Faculty Bibliography

In adults, left temporal lobe pathology is typically associated with verbal memory deficits, whereas right temporal lobe pathology is thought to produce visual memory deficits in right-handed individuals. However, in children and adolescents with temporal lobe pathology, conclusions regarding material specificity of memory deficits remain unclear. The goal of the present case series is to examine the profile of verbal and visual memory impairment in children with temporal lobe tumors. Three patients with identified right temporal tumors and three patients with left temporal tumors are included. The Wide Range Assessment of Memory and Learning-Second Edition (WRAML-2) was administered as part of a larger neuropsychological battery. As anticipated, participants with right temporal lesions showed impaired visual memory relative to intact verbal memory. Interestingly, although the discrepancies between verbal and visual indices were less extreme, those with left temporal lesions showed a similar memory profile. These seemingly counterintuitive findings among left temporal tumor patients likely reflect less hemispheric specialization in children in comparison to adults and the fact that early developmental lesions in the left hemisphere may lead to functional reorganization of language-based skills.

INTRODUCTION: Tuberous sclerosis is associated with three central nervous system pathologies: cortical/subcortical tubers, subependymal nodules (SENs), and subependymal giant cell astrocytomas (SEGAs). Tubers are associated with epilepsy, which is often medication-resistant and often leads to resective surgery. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be effective reducing seizure burden in some patients with tuberous sclerosis complex (TSC)-related refractory epilepsy. mTORi have also been shown to be an alternative for surgery treating SEGAs. We describe several cases of resected tubers that contained SEGA tissue without an intraventricular SEGA. METHODS: After institutional review board (IRB) protocol approval, we retrospectively reviewed the surgical-pathological data for all TSC patients who underwent cortical resections for treatment of refractory epilepsy at NYU Langone Medical Center and Tel Aviv Medical Center between 2003 and 2013. Data included demographics, epilepsy type, MRI characteristics, epilepsy outcome, and histopathological staining. RESULTS: We reviewed cortical resections from 75 patients with complete pathological studies. In three patients, cortical lesions demonstrated histopathological findings consistent with a SEGA within the resected tuber tissue, with no intraventricular SEGA. All lesions were cortically based and none had any intraventricular extension. No patient had been treated before surgery with an mTORi. Two of the three patients remain Engel grade I-II. All lesions stained positive for glial fibrillary acidic protein (GFAP), synaptophysin, and neuronal nuclear antigen (NeuN). CONCLUSION: This is the first description of cortical tubers harboring SEGA tissue. This observation though preliminary may suggest a subgroup of patients with intractable epilepsy in whom mTORi may be considered before surgical intervention.

New developments in diagnostic methods, technical improvements in the surgical field, and a better understanding of the effects of epilepsy on the developing brain are contributing to the general observation that more children with epilepsy are being treated surgically. Malformations of cortical development are the most common cause of seizures in pediatric surgical candidates, and the best predictor of seizure freedom after surgery appears to be the complete removal of the epileptogenic lesion. To achieve this goal in challenging cases, such as magnetic resonance imaging-negative or multifocal lesions, a staged approach with pre- and/or post-resective invasive electroencephalography monitoring has increasingly been used at a number of centers. As the experience with this approach has grown, and the risks and benefits are better understood, a larger number of patients with epilepsy have been identified as potential surgical candidates. In this review, a number of the recent developments in pediatric epilepsy surgery are discussed.

Intrathecal baclofen therapy is widely accepted as a treatment option for patients with severe spasticity. The current treatment of spasticity in patients with Sjogren-Larsson syndrome is largely symptomatic, given that no effective causal therapy treatments are available. We report the outcome of 2 patients with Sjogren-Larsson syndrome who had pump implantation for intrathecal baclofen. We observed a positive response, with a decrease of spasticity, reflecting in the Modified Ashworth Scale, and parents and caregivers observed a functional improvement in both patients. One patient experienced skin irritation 15 months after surgery, necessitating pump repositioning. No infection occurred. Our report shows that intrathecal baclofen therapy can have a positive therapeutic effect on spasticity in patients with Sjogren-Larsson syndrome, and therefore may be a promising addition to current treatments.

INTRODUCTION: Tuberous Sclerosis Complex (TSC) has an incidence of 1/6,000 in the general population. Overall care may be very complex and costly. We examine trends in healthcare utilization and outcomes of TSC patients over the last decade. METHODS: The National Inpatient Sample (NIS) database for inpatient hospitalizations was searched for admission of patients with TSC. RESULTS: During 2000-2010, the NIS captured 5655 TSC patients. The majority patients were admitted to teaching hospitals (71.7%). Over time, the percentage of craniotomies performed per year remained stable (p = 0.351). Relevant diagnoses included neuro-oncologic pathology (5.4%), hydrocephalus (6.5%), and epilepsy (41.2%). Hydrocephalus significantly increased length of stay and hospital charges. A higher percentage of patients who underwent craniotomy had hydrocephalus (29.8% versus 5.3%; p < 0.001), neuro-oncologic pathology (43.5% versus 3.4%; p < 0.001), other cranial pathologies (4.2% versus 1.2%; p < 0.001), and epilepsy (61.4% versus 40.1%; p < 0.001). CONCLUSION: Our study identifies aspects of inpatient healthcare utilization, outcomes, and cost of a large number of patients with TSC. These aspects include related diagnoses and procedures that contribute to longer length of stay, increased hospital cost, and increased in-hospital mortality, which can inform strategies to reduce costs and improve care of patients with TSC.

OBJECTIVE: Neuroendoscopy is increasingly being used in the management of intraventricular brain tumors. The role of endoscopy in diagnostic biopsy is well established. Expansion of these techniques may allow for definitive resection of intraventricular tumors. We report the feasibility and outcomes of endoscopic resection of select intraventricular tumors in children. METHODS: The clinical characteristics of 11 children with solid intraventricular tumors who underwent tumor resection were reviewed. Twelve procedures were performed. RESULTS: Gross-total resection was achieved in 11 of 12 cases (92%). Maximal tumor diameter ranged from 9 to 26 mm (mean, 16.6 mm). Pathologic results included subependymal giant cell astrocytomas, ependymomas, nongerminomatous germ cell tumors, and pilocytic astrocytomas. Mean follow-up was 35 months (range, 10-109 months). All patients returned to their neurologic baselines after surgery. Local tumor recurrence occurred in 1 patient and distant recurrence in another. In 1 patient, a transitory intraoperative increase of intracranial pressure without clinical implications occurred. There was no permanent morbidity or mortality in this series. Hydrocephalus was present preoperatively in 5 cases and was treated either with tumor removal alone or with an additional endoscopic third ventriculostomy. No patient required a ventriculoperitoneal shunt. CONCLUSIONS: Neuroendoscopic gross-total resection of solid intraventricular tumors is a safe and efficacious procedure in carefully selected pediatric patients.

BACKGROUND: There are no established variables that predict the success of curative resective epilepsy surgery in children with tuberous sclerosis complex (TSC). OBJECTIVE: We performed a multicenter observational study to identify preoperative factors associated with seizure outcome in children with TSC undergoing resective epilepsy surgery. METHODS: A retrospective chart review was performed in eligible children at New York Medical Center, Miami Children's Hospital, Cleveland Clinic Foundation, BC Children's Hospital, Hospital for Sick Children, and Sainte-Justine Hospital between January 2005 and December 2013. A time-to-event analysis was performed. The "event" was defined as seizures after resective epilepsy surgery. RESULTS: Seventy-four patients (41 male) were included. The median age of the patients at the time of surgery was 120 months (range, 3-216 months). The median time to seizure recurrence was 24.0 +/- 12.7 months. Engel Class I outcome was achieved in 48 (65%) and 37 (50%) patients at 1- and 2-year follow-up, respectively. On univariate analyses, younger age at seizure onset (hazard ratio [HR]: 2.03, 95% confidence interval [CI]: 1.03-4.00, P = .04), larger size of predominant tuber (HR: 1.03, 95% CI: 0.99-1.06, P = .12), and resection larger than a tuberectomy (HR: 1.86, 95% CI: 0.92-3.74, P = .084) were associated with a longer duration of seizure freedom. In multivariate analyses, resection larger than a tuberectomy (HR: 2.90, 95% CI: 1.17-7.18, P = .022) was independently associated with a longer duration of seizure freedom. CONCLUSION: In this large consecutive cohort of children with TSC and medically intractable epilepsy, a greater extent of resection (more than just the tuber) is associated with a greater probability of seizure freedom. This suggests that the epileptogenic zone may include the cortex surrounding the presumed offending tuber. ABBREVIATIONS: EEG, electroencephalographyEZ, epileptic zoneIPD, individual participant dataTSC, tuberous sclerosis complex.

Objective: We report the feasibility and outcomes of endoscopic resection of select intraventricular tumors in children. Methods: The clinical characteristics of 11 children with solid intraventricular tumors who underwent tumor resection were reviewed. 12 procedures were performed. Results: Gross total resection was achieved in 11 of 12 cases (92%). Maximal diameter ranged from 9-26 mm (mean 16.6 mm). Pathology included subependymal giant cell astrocytomas (SEGA), ependymomas, non-germinomatous germ cell tumor (NGGCT) and pilocytic astrocytoma. Mean follow-up was 35 months (range 10-109 months). All patients returned to their neurological baseline following surgery. Local tumor recurrence occurred in one patient and distant recurrence in another. Complications occurred in one patient, no permanent morbidity or mortality occurred. Hydrocephalus was present preoperatively in 5 cases and was treated with tumor removal alone or with additional endoscopic third ventriculostomy. No patient required a ventriculoperitoneal shunt. Conclusion: Neuroendoscopic gross-total resection of solid intraventricular tumors is a safe procedure in carefully selected pediatric patients

A 4-year-old girl with a history of thoracic meningocele repair at the age of 3 months presented with progressive myelopathy. An intramedullary thoracic epidermoid was identified on MRI. The patient underwent excision of the epidermoid and subsequently returned to neurological baseline. This case illustrates the potential for delayed development of intraspinal epidermoid after initial repair of a simple meningocele.