Comparing the determination of language dominance using fMRI with results of the Wada test in 100 patients with different localization-related epilepsies, the authors found 91% concordance between both tests. The overall rate of false categorization by fMRI was 9%, ranging from 3% in left-sided temporal lobe epilepsy (TLE) to 25% in left-sided extratemporal epilepsy. Language fMRI might reduce the necessity of the Wada test for language lateralization, especially in TLE.

Summary:Purpose: The detrimental effect of frequent early seizures on the cognitive potential of children is a significant clinical issue. Epilepsy surgery in childhood offers a good prognosis for seizure control and improved developmental outcome. We studied the postoperative outcome and the developmental velocity after surgery and analyzed risk factors for developmental delay in 50 consecutive preschool children treated surgically for severe epilepsy at ages 3 to 7 years.Methods: Pre-and postoperative developmental quotients (DQs) were analyzed with analysis of variance; stepwise linear regressions were performed on preoperative DQs and on a difference score between post-and preoperative DQs to determine risk factors for preoperative development and factors influencing postoperative development.Results: Of the 50 patients, 70% were retarded, with IQ < 70; 16% were of average intelligence, with IQ ranging from 85 to 115. Age at seizure onset and extent of lesion were predictive variables for preoperative cognitive development. Six to 12 months after surgery (early postoperative phase), 66% were seizure free (Engel outcome class I), 26% had substantial to worthwhile seizure reduction (classes II and III), and 8% were unchanged (class IV). Forty-one (82%) children showed stable velocity of development; three children showed gains of ≥15 IQ points; three had developmental decline (loss of ≥10 IQ points), which was transient in two children; and three children moved from not assessable to assessable. At last follow-up (6 months to 10 years after surgery), 11 children showed IQ/DQ gains of ≥15 IQ points. Gains in IQ were observed only in seizure-free children and were stable over time. Shorter duration of epilepsy was significantly associated with a postoperative increase in DQ.Conclusions: (a) Substantial global mental delay is common in young children treated for epilepsy with surgery; (b) In most patients, postoperative development proceeded at a stable velocity; (c) Catch-up development may occur but only in seizure-free patients; (d) Substantial cognitive losses were noted in only one child. and (e) Early seizure control stabilized developmental velocity in this patient cohort.

kinsonism and dementia. Brain MRI show iron in the substantia nigra and globus pallidus, as well as a halo of T1 hyperintense signal in the substantia nigra and cerebral peduncles. Male and female patients were phenotypically similar. Conclusion: We describe the first X-lined form of NBIA, due to mutations in WDR45. The phenotypic similarity between the sexes is likely due to somatic mosaicism in surviving males. WDR45 encodes a beta-propeller protein postulated to play a role in autophagy, a novel disease mechanism in NBIA. The newly termed BPAN (beta-propellar associated neurodegenera-tion) appears to be a significant cause of NBIA. Objectives: The hypothesis of epileptic encephalopathy posits that epileptiform discharge promotes changes in the connection between neurons in early childhood due to ongoing developmental structural plasticity. The presence of remodelled cortical architecture is evaluated in infants following presentation with 'idiopathic' WS. Methods: Cross-sectional study. Quantitative magnetic resonance imaging (3D structural MRI and diffusion tensor imaging) data was compared for 22 patients with newly diagnosed WS and 12 healthy age controls using voxel based morphometry (VBM) and tract-based spatial statistics (TBSS). Results: WS patients showed decreased white matter volume of the temporal lobes compared to controls (p<0.005), but did not show difference in white matter integrity on DTI metrics (frac-tional anisotropy and mean diffusivity). WS patients showed increased temporal lobe grey matter density (p<0.005), particularly in the perirhinal cortex. The structural changes occurred bilaterally. Conclusions: Following idiopathic West Syndrome a number of persistent neurological deficits referable to the temporal lobe are observed, including impaired receptive language, and combined visual-and-auditory agnosia. These are likely to reflect an altered connection pattern within the temporal lobe that is demonstrated here. The changes were found despite successful resolution of infantile spasm seizures on treatment, highlighting a need for treatment options that prevent the alteration of connection architecture in WS. Objective: To review the clinical features determining the offer of epilepsy surgery and surgical outcome in children with Tuberous Sclerosis Complex (TSC). Methods: Clinical data of 122 patients with TSC and complex epilepsy, referred to Great Ormond Street Hospital for epilepsy surgery from 2000 to 2012, were reviewed retrospectively. Pre-surgical evaluation included ictal video electroencephalogram (EEG) monitoring and brain Magnetic Resonance Imaging (MRI) in all, with selected patients undergoing ictal/interictal Single Photon Emission Computed Tomography (SPECT) and/or intracranial EEG. Patients undergoing surgery were followed at 6 months, 1 and 2 years post-operatively; seizure outcome was measured using Modified Engel classification. Results: Of the 122 patients screened, 53 were discussed at the Epilepsy Surgery Meeting based on the presence of continuing seizures, latera...

No abstract

Both Sulthiame and Levetiracetam influence the EEG of children with BECTS. Persistent EEG pathologies are associated with treatment failures.