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Edward F. Chang, Doris D. Wang, David W. Perry, Nicholas M. Barbaro and Mitchel S. Berger

Object

Language dominance in the right hemisphere is rare. Therefore, the organization of essential language sites in the dominant right hemisphere is unclear, especially compared with cases involving the more prevalent left dominant hemisphere.

Methods

The authors reviewed the medical records of 15 patients who underwent awake craniotomy for tumor or epilepsy surgery and speech mapping of right hemisphere perisylvian language areas at the University of California, San Francisco. All patients were determined to have either complete right-sided or bilateral language dominance by preoperative Wada testing.

Results

All patients but one were left-handed. Of more than 331 total stimulation sites, 27 total sites were identified as essential for language function (14 sites for speech arrest/anarthria; 12 for anomia; and 1 for alexia). While significant interindividual variability was observed, the general pattern of language organization was similar to classic descriptions of frontal language production and posterior temporal language integration for the left hemisphere. Speech arrest sites were clustered in the ventral precentral gyrus and pars opercularis. Anomia sites were more widely distributed, but were focused in the posterior superior and middle temporal gyri as well as the inferior parietal gyrus. One alexia site was found over the superior temporal gyrus. Face sensory and motor cortical sites were also identified along the ventral sensorimotor strip. The prevalence and specificity of essential language sites were greater in unilateral right hemisphere–dominant patients, compared with those with bilateral dominance by Wada testing.

Conclusions

The authors' results suggest that the organization of language in right hemisphere dominance mirrors that of left hemisphere dominance. Awake speech mapping is a safe and reliable surgical adjunct in these rare clinical cases and should be done in the setting of right hemisphere dominance to avoid preventable postoperative aphasia.

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Doris D. Wang, Kenneth W. Martin, Kurtis I. Auguste and Peter P. Sun

Disorders of CSF dynamics such as syringomyelia and obstructive hydrocephalus can be caused by thin mobile obstructive lesions not visible on traditional MRI sequences. New imaging techniques with balanced steady-state free precession (bSSFP) and dynamic imaging with bSSFP cine allow visualization of these pulsatile structures within the CSF space. The authors present 2 cases involving pediatric patients—one who developed presumed idiopathic syringomyelia and one with presumed communicating hydrocephalus in association with Pfeiffer syndrome—who harbored thin dynamic obstructive lesions seen on bSSFP cine studies using 1.5-T MRI.

In combination with traditional CSF cine studies and bSSFP, bSSFP cine sequence was able to detect dynamic membranous adhesions not seen on traditional MRI sequences. These previously undetectable lesions on traditional MRI sequences were the etiology of CSF obstruction, and tailored surgical approaches were performed to avoid shunting in both patients. These reports demonstrate the clinical utility for using these novel imaging tools for the detection of thin adhesions and dynamic lesions in the central nervous system. Balanced SSFP cine sequences can supplement conventional MR modalities to identify these otherwise poorly visualized lesions responsible for presumed communicating hydrocephalus or idiopathic syringomyelia.

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Kunal P. Raygor, Doris D. Wang, Mariann M. Ward, Nicholas M. Barbaro and Edward F. Chang

OBJECTIVE

Microvascular decompression (MVD) and stereotactic radiosurgery (SRS) are common surgical treatments for trigeminal neuralgia (TN). Many patients who receive SRS have pain recurrence; the ideal second intervention is unknown. The authors directly compared pain outcomes after MVD and repeat SRS in a population of patients in whom SRS failed as their first-line procedure for TN, and they identified predictors of pain control.

METHODS

The authors reviewed a prospectively collected database of patients undergoing surgery for TN between 1997 and 2014 at the University of California, San Francisco (UCSF). Standardized data collection focused on preoperative clinical characteristics, surgical characteristics, and postoperative outcomes. Patients with typical type 1, idiopathic TN with ≥ 1 year of follow-up were included.

RESULTS

In total, 168 patients underwent SRS as their first procedure. Of these patients, 90 had residual or recurrent pain. Thirty of these patients underwent a second procedure at UCSF and had ≥ 1 year of follow-up; 15 underwent first-time MVD and 15 underwent repeat SRS. Patients undergoing MVD were younger than those receiving repeat SRS and were more likely to receive ≥ 80 Gy during the initial SRS. The average follow-up was 44.9 ± 33.6 months for MVD and 48.3 ± 45.3 months for SRS. All patients achieved complete pain freedom without medication at some point during their follow-up. At last follow-up, 80% of MVD-treated patients and 33.3% of SRS-treated patients had a favorable outcome, defined as Barrow Neurological Institute Pain Intensity scores of I–IIIa (p < 0.05). Percentages of patients with favorable outcome at 1 and 5 years were 86% and 75% for the MVD cohort and 73% and 27% for the SRS cohort, respectively (p < 0.05). Multivariate Cox proportional hazards analysis demonstrated that performing MVD was statistically significantly associated with favorable outcome (HR 0.12, 95% CI 0.02–0.60, p < 0.01). There were no statistically significant predictors of favorable outcome in the MVD cohort; however, the presence of sensory changes after repeat SRS was associated with pain relief (p < 0.01).

CONCLUSIONS

Patients who received MVD after failed SRS had a longer duration of favorable outcome compared to those who received repeat SRS; however, both modalities are safe and effective. The presence of post-SRS sensory changes was predictive of a favorable pain outcome in the SRS cohort.

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Doris D. Wang, Abby E. Deans, A. James Barkovich, Tarik Tihan, Nicholas M. Barbaro, Paul A. Garcia and Edward F. Chang

Object

Focal cortical dysplasia (FCD) represents a spectrum of developmental cortical abnormalities and is one of the most common causes of intractable epilepsy in children and young adults. Outcomes after surgery for FCD are highly variable, and prognosticators of seizure freedom are unclear. In a subset of FCDs, a transmantle sign is observed on imaging that focally spans the entire cerebral mantle from the ventricle to the cortical surface. The aim of this study was to characterize seizure control outcomes and prognostic significance of the transmantle sign in FCD epilepsy.

Methods

Fourteen patients with the transmantle sign underwent epilepsy surgery for medically refractory epilepsy. Thirteen patients underwent resective surgery and 1 underwent multiple subpial transections with vagus nerve stimulator placement. Patient demographics, MRI, electroencephalography, intraoperative electrocorticography (ECoG), and pathology were reviewed. The results of this series were compared with those of 114 previously reported patients with FCD without the transmantle sign.

Results

All patients were found to have childhood seizure onset and concordant MRI and ECoG findings. The primary MRI findings associated with transmantle sign included gray-white junction blurring, appearance of cortical thickening, T2 or FLAIR abnormality, and bottom-of-the-sulcus dysplasia. The transmantle sign was usually a focal finding, typically confined to 1 or several gyri with well-circumscribed epileptic tissue. Correlation of the transmantle sign with FCD histopathological subtypes was highly variable. Patients who underwent complete resection of MRI and ECoG abnormalities (12 of 13 patients) became seizure free. When compared with 114 FCD patients without the transmantle sign, patients with the transmantle sign showed significantly improved seizure-free outcomes after complete resections (p = 0.04).

Conclusions

The presence of the transmantle sign in patients with medically refractory partial epilepsy is associated with highly favorable seizure control outcomes after surgical treatment.

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Dario J. Englot, John D. Rolston, Doris D. Wang, Peter P. Sun, Edward F. Chang and Kurtis I. Auguste

Temporal lobe epilepsy (TLE) is the most common form of epilepsy in adults and is responsible for 15%–20% of epilepsy cases in children. Class I evidence strongly supports the use of temporal lobectomy for intractable TLE in adults, but fewer studies have examined seizure outcomes and predictors of seizure freedom after temporal lobectomy in pediatric patients. The authors performed a systematic review and meta-analysis of studies including 10 or more pediatric patients (age ≤ 19 years) published over the last 20 years examining seizure outcomes after temporal lobectomy for TLE. Thirty-six studies met their inclusion criteria. These 36 studies included 1318 pediatric patients with a mean age (± SEM) of 10.7 ± 0.3 years. Overall, seizure freedom (Engel Class I outcome) was achieved in 1002 cases (76%); 316 patients (24%) continued to have seizures (Engel Class II–IV outcome). All patients had at least 1 year of follow-up. Statistically significant predictors of seizure freedom after surgery included lesional epilepsy etiology (odds ratio [OR] 1.08, 95% confidence interval [CI] 1.02–1.15), abnormal findings on preoperative MRI (OR 1.27, 95% CI 1.16–1.40), and lack of generalized seizures (OR 1.36, 95% CI 1.20–1.56). Among lesional epilepsy cases, there was a trend toward better outcome with gross-total lesionectomy than with subtotal resection. Approximately three-fourths of pediatric patients with TLE attain seizure freedom after temporal lobectomy. Favorable outcomes may be predicted by lesional epilepsy etiology, abnormal MRI, and lack of generalized seizures. Pediatric patients with medically refractory TLE should be referred to a comprehensive pediatric epilepsy center for surgical evaluation.

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Dario J. Englot, David Ouyang, Doris D. Wang, John D. Rolston, Paul A. Garcia and Edward F. Chang

Object

Epilepsy surgery remains significantly underutilized. The authors recently reported that the number of lobectomies for localized intractable epilepsy in the US has not changed despite the implementation of clear evidence-based guidelines 10 years ago supporting early referral for surgery. To better understand why epilepsy surgery continues to be underused, the authors' objective was to carefully examine hospital-related factors related to the following: 1) where patients are being admitted for the evaluation of epilepsy, 2) rates of utilization for surgery across hospitals, and 3) perioperative morbidity between hospitals with low versus high volumes of epilepsy surgery.

Methods

The authors performed a population-based cohort study of US hospitals between 1990 and 2008 using the Nationwide Inpatient Sample (NIS), stratifying epilepsy surgery rates and trends as well as perioperative morbidity rates by hospital surgical volume.

Results

The number of lobectomies for epilepsy performed at high-volume centers (> 15 lobectomies/year) significantly decreased between 1990 and 2008 (F = 20.4, p < 0.001), while significantly more procedures were performed at middle-volume hospitals (5–15 lobectomies/year) over time (F = 16.1, p < 0.001). No time trend was observed for hospitals performing fewer than 5 procedures per year. However, patients admitted to high-volume centers were significantly more likely to receive lobectomy than those at low-volume hospitals (relative risk 1.05, 95% CI 1.03–1.08, p < 0.001). Also, the incidence of perioperative adverse events was significantly higher at low-volume hospitals (12.9%) than at high-volume centers (6.1%) (relative risk 1.08, 95% CI 1.03–1.07, p < 0.001).

Conclusions

Hospital volume is an important predictor of epilepsy surgery utilization and perioperative morbidity. Patients with medically refractory epilepsy should be referred to a comprehensive epilepsy treatment center for surgical evaluation by an experienced clinical team.

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John D. Rolston, Dario J. Englot, Doris D. Wang, Tina Shih and Edward F. Chang

Epilepsy is a devastating disease, often refractory to medication and not amenable to resective surgery. For patients whose seizures continue despite the best medical and surgical therapy, 3 stimulation-based therapies have demonstrated positive results in prospective randomized trials: vagus nerve stimulation, deep brain stimulation of the thalamic anterior nucleus, and responsive neurostimulation. All 3 neuromodulatory therapies offer significant reductions in seizure frequency for patients with partial epilepsy. A direct comparison of trial results, however, reveals important differences among outcomes and surgical risk between devices. The authors review published results from these pivotal trials and highlight important differences between the trials and devices and their application in clinical use.

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Dario J. Englot, Doris D. Wang, John D. Rolston, Tina T. Shih and Edward F. Chang

Object

Frontal lobe epilepsy (FLE) is the second-most common focal epilepsy syndrome, and seizures are medically refractory in many patients. Although various studies have examined rates and predictors of seizure freedom after resection for FLE, there is significant variability in their results due to patient diversity, and inadequate follow-up may lead to an overestimation of long-term seizure freedom.

Methods

In this paper the authors report a systematic review and meta-analysis of long-term seizure outcomes and predictors of response after resection for intractable FLE. Only studies of at least 10 patients examining seizure freedom after FLE surgery with postoperative follow-up duration of at least 48 months were included.

Results

Across 1199 patients in 21 studies, the overall rate of postoperative seizure freedom (Engel Class I outcome) was 45.1%. No trend in seizure outcomes across all studies was observed over time. Significant predictors of long-term seizure freedom included lesional epilepsy origin (relative risk [RR] 1.67, 95% CI 1.36–28.6), abnormal preoperative MRI (RR 1.64, 95% CI 1.32–2.08), and localized frontal resection versus more extensive lobectomy with or without an extrafrontal component (RR 1.71, 95% CI 1.26–2.43). Within lesional FLE cases, gross-total resection led to significantly improved outcome versus subtotal lesionectomy (RR 1.99, 95% CI 1.47–2.84).

Conclusions

These findings suggest that FLE patients with a focal and identifiable lesion are more likely to achieve seizure freedom than those with a more poorly defined epileptic focus. While seizure freedom can be achieved in the surgical treatment of medically refractory FLE, these findings illustrate the compelling need for improved noninvasive and invasive localization techniques in FLE.

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Dario J. Englot, John D. Rolston, Doris D. Wang, Kevin H. Hassnain, Charles M. Gordon and Edward F. Chang

Object

In the US, approximately 500,000 individuals are hospitalized yearly for traumatic brain injury (TBI), and posttraumatic epilepsy (PTE) is a common sequela of TBI. Improved treatment strategies for PTE are critically needed, as patients with the disorder are often resistant to antiepileptic medications and are poor candidates for definitive resection. Vagus nerve stimulation (VNS) is an adjunctive treatment for medically refractory epilepsy that results in a ≥ 50% reduction in seizure frequency in approximately 50% of patients after 1 year of therapy. The role of VNS in PTE has been poorly studied. The aim of this study was to determine whether patients with PTE attain more favorable seizure outcomes than individuals with nontraumatic epilepsy etiologies.

Methods

Using a case-control study design, the authors retrospectively compared seizure outcomes after VNS therapy in patients with PTE versus those with nontraumatic epilepsy (non-PTE) who were part of a large prospectively collected patient registry.

Results

After VNS therapy, patients with PTE demonstrated a greater reduction in seizure frequency (50% fewer seizures at the 3-month follow-up; 73% fewer seizures at 24 months) than patients with non-PTE (46% fewer seizures at 3 months; 57% fewer seizures at 24 months). Overall, patients with PTE had a 78% rate of clinical response to VNS therapy at 24 months (that is, ≥ 50% reduction in seizure frequency) as compared with a 61% response rate among patients with non-PTE (OR 1.32, 95% CI 1.07–1.61), leading to improved outcomes according to the Engel classification (p < 0.0001, Cochran-Mantel-Haenszel statistic).

Conclusions

Vagus nerve stimulation should be considered in patients with medically refractory PTE who are not good candidates for resection. A controlled prospective trial is necessary to further examine seizure outcomes as well as neuropsychological outcomes after VNS therapy in patients with intractable PTE.

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Ninghui Zhao, Doris D. Wang, Xiaobin Huang, Surya K. Karri, Haiying Wu and Mingxiu Zheng

The authors report, to the best of their knowledge, the first case of a spontaneous tension pneumocephalus with subcutaneous emphysema. Hyperpneumatization of the cranium and mechanical compression contributed jointly to the formation of a fistula, and air pressure caused a subsequent disruption of the suture and air leakage into the subcutaneous space. A minimally invasive otological procedure proved efficacious for resolution.