Faculty Bibliography

OBJECTIVE: Tuberous sclerosis complex (TSC) is associated with medically refractory epilepsy and developmental delay in children and usually results from cortical tubers. Seizures that begin in young patients are often refractory and may contribute to development delay. Functional outcome is improved when seizures are controlled at an early age. Previous reports have shown modest benefit from surgical resection of single tubers/seizure foci in older children; however, many children with TSC develop uncontrolled seizures before age 1. To identify patients who might benefit from surgery and to maximize outcome, we used a novel surgical approach in young children that consists of invasive intracranial monitoring, which is typically 3-staged and often bilateral. METHODS: Of 110 consecutive children who underwent epilepsy surgery by a single surgeon in the past 6 years, 25 patients (9 boys and 16 girls) had TSC. At the time of their first surgery at our institution, they were a median age of 4.0 years. A total of 31 separate admissions for epilepsy surgery in these 25 patients were identified. Bilateral electrode placement was performed in 13 children whose seizures could not be lateralized definitively preoperatively, and 22 patients underwent 3-stage surgeries. RESULTS: At 6 months or longer after the initial resection, 21 (84%) children were class I, 2 (8%) children were class II, and 2 (8%) children were class IV. At a mean follow-up of 28 months, 17 (68%) children were class I, 6 (24%) were class II, and 2 (8%) were class III. Four of the 5 children who initially were rejected as surgical candidates because of multifocality and who required initial bilateral electrode study are now seizure-free. CONCLUSIONS: This approach can help to identify both primary and secondary epileptogenic zones in young TSC patients with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery. Long-term follow-up will determine whether this approach has durable effects

The occurrence of an aura is often considered evidence of a partial rather than an idiopathic generalized epilepsy syndrome. The authors examined this hypothesis by prospectively recording reports of auras by patients being admitted for video-EEG monitoring. Auras were equally common (70%) among patients with idiopathic generalized epilepsy as they were among those with localization-related epilepsy. Presence of an aura is not a reliable indicator of localization-related epilepsy

False-positive (FP) errors during recognition memory testing often interfere with interpretation of Wada test results. This study examined which clinical and neuropsychological variables provide the best prediction of these errors. Fifty-six patients completed the Wada test and the California Verbal Learning Test (CVLT). Subjects with three or more FP errors on the recognition trials of the CVLT were nearly twice as likely to exhibit FP responding during the Wada test. Further analysis indicates that FP errors during the Wada test appear to be the primary result of a stable and liberal response-bias rather than a result of any other neurological or procedure-related factor

BACKGROUND: Several risk factors have been attributed to seizure recurrence after surgery. It is unknown whether race/ethnicity plays a role in outcome. OBJECTIVE: To evaluate whether race/ethnicity plays a role in seizure recurrence after surgery. DESIGN: Cohort study. SETTING: We evaluated data obtained from the epilepsy centers at the University of Alabama at Birmingham and New York University, New York, NY. PATIENTS: All patients included had a diagnosis of mesial temporal sclerosis and underwent temporal lobectomy. MAIN OUTCOME MEASURES: Occurrence of seizure after surgery was registered 1 year after surgery. We used multiple logistic regression analysis to model the presence of seizure recurrence after surgery and generated odds ratios (ORs) for seizure recurrence after surgery for African American and Hispanic patients relative to white patients. An unadjusted model incorporated only race/ethnicity as the independent variable, and an adjusted model included socioeconomic status, age, duration of epilepsy, education, history of febrile seizures, sex, handedness, lateralization of epileptogenic focus, and number of antiepileptics as the independent variables. RESULTS: Two hundred fifty-two patients underwent surgical treatment with pathological confirmation of mesial temporal sclerosis. No differences were found between racial/ethnic groups in terms of seizure recurrence in any models. For African American patients, the ORs were 0.9 (95% confidence interval [CI], 0.4-2.1) for the unadjusted model and 0.8 (95% CI, 0.3-2.0) for the adjusted model; for Hispanic patients, the ORs were 1.6 (95% CI, 0.8-3.2) for the unadjusted model and 1.1 (95% CI, 0.5-2.6) for the adjusted model, relative to white patients. CONCLUSION: Our data suggest that although sex appears to play a role in the outcomes of surgery for temporal lobe epilepsy, race and socioeconomic status do not

In patients with tuberous sclerosis complex (TSC), the high rates of mental retardation are associated with cortical tubers, seizure activity, and genetic factors. The goal of the study was to investigate the relationship between bilateral cortical tubers and seizure variables and mental retardation in individuals with TSC. The records of 27 patients with TSC (age 6 months to 33 years) undergoing neuropsychological assessment and the following clinical variables were examined: bilateral versus non-bilateral cortical tubers, the age of seizure onset, and presence of infantile spasms. Results were statistically analyzed. Bilateral cortical tubers (p=0.02) and early age of seizure onset (p=0.04) were significantly related to impaired cognitive functioning. Only one of the seven patients with normal cognitive functioning had bilateral tubers, whereas 13/21 patients with intellectual impairment had bilateral tubers. Patients with normal cognitive functioning experienced a mean age of seizure onset after 6 years. A trend was observed between infantile spasms and cognitive functioning (p=0.06); the lack of statistical significance likely reflects the small sample size. Neither age nor gender was related to cognitive status. Further investigation incorporating additional neuroimaging factors, antiepileptic treatment effects, and genetic variables, is needed