilepsy centers that regularly perform LITT for cases of self-reported postoperative visual deficits. For these patients all pre-, intra- and postoperative MRIs were co-registered with an anatomic atlas derived from 7T MRI data. This was used to estimate thermal injury to early visual pathways and measure imaging variables relevant to the LITT procedure. Using logistic regression, we then compared 14 variables derived from demographics, mesiotemporal anatomy, and the surgical procedure for the patients with visual deficits to a normal cohort comprised of the first 30 patients to undergo this procedure at a single institution. RESULTS: Of 90 patients that underwent LITT for mTLE, 6 (6.7%) reported a postoperative visual deficit. These included 2 homonymous hemianopsias (HHs), 2 quadrantanopsias, and 2 cranial nerve (CN) IV palsies. These deficits localized to the posterior aspect of the ablation, corresponding to the hippocampal body and tail, and tended to have greater laser energy delivered in that region than the normal cohort. The patients with HH had insult localized to the lateral geniculate nucleus, which was -associated with young age and low choroidal fissure CSF volume. Quadrantanopsia, likely from injury to the optic radiation in Meyer's loop, was correlated with a lateral trajectory and excessive energy delivered at the tail end of the ablation. Patients with CN IV injury had extension of contrast to the tentorial edge associated with a mesial laser trajectory. CONCLUSIONS: LITT for epilepsy may be complicated by various classes of visual deficit, each with distinct etiology and clinical significance. It is our hope that by better understanding these injuries and their mechanisms we can eventually reduce their occurrence by identifying at-risk patients and trajectories and appropriately tailoring the ablation procedure.

Duodenal penetration of an inferior vena cava filter: case report and literature review.

The insertion of inferior vena cava filters (IVCF) is a well-known therapy used in the prevention of pulmonary embolism (PE). The incidence of IVCF-related complications is low and complete caval penetration of a filter with adjacent organ injury has a reported incidence of 0­1%. We report the case of an 18-year-old male who sustained a spinal cord injury after a motor vehicle crash. The patient received a prophylactic IVCF and subsequently presented with right flank pain, postprandial nausea, and vomiting. His exam was benign and a computed tomography scan revealed extra-caval penetration of the filter with struts within the duodenal lumen and psoas muscle. The patient underwent an exploratory laparotomy with extraction of the filter, inferior vena cava venorrhaphy, and repair of the duodenal injury. This complication illustrates the potential morbidity of a common procedure and emphasizes the importance of investigating the IVCF as a possible source of abdominal pain.

Magnetic resonance imaging-guided stereotactic laser ablation therapy for the treatment of pediatric epilepsy: a retrospective multiinstitutional study.

OBJECTIVE: The authors of this study evaluated the safety and efficacy of stereotactic laser ablation (SLA) for the treatment of drug-resistant epilepsy (DRE) in children. METHODS: Seventeen North American centers were enrolled in the study. Data for pediatric patients with DRE who had been treated with SLA between 2008 and 2018 were retrospectively reviewed. RESULTS: A total of 225 patients, mean age 12.8 ± 5.8 years, were identified. Target-of-interest (TOI) locations included extratemporal (44.4%), temporal neocortical (8.4%), mesiotemporal (23.1%), hypothalamic (14.2%), and callosal (9.8%). Visualase and NeuroBlate SLA systems were used in 199 and 26 cases, respectively. Procedure goals included ablation (149 cases), disconnection (63), or both (13). The mean follow-up was 27 ± 20.4 months. Improvement in targeted seizure type (TST) was seen in 179 (84.0%) patients. Engel classification was reported for 167 (74.2%) patients; excluding the palliative cases, 74 (49.7%), 35 (23.5%), 10 (6.7%), and 30 (20.1%) patients had Engel class I, II, III, and IV outcomes, respectively. For patients with a follow-up ≥ 12 months, 25 (51.0%), 18 (36.7%), 3 (6.1%), and 3 (6.1%) had Engel class I, II, III, and IV outcomes, respectively. Patients with a history of pre-SLA surgery related to the TOI, a pathology of malformation of cortical development, and 2+ trajectories per TOI were more likely to experience no improvement in seizure frequency and/or to have an unfavorable outcome. A greater number of smaller thermal lesions was associated with greater improvement in TST. Thirty (13.3%) patients experienced 51 short-term complications including malpositioned catheter (3 cases), intracranial hemorrhage (2), transient neurological deficit (19), permanent neurological deficit (3), symptomatic perilesional edema (6), hydrocephalus (1), CSF leakage (1), wound infection (2), unplanned ICU stay (5), and unplanned 30-day readmission (9). The relative incidence of complications was higher in the hypothalamic target location. Target volume, number of laser trajectories, number or size of thermal lesions, or use of perioperative steroids did not have a significant effect on short-term complications. CONCLUSIONS: SLA appears to be an effective and well-tolerated treatment option for children with DRE. Large-volume prospective studies are needed to better understand the indications for treatment and demonstrate the long-term efficacy of SLA in this population.

Temporal lobe pleomorphic xanthoastrocytoma and acquired BRAF mutation in an adolescent with the constitutional 22q11.2 deletion syndrome.

DiGeorge syndrome, or velocardiofacial syndrome (DGS/VCFS), is a rare and usually sporadic congenital genetic disorder resulting from a constitutional microdeletion at chromosome 22q11.2. While rare cases of malignancy have been described, likely due to underlying immunodeficiency, central nervous system tumors have not yet been reported. We describe an adolescent boy with DGS/VCFS who developed a temporal lobe pleomorphic xanthoastrocytoma. High-resolution single nucleotide polymorphism array studies of the tumor confirmed a constitutional 22q11.21 deletion, and revealed acquired gains, losses and copy number neutral loss of heterozygosity of several chromosomal regions, including a homozygous deletion of the CDKN2A/B locus. The tumor also demonstrated a common V600E mutation in the BRAF oncogene. This is the first reported case of a patient with DiGeorge syndrome developing a CNS tumor of any histology and expands our knowledge about low-grade CNS tumor molecular genetics.

Palliative functional hemispherectomy for treatment of refractory status epilepticus associated with Alpers' disease.

PURPOSE: Palliative epilepsy surgery is considered for patients that would benefit from surgical therapy for intractable epilepsy but are not candidates for curative procedures. In many cases, the goals of therapy focus on improved quality of life more than seizure freedom. We discuss the use of epilepsy surgery for refractory status epilepticus, as well as the rationale and ethical considerations for employing a palliative procedure in otherwise fatal diseases. METHODS: We present a child with Alpers' disease presenting with refractory status epilepticus which was treated with functional hemispherectomy after failure of multiple typical therapies. Hemispherectomy allowed for the child to be extubated and ultimately discharged to home with her family. Unfortunately, the child died several months later after developing new-onset liver failure in the setting of a viral illness. CONCLUSION: Functional hemispherectomy was effective for the treatment of refractory status epilepticus in Alpers' disease. We believe that the procedure resulted in improved quality of life which was the primary outcome goal. Palliative procedures should be considered in diseases with ultimate fatal outcome when the short-term benefits outweigh the risks. The ethical aspects of treatment must be carefully considered to insure treatment is provided in the best interest of the patient.

Queer Students, International Service-Learning, and Inclusivity: Insights into Identity, Activism, and Learning.

This qualitative study examines the experiences of LGBTQ students involved in international service-learning, exploring the possibilities, positive impacts, and complexities of navigating LGBTQ identities while participating in international service-learning. Analysis of their experiences yielded three themes that played an important role shaping their experiences: balancing their stance as learners and as activists, viewing their LGBTQ identities as assets as well as objects of structural barriers, and navigating language to make sense of their context and make themselves understood. These findings have implications for how universities and community partners prepare, train, and support students before, during and after their experiences abroad.

Early implementation of stereoelectroencephalography in children: a multiinstitutional case series.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Magnetic resonance-guided stereotactic laser ablation therapy for the treatment of pediatric brain tumors: a multiinstitutional retrospective study.

OBJECTIVE: This study aimed to assess the safety and efficacy of MR-guided stereotactic laser ablation (SLA) therapy in the treatment of pediatric brain tumors. METHODS: Data from 17 North American centers were retrospectively reviewed. Clinical, technical, and radiographic data for pediatric patients treated with SLA for a diagnosis of brain tumor from 2008 to 2016 were collected and analyzed. RESULTS: A total of 86 patients (mean age 12.2 ± 4.5 years) with 76 low-grade (I or II) and 10 high-grade (III or IV) tumors were included. Tumor location included lobar (38.4%), deep (45.3%), and cerebellar (16.3%) compartments. The mean follow-up time was 24 months (median 18 months, range 3-72 months). At the last follow-up, the volume of SLA-treated tumors had decreased in 80.6% of patients with follow-up data. Patients with high-grade tumors were more likely to have an unchanged or larger tumor size after SLA treatment than those with low-grade tumors (OR 7.49, p = 0.0364). Subsequent surgery and adjuvant treatment were not required after SLA treatment in 90.4% and 86.7% of patients, respectively. Patients with high-grade tumors were more likely to receive subsequent surgery (OR 2.25, p = 0.4957) and adjuvant treatment (OR 3.77, p = 0.1711) after SLA therapy, without reaching significance. A total of 29 acute complications in 23 patients were reported and included malpositioned catheters (n = 3), intracranial hemorrhages (n = 2), transient neurological deficits (n = 11), permanent neurological deficits (n = 5), symptomatic perilesional edema (n = 2), hydrocephalus (n = 4), and death (n = 2). On long-term follow-up, 3 patients were reported to have worsened neuropsychological test results. Pre-SLA tumor volume, tumor location, number of laser trajectories, and number of lesions created did not result in a significantly increased risk of complications; however, the odds of complications increased by 14% (OR 1.14, p = 0.0159) with every 1-cm3 increase in the volume of the lesion created. CONCLUSIONS: SLA is an effective, minimally invasive treatment option for pediatric brain tumors, although it is not without risks. Limiting the volume of the generated thermal lesion may help decrease the incidence of complications.

Preservation of a subcutaneous pocket for vagus nerve stimulation pulse generator during magnetoencephalography. Technical note.

Patients with epilepsy and an implanted vagus nerve stimulation (VNS) device who are referred for consideration of definitive epilepsy surgery (removal of the epileptogenic cortex) may require magnetoencephalography (MEG), a study requiring explantation of the pulse generator, as part of their evaluation. Nonetheless, these patients may not wish to abandon palliative VNS therapy should definitive surgery prove unsuccessful or impossible. To avoid obliteration of the pocket by scar tissue after the pulse generator is explanted, the authors have preserved the dead space in several patients with insertion of a similarly sized silicone block. This block is easily replaced with the pulse generator if continued VNS therapy is appropriate, and is left in place in patients who appear to no longer require VNS therapy. Upon completion of MEG, if pulse generator replacement proves desirable, atraumatic retrieval of the electrode connector pin and body is easy. Silicone block implantation during what may prove to be temporary device explantation facilitates reuse of the original pulse generator implantation site and atraumatic distal electrode wire retrieval.

Coregistration of multimodal imaging is associated with favourable two-year seizure outcome after paediatric epilepsy surgery.

Multimodal coregistration uses multiple image datasets coregistered to an anatomical reference (i.e. MRI), allowing multiple studies to be viewed together. Commonly used in intractable epilepsy evaluation and generally accepted to improve localization of the epileptogenic zone, data showing that coregistration improves outcome is lacking. We compared seizure freedom following epilepsy surgery in paediatric patients, evaluated before and after the use of coregistration protocols at our centre, to determine whether this correlated with a change in outcome. We included paediatric epilepsy surgery patients with at least one anatomical and one functional neuroimaging study as part of their presurgical evaluation. Preoperatively designated palliative procedures and repeat surgeries were excluded. Multiple pre-, peri-, and postoperative variables were compared between groups with the primary outcome of seizure freedom. In total, 115 were included with an average age of 10.63 years (0.12-20.7). All evaluations included video-EEG (VEEG) and MRI. Seven (6%) had subtraction single-photon emission CT (SPECT), 46 (40%) had positron emission tomography (PET), and 62 (54%) had both as part of their evaluation. Sixty (52%) had extratemporal epilepsy and 25 (22%) were MRI-negative. Sixty-eight (59%) had coregistration. Coregistered patients were less likely to undergo invasive EEG monitoring (p=0.045) and were more likely to have seizure freedom at one (p=0.034) and two years (p<0.001) post-operatively. A logistic regression accounting for multiple covariates supported an association between the use of coregistration and favourable post-surgical outcome. Coregistered imaging contributes to favourable postoperative seizure reduction compared to visual analysis of individual modalities. Imaging coregistration is associated with improved outcome, independent of other variables after surgery. Coregistered imaging may reduce the need for invasive EEG monitoring, likely due to improved confidence in presurgical localization. These findings support the use of multimodal coregistered imaging as part of the presurgical assessment in patients evaluated for surgical treatment of intractable epilepsy.

Magnetic resonance imaging-guided laser interstitial thermal therapy as treatment for intractable insular epilepsy in children.

OBJECTIVE Seizure onset within the insula is increasingly recognized as a cause of intractable epilepsy. Surgery within the insula is difficult, with considerable risks, given the rich vascular supply and location near critical cortex. MRI-guided laser interstitial thermal therapy (LiTT) provides an attractive treatment option for insular epilepsy, allowing direct ablation of abnormal tissue while sparing nearby normal cortex. Herein, the authors describe their experience using this technique in a large cohort of children undergoing treatment of intractable localization-related epilepsy of insular onset. METHODS The combined epilepsy surgery database of Cook Children's Medical Center and Dell Children's Hospital was queried for all cases of insular onset epilepsy treated with LiTT. Patients without at least 6 months of follow-up data and cases preoperatively designated as palliative were excluded. Patient demographics, presurgical evaluation, surgical plan, and outcome were collected from patient charts and described. RESULTS Twenty patients (mean age 12.8 years, range 6.1-18.6 years) underwent a total of 24 LiTT procedures; 70% of these patients had normal findings on MRI. Patients underwent a mean follow-up of 20.4 months after their last surgery (range 7-39 months), with 10 (50%) in Engel Class I, 1 (5%) in Engel Class II, 5 (25%) in Engel Class III, and 4 (20%) in Engel Class IV at last follow-up. Patients were discharged within 24 hours of the procedure in 15 (63%) cases, in 48 hours in 6 (24%) cases, and in more than 48 hours in the remaining cases. Adverse functional effects were experienced following 7 (29%) of the procedures: mild hemiparesis after 6 procedures (all patients experienced complete resolution or had minimal residual dysfunction by 6 months), and expressive language dysfunction after 1 procedure (resolved by 3 months). CONCLUSIONS To their knowledge, the authors present the largest cohort of pediatric patients undergoing insular surgery for treatment of intractable epilepsy. The patient outcomes suggest that LiTT can successfully treat intractable seizures originating within the insula and offers an attractive alternative to open resection. This is the first description of LiTT applied to insular epilepsy and represents one of only a few series describing the use of LiTT in children. The results indicate that seizure reduction after LiTT compares favorably to that after conventional open surgical techniques.

Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma.

We present 8 examples of a neoplasm with features of both astrocytoma and ependymoma that may represent a distinct clinicopathologic entity. The cerebral hemispheric tumors occurred in patients that were 3, 4, 12, 14, 15, 26, 30, and 37 years of age. All presented with seizures that, with the exception of 2, began in childhood. Magnetic resonance imaging studies showed ill-defined, T2-hyperintense, generally noncontrast-enhancing lesions that, although centered on the cortex or amygdala, extended into the underlying white matter for a short distance. Histologically, the variably infiltrative tumors were distinctively angiocentric with well-developed perivascular pseudorosettes in some cases. Longitudinal and/or circumferential orientations of perivascular cells were common also. The cells were uniform in their cytologic features from case to case and were bipolar in all but one case. A glial nature was inferred from immunoreactivity for GFAP, and ependymal differentiation was suggested by positivity for EMA in three cases and ultrastructural features in one. Overall, the tumors were biologically indolent except for one that recurred and ultimately proved fatal.

Seasonal variations in posttraumatic wound infections after open extremity fractures.

BACKGROUND: The vast majority of the orthopedic trauma literature has dealt with risk of infection as a function of time to debridement and severity of open fracture. The goal of this analysis was to determine if either the incidence or causative organism of posttraumatic infection varies with the season in which the open fracture occurred. No such study has been previously published. METHODS: This is a retrospective chart review of all skeletally mature patients sustaining an open fracture of either the upper or the lower extremity long bones from 2007 to 2012. Charts were reviewed to extract information regarding date of injury, Gustilo-Anderson grade of open fracture, Injury Severity Score (ISS), time to surgical debridement, any posttraumatic wound infection (deep or superficial), and the causative organisms. Patients were placed into one of four groups based on the time of year: spring (March to May), summer (June to August), fall (September to November), and winter (December to February). Patients were excluded if they were skeletally immature, smokers, or also diagnosed with a condition that would increase risk of infection. Statistical analysis was performed to assess whether any observed differences were of significance. RESULTS: All four groups were similar with respect to Gustilo grade, ISS, and time to surgical debridement. A total of 1,128 open fractures were treated between 2007 and 2012. There were 58 total infections for an overall incidence of 4.3%. The incidence of infection based on season was 6.0% for spring, 4.9% for summer, 2.8% for fall, and 3.6% for winter (p < 0.05). The incidence of infection was 5.5% for spring and summer combined and 3.9% for winter and fall combined (p < 0.05). Gram-positive organisms were more prevalent in the spring and summer seasons (p < 0.05). Although gram-negative organisms were cultured more commonly in the fall and winter seasons (52%) compared with the spring and summer seasons (36%), they were evenly distributed with gram-positive organisms during the winter and fall. CONCLUSION: A seasonal variation exists regarding the incidence of infection and causative organisms for posttraumatic wound infection following open extremity fractures. The incidence of infection is significantly higher in the spring and summer months with a preponderance of gram-positive organisms. Patients with infection after injuries sustained in the fall and winter months are more likely to have a Gustilo Grade 3 injury and gram-negative bacteria as the causative organism. LEVEL OF EVIDENCE: Epidemiologic study, level III.

Epilepsy surgery for early infantile epileptic encephalopathy (ohtahara syndrome).

Early infantile epileptic encephalopathy or Ohtahara syndrome is the earliest form of the age-dependent epileptic encephalopathies. Its manifestations include tonic spasms, focal motor seizures, suppression burst pattern, pharmaco-resistance, and dismal prognosis. The purpose of this study was to evaluate the effectiveness of epilepsy surgery in selected infants. We identified 11 patients, 9 from the literature and 2 from our institution that fulfilled diagnostic criteria of Ohtahara syndrome and had undergone epilepsy surgery in infancy. Seven of the 11 infants have remained seizure free (Engel class IA) and four are reportedly having rare to infrequent seizures (Engel class IIB). All patients experienced "catch up" development. In contrast to Ohtahara's15 pharmacotherapy managed patients, who had a mortality rate of approximately fifty percent, and those that survived continued to have seizures and were severely impaired, the outcome of selected surgically managed patients is much more favorable.

Atypical imaging evolution of sturge-weber syndrome without facial nevus.

We report a patient with Sturge-Weber syndrome without facial angioma, who presented with seizures and normal initial imaging results. The patient experienced several years without seizures before a sudden increase in seizure frequency, followed by an atypical evolution of imaging findings prompting biopsy to establish the diagnosis. This case highlights not only the rare presentation of isolated leptomeningeal angiomatosis, but also the potential for atypical evolution of imaging findings through the course of the disease. We detail the imaging findings of our case and review the potential pathophysiological basis for this appearance. Our experience suggests that repeat imaging is warranted in patients with suspected Sturge-Weber syndrome or those with intractable cryptogenic epilepsy, because some imaging features of Sturge-Weber syndrome may manifest over time.

Lorcaserin (APD356), a selective 5-HT(2C) agonist, reduces body weight in obese men and women.

Lorcaserin (APD356) is a potent, selective 5-HT(2C) agonist with ~15-fold and 100-fold selectivity vs. 5-HT(2A) and 5-HT(2B) receptors, respectively. This study evaluated the safety and efficacy of lorcaserin for weight reduction in obese patients during a 12-week period. The randomized, double-blind, placebo-controlled, parallel-arm study enrolled 469 men and women between ages 18 and 65 and with BMI 30-45 kg/m(2). Patients received placebo, lorcaserin 10 mg q.d., lorcaserin 15 mg q.d., or lorcaserin 10 mg b.i.d. for 12 weeks, and were counseled to maintain their usual diet and activity. The primary end point was change in weight from baseline to day 85 by completer analysis. Safety analyses included echocardiograms at Screening and day 85/study exit. Lorcaserin was associated with progressive weight loss of 1.8 kg, 2.6 kg, and 3.6 kg at 10 mg q.d., 15 mg q.d., and 10 mg b.i.d., respectively, compared to placebo weight loss of 0.3 kg (P < 0.001 for each group). Similar results were seen by intent-to-treat last observation-carried forward (ITT-LOCF) analysis. The proportions of completers achieving > or =5% of initial body weight were 12.8, 19.5, 31.2, and 2.3% in the 10 mg q.d., 15 mg q.d., 10 mg b.i.d., and placebo groups, respectively. The most frequent adverse events (AEs) were transient headache, nausea, and dizziness. Echocardiograms showed no apparent drug-related effects on heart valves or pulmonary artery pressure (PAP). Lorcaserin was well tolerated and efficacious for weight reduction in this 12-week study. Longer-term trials employing behavior modification will be needed to more fully assess its safety and efficacy.