Objective Antiepileptic drugs (AEDs) have cognitive side effects that, particularly in children, may affect intellectual functioning. With the TimeToStop (TTS) study, we showed that timing of AED withdrawal does not majorly influence long‐term seizure outcomes. We now aimed to evaluate the effect of AED withdrawal on postoperative intelligence quotient (IQ), and change in IQ (delta IQ) following pediatric epilepsy surgery. Methods We collected IQ scores of children from the TTS cohort with both pre‐ and postoperative neuropsychological assessments (NPAs; n = 301) and analyzed whether reduction of AEDs prior to the latest NPA was related to postoperative IQ and delta IQ, using linear regression analyses. Factors previously identified as independently relating to (delta) IQ, and currently identified predictors of (delta) IQ, were considered possible confounders and used for adjustment. Additionally, we adjusted for a compound propensity score that contained previously identified determinants of timing of AED withdrawal. Results Mean interval to the latest NPA was 19.8 ± 18.9 months. Reduction of AEDs at the latest NPA significantly improved postoperative IQ and delta IQ (adjusted regression coefficient [RC] = 3.4, 95% confidence interval [CI] = 0.6–6.2, p = 0.018 and RC = 4.5, 95% CI = 1.7–7.4, p = 0.002), as did complete withdrawal (RC = 4.8, 95% CI = 1.4–8.3, p = 0.006 and RC = 5.1, 95% CI = 1.5–8.7, p = 0.006). AED reduction also predicted ≥10‐point IQ increase ( p = 0.019). The higher the number of AEDs reduced, the higher was the IQ (gain) after surgery (RC = 2.2, 95% CI = 0.6–3.7, p = 0.007 and RC = 2.6, 95% CI = 1.0–4.2, p = 0.001, IQ points per AED reduced). Interpretation Start of AED withdrawal, number of AEDs reduced, and complete AED withdrawal were associated with improved postoperative IQ scores and gain in IQ, independent of other determinants of cognitive outcome. Ann Neurol 2015;78:104–114

We report a case of a 9-year-old boy with normal cognitive and neurological status. Pharmacoresistant epilepsy started at 2 years of age when he woke up from sleep with epigastric painful sensations. During the course, somatosensory seizures with pain in the legs and, from 3.2 years on, gelastic seizures and dyscognitive seizures occurred. High-resolution magnetic resonance image (MRI) of the brain revealed bilateral independent hypothalamic hamartomas in the supramamillary region. After presurgical evaluation, a decision for an endoscopic resection of the bigger, right-sided hamartoma was made. Seizures improved transiently after surgery (Engel 3a). However, no significant improvement was noted 6 months after surgery. As the only negative consequence, a marked weight gain followed surgery. Cognitive functions remained stable. On the basis of this case, we discuss the therapeutic options for this rare condition which include resection of the contralateral hamartoma or stereotactic laser ablation.

We are reporting on a 13.5-year-old girl with tuberous sclerosis complex (TSC) who was treated with everolimus because of giant cell astrocytoma and bilateral angiomyolipoma. She suffered from pharmacoresistant partial epilepsy with clusters of tonic and tonic-clonic seizures. Treatment with carbamazepine and sulthiame had led to a stable situation for more than 2.5 years. The dosage of everolimus had to be increased and refractory status epilepticus followed after 12 days. In the absence of any other possible cause, we believe that the status epilepticus was provoked by everolimus. So far, only a few cases of possible seizure aggravation by everolimus have been reported. The clinical relevance of possible negative effects in epileptic patients remains unclear. Similar observations should be documented and reported.

Case Report: Within the last 3 year we saw seven inpatients with double cortex syndrome at our Department in Kork. Five out of seven girls had a history of visual auras which were accompanied by panic attacks in two. We report of a girl with impressive transient MRI findings supporting the hypothesis of visual auras.

Purpose The new International League Against Epilepsy (ILAE) classification for focal cortical dysplasia (FCD) differentiates between patients with isolated FCD (type 1) and FCD with an associated hippocampal sclerosis (HS) (type 3a). In contrast to the former FCD classification by Palmini, which considered only histologic features, the novel ILAE classification also relies on magnetic resonance imaging (MRI) findings and presumed pathogenesis. We investigated in a cohort of 100 patients with exclusively temporal FCD if the new subdivision of FCD is reflected in clinical characteristics. Methods Thirty-one patients with FCD type 1 and 50 patients with FCD type 3a in the temporal lobe were included. In all patients MRI and histology of the FCD were available. Both patient groups were compared to 19 patients with temporal FCD type 2 with clearly different histologic appearance. Key Findings Patients with FCD type 1 and type 3a presented with similar clinical features in many respects. In univariate analyses, no statistically significant differences were found as to age at epilepsy onset (p=0.07) and epilepsy surgery (p=0.14), a normal appearing neocortical temporal lobe (p=0.08) or diagnosis of FCD by visual inspection of MRI (p=0.08), preoperative seizure frequency (p=0.06), and the predominance of an epigastric aura (p=0.08). The postoperative outcome was nearly identical 1year (p=0.8) and 2 (p=0.8), 3 (p=0.8), 5 (p=0.7), and 8 (p=1.0) years postoperatively. Only febrile seizures (p=0.025) and an aura (p=0.03) were significantly more frequently reported in patients with FCD type 3a. Similar results were obtained from a multivariate logistic regression analysis. Patients with FCD type 2 were more different: Compared to FCD type 3a, age at epilepsy surgery was significantly lower (p=0.004) and auras (p=0.005) were significantly less frequently reported. Epigastric auras (p=0.04) and febrile seizures (p=0.025) occurred significantly less frequently in patients with FCD type 2 without HS compared to FCD type 3a. The diagnosis of an FCD was significantly more frequently made (p=0.03) by visual inspection of the MRI compared to FCD type 1. Significance Clinical features did not allow to clear separation of temporal FCD types 1 and 3a. Statistically significant differences were seen in a history of febrile seizures and the occurrence of auras more common in FCD type 3a. However, FCD type 2 in the same localization but with different histology presented with further differences such as more frequent FCD diagnosis by visual inspection of MRI, earlier operation, and less frequent epigastric auras.

Introduction: Epilepsy with gelastic seizures and hypothalamic hamartomas is a rare but well defined syndrome with a typical onset in early childhood. In general this syndrom is pharmakoresistent. Most patients suffer not only from seizures but neuropsychological and social deficits.

Purpose: Resective surgery in early childhood is becoming an important treatment option in refractory structural epilepsy. We report on epilepsy outcomes, perioperative complications and further development in very young children who underwent epilepsy surgery at the Epilepsy Centre, University Hospital Freiburg.

Introduction: Hemispheric brain lesions are commonly associated with neurological deficit and early onset of catastrophic epilepsy with multiple seizure types and deleterious effects on cognitive development. Functional hemispherotomy is an established surgical procedure that enables functional isolation of a single or multiple unilateral epileptogenic regions.