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Varina L. Boerwinkle, Lucia Mirea, William D. Gaillard, Bethany L. Sussman, Diana Larocque, Alexandra Bonnell, Jennifer S. Ronecker, Matthew M. Troester, John F. Kerrigan, Stephen T. Foldes, Brian Appavu, Randa Jarrar, Korwyn Williams, Angus A. Wilfong and P. David Adelson

patients undergo surgery. 7 The primary determinant of surgical candidacy and postoperative seizure frequency is accurate localization of the seizure onset zone (SOZ), which, when excised or disconnected, should lead to cure. 25 Recently, several studies have indicated that resting-state functional MRI (rs-fMRI) localizes where seizures originate in the brain, 2–4 and its implementation improves surgical outcome. 5 Agreement of rs-fMRI SOZ and intracranial electroencephalography (EEG) SOZ localization is 90%. 4 Quantification of resection with respect to preoperative

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Mark Van Poppel, James W. Wheless, Dave F. Clarke, Amy McGregor, Mark H. McManis, Freedom F. Perkins Jr., Katherine Van Poppel, Stephen Fulton and Frederick A. Boop

surgery, VNS is a common obstacle to obtaining MSI data. Although VNS is safe for use with MRI and MEG studies, it can preclude language localization due to significant artifact. 14 New algorithms minimize artifact, hopefully allowing more of these patients to undergo MEG. Conclusions Our case series shows that MEG can accurately identify both epileptogenic foci and receptive language cortices by using passive functional mapping in sedated and sleeping patients to evaluate them for surgical candidacy. The information that MSI localization provides can also be

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Ravindra Arya, Jeffrey R. Tenney, Paul S. Horn, Hansel M. Greiner, Katherine D. Holland, James L. Leach, Michael J. Gelfand, Leonid Rozhkov, Hisako Fujiwara, Douglas F. Rose, David N. Franz and Francesco T. Mangano

distributed epileptogenic networks, and the improvement in EEG findings probably reflects the result of removing, or at least disrupting, the dominant seizure-generating network, which results in reduced cerebral irritability. There is a remarkable paucity of comparative data on this aspect in the TSC epilepsy surgery literature. Traditionally, surgical candidacy for patients with TSC was limited to those who had a single dominant tuber with concordant neurophysiological data, 7 , 8 , 15 and other children were usually denied resective surgery. This pessimistic outlook

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Ashish H. Shah, George M. Ibrahim, Jun Sasaki, John Ragheb, Sanjiv Bhatia and Toba N. Niazi

OBJECTIVE

Although endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has gained increasing prominence in the management of hydrocephalus caused by intraventricular hemorrhage of prematurity, the rates of long-term shunt independence remain low. Furthermore, limited evidence is available to identify infants who might benefit from the procedure. The authors tested the hypothesis that elevated venous pressure that results from comorbid cardiac disease might predispose patients to ETV/CPC failure and shunt dependence.

METHODS

A retrospective analysis was performed on a consecutive series of 48 infants with hydrocephalus who underwent ETV/CPC and also underwent preoperative echocardiography between 2007 and 2014. Comorbid cardiac abnormalities that are known to result in elevated right heart pressure were reviewed. Associations between ETV/CPC success and the presence of pulmonary hypertension, right ventricular hypertrophy, left-to-right shunting, ventricular septal defect, or patent ductus arteriosus were determined using multivariate logistic regression analysis.

RESULTS

Of the 48 children who met the inclusion criteria, ETV/CPC failed in 31 (65%). In univariate analysis, no single echocardiogram abnormality was associated with shunt failure, but the presence of 2 or more concurrent echocardiogram abnormalities was associated with ETV/CPC failure (17 [85%] of 20 vs 14 [50%] of 28, respectively; p = 0.018). In multivariate logistic regression analysis, when the authors adjusted for the child’s ETV success score, the presence of 2 abnormalities remained independently associated with poor outcome (2 or more echocardiogram abnormalities, OR 0.13, 95% CI 0.01–0.7, p = 0.032; ETV success score, OR 1.1, 95% CI 1–1.2, p = 0.05).

CONCLUSIONS

In this study, cardiac abnormalities were inversely associated with the success of ETV/CPC in infants with hydrocephalus of prematurity. ETV/CPC might not be as efficacious in patients with significant cardiac anomalies. These results provide a basis for future efforts to stratify surgical candidacy for ETV/CPC on the basis of comorbid abnormalities. Proper cardiac physiological pressure monitoring might help elucidate the relationship between cardiac abnormalities and hydrocephalus.

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Edward F. Chang, Srikantan S. Nagarajan, Mary Mantle, Nicholas M. Barbaro and Heidi E. Kirsch

Object

Routine scalp electroencephalography (EEG) cannot always distinguish whether generalized epileptiform discharges are the result of primary bilateral synchrony or secondary bilateral synchrony (SBS) from a focal origin; this is an important distinction because the latter may be amenable to resection. Whole-head magnetoencephalography (MEG) has superior spatial resolution compared with traditional EEG, and can potentially elucidate seizure foci in challenging epilepsy cases in which patients are undergoing evaluation for surgery.

Methods

Sixteen patients with medically intractable epilepsy in whom SBS was suspected were referred for magnetic source (MS) imaging. All patients had bilateral, synchronous, widespread, and most often generalized spike-wave discharges on scalp EEG studies, plus some other clinical (for example, seizure semiology) or MR imaging feature (for example, focal lesion) suggesting focal onset and hence possible surgical candidacy. The MS imaging modality is the combination of whole-head MEG and parametric reconstruction of corresponding electrical brain sources. An MEG and simultaneous EEG studies were recorded with a 275-channel whole-head system. Single-equivalent current dipoles were estimated from the MEG data, and dipole locations and orientations were superimposed on patients' MR images.

Results

The MS imaging studies revealed focal dipole clusters in 12 (75%) of the 16 patients, of which a single dipole cluster was identified in 7 patients (44%). Patient age, seizure type, duration of disease, video-EEG telemetry, and MR imaging results were analyzed to determine factors predictive of having clusters revealed on MS imaging. Of these factors, only focal MR imaging anatomical abnormalities were associated with dipole clusters (chi-square test, p = 0.03). Selective resections (including the dipole cluster) in 7 (87%) of 8 patients resulted in seizure-free or rare seizure outcomes (Engel Classes I and II).

Conclusions

Magnetic source imaging may provide noninvasive anatomical and neurophysiological confirmation of localization in patients in whom there is a suspicion of SBS (based on clinical or MR imaging data), especially in those with an anatomical lesion. Identification of a focal seizure origin has significant implications for both resective and nonresective treatment of intractable epilepsy.

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Jetan H. Badhiwala, Brij Karmur, Lior M. Elkaim, Naif M. Alotaibi, Benjamin R. Morgan, Nir Lipsman, Philippe De Vloo, Suneil K. Kalia, Andres M. Lozano and George M. Ibrahim

OBJECTIVE

Although deep brain stimulation (DBS) is an accepted treatment for childhood dystonia, there is significant heterogeneity in treatment response and few data are available to identify ideal surgical candidates.

METHODS

Data were derived from a systematic review and individual patient data meta-analysis of DBS for dystonia in children that was previously published. Outcomes were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale for movement (BFMDRS-M) and for disability (BFMDRS-D). The authors used partial least squares, bootstrapping, and permutation statistics to extract patterns of contributions of specific preoperative characteristics to relationship with distinct outcomes, in all patients and in patients with primary and secondary dystonia separately.

RESULTS

Of 301 children undergoing DBS for dystonia, 167 had primary dystonia, 125 secondary dystonia, and 9 myoclonus dystonia. Three dissociable preoperative phenotypes (latent variables) were identified and associated with the following: 1) BFMDRS-M at last follow-up; 2) relative change in BFMDRS-M score; and 3) relative change in BFMDRS-D score. The phenotype of patients with secondary dystonia, with a high BFMDRS-M score and truncal involvement, undergoing DBS at a younger age, was associated with a worse postoperative BFMDRS-M score. Children with primary dystonia involving the trunk had greater improvement in BFMDRS-M and -D scores. Those with primary dystonia of shorter duration and proportion of life with disease, undergoing globus pallidus DBS, had greater improvements in BFMDRS-D scores at long-term follow-up.

CONCLUSIONS

In a comprehensive, data-driven, multivariate analysis of DBS for childhood dystonia, the authors identified novel and dissociable patient phenotypes associated with distinct outcomes. The findings of this report may inform surgical candidacy for DBS.

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Joanna Y. Wang, Anubhav G. Amin, George I. Jallo and Edward S. Ahn

Object

The most common neurosurgical condition observed in preterm infants is intraventricular hemorrhage (IVH), which often results in posthemorrhagic hydrocephalus (PHH). These conditions portend an unfavorable prognosis; therefore, the potential for poor neurodevelopmental outcomes necessitates a better understanding of the comparative effectiveness of 2 temporary devices commonly used before the permanent insertion of a ventriculoperitoneal (VP) shunt: the ventricular reservoir and the ventriculosubgaleal shunt (VSGS).

Methods

The authors analyzed retrospectively collected information for 90 patients with IVH and PHH who were treated with insertion of a ventricular reservoir (n = 44) or VSGS (n = 46) at their institution over a 14-year period.

Results

The mean gestational age and weight at device insertion were lower for VSGS patients (30.1 ± 1.9 weeks, 1.12 ± 0.31 kg) than for reservoir patients (31.8 ± 2.9 weeks, 1.33 ± 0.37 kg; p = 0.002 and p = 0.004, respectively). Ventricular reservoir insertion was predictive of more CSF taps prior to VP shunt placement compared with VSGS placement (10 ± 8.7 taps vs 1.6 ± 1.7 taps, p < 0.001). VSGS patients experienced a longer time interval prior to VP shunt placement than reservoir patients (80.8 ± 67.5 days vs 48.8 ± 26.4 days, p = 0.012), which corresponded to VSGS patients gaining more weight by the time of shunt placement than reservoir patients (3.31 ± 2.0 kg vs 2.42 ± 0.63 kg, p = 0.016). Reservoir patients demonstrated a trend toward more positive CSF cultures compared with VSGS patients (n = 9 [20.5%] vs n = 5 [10.9%], p = 0.21). There were no significant differences in the rates of overt device infection requiring removal (reservoir, 6.8%; VSGS, 6.5%), VP shunt insertion (reservoir, 77.3%; VSGS, 76.1%), or early VP shunt infection (reservoir, 11.4%; VSGS, 13.0%) between the 2 cohorts.

Conclusions

Although the rates of VP shunt requirement and device infection were similar between patients treated with the reservoir versus the VSGS, VSGS patients were significantly older and had achieved greater weights at the time of VP shunt insertion. The authors' results suggest that the VSGS requires less labor-intensive management by ventricular tapping; the VSGS patients also attained higher weights and more optimal surgical candidacy at the time of VP shunt insertion. The potential differences in long-term developmental and neurological outcomes between VSGS and reservoir placement warrant further study.

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George M. Ibrahim, Benjamin W. Barry, Aria Fallah, O. Carter Snead III, James M. Drake, James T. Rutka and Mark Bernstein

awareness of discrepancies in evaluation between different modalities surveillance of children for future surgical candidacy disclosure of evaluation methods & discrepancies during informed consent equity despite inequality application of best practices given current resources referral of complex cases to tertiary & quaternary centers societal benefit consideration of cost effectiveness of interventions Access In the most basic sense, health care providers have an ethical obligation to facilitate access to epilepsy surgery for selected

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into the C5.0 statistical package in R. A 60/40 model was employed, whereby 60% of randomized data were used to train the decision tree, while the remaining 40% were used to test the decision tree. The outcome of interest for the decision tree was a severe lesion meeting requirements for surgical candidacy. Results: A decision tree prediction algorithm was generated from the entered variables (Figure 1). Variables utilized in the final decision tree included presence of Horner's syndrome, presence of a pseudomeningocele, Narakas grade, clavicle fracture at

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Ahmed M. Raslan, Reynaldo DeJesus, Caglar Berk, Andrew Zacest, Jim C. Anderson and Kim J. Burchiel

P rimary hemifacial spasm is a neurological syndrome secondary to vascular impingement of CN VII in the cerebellopontine angle 1 , 13 , 16 and may be cured surgically if definitive microsurgical decompression is performed. 2 , 9 , 11 Although surgical decision-making has traditionally been clinically driven, recently the role of improved neuroimaging studies such as 3-T MR imaging sequences to guide or assist the surgical candidacy decision has been questioned. 3 Currently, many imaging techniques are used to demonstrate NVC before surgery. High