Summary:Purpose: Epilepsy is a relatively frequent disease in children, with considerable impact on cognitive and social life. Successful epilepsy surgery depends on unambiguous focus identification and requires a comprehensive presurgical workup, including several neuroimaging techniques [magnetic resonance imaging, positron emission tomography (PET), and single-photon emission computed tomography (SPECT)]. These may be difficult to apply in younger or developmentally delayed children or both, requiring sedation, and hence, a significant workforce. Modern electric source imaging (ESI) provides accurate epileptic source-localization information in most patients, with minimal patient discomfort or need for cooperation. The purpose of the present study was to determine the usefulness of ESI in pediatric EEG recordings performed with routine electrode arrays.Methods: Preoperative EEGs recorded from 19 to 29 scalp electrodes were reviewed, and interictal epileptiform activity was analyzed by using a linear source-imaging procedure (depthweighted minimum norm) in combination with statistical parametric mapping.Results: In 27 (90%) of 30 patients, the ESI correctly localized the epileptogenic region. These numbers compare favorably with the results from other imaging techniques in the same patients (PET, 82%; ictal SPECT, 70%). In extratemporal epilepsy, ESI was correct in all cases, and in temporal lobe epilepsy, in 10 of 13 cases. In two temporal lobe patients showing less-accurate ESI results, 128-electrode data could be analyzed, and in both cases, the 128-electrode ESI was correct.Conclusions: ESI with standard clinical EEG recordings provides excellent localizing information in pediatric patients, in particular in extratemporal lobe epilepsy. The lower yield in temporal lobe epilepsy seems to be due to undersampling of basal temporal areas with routine scalp recordings. Key Words: Epilepsy-Pediatric-EEG-Source imaging.The indication for early surgical intervention in children with refractory epilepsy should be actively investigated for two reasons: (a) to avoid the deleterious effect of epileptic seizures and antiepileptic medication on cognitive, intellectual, and social development (1,2); and (b) to increase the chances of postoperative functional reorganization due to the expected higher degree of brain plasticity in children (3-5). The surgical outcome is similar to that in adults (6-8) (i.e., between 60 and 80% of patients with temporal lobe epilepsy are postoperatively seizure free) (8-10). However, in extratemporal epilepsy, which is more frequent in pediatric patients, the outcome is still somewhat less favorable compared with surgical treatment of temporal lobe seizures. Overall, 50-75% benefit from surgical treatment, and evidence converges that the outcome deAccepted January 15, 2006. Address correspondence and reprint requests to Dr. G. Lantz at Functional Brain Mapping Laboratory, Department of Neurology, University Hospital, 24 Rue Micheli-du-Crest, CH-1211 Genève 14, Switzerland. E-mail Goran.Lant...

Extensive intracranial calcifications and leukoencephalopathy are seen in both Coats plus and leukoencephalopathy with calcifications and cysts (LCC; Labrune syndrome). Coats plus syndrome is additionally characterized by the presence of bilateral retinal telangiectasia and exudates while LCC shows the progressive formation of parenchymal brain cysts. Despite these apparently distinguishing features, recent evidence suggests that Coats plus and LCC represent the same clinical entity with a common primary pathogenesis involving a small vessel obliterative microangiopathy. Here, we describe eight previously unreported cases, and present an update on one of the original Coats plus patients to highlight the emerging core clinical features of the "cerebroretinal microangiopathy with calcification and cysts" (CRMCC) phenotype.

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The estimation of the global burden of measles is challenging in the absence of reliable and comparable surveillance systems worldwide. A static model is described that enables estimation of measles morbidity, mortality, and disability for the year 2000 on the basis of country-specific information (i.e., demographic profile, vaccine coverage, and estimates of case-fatality ratios). This approach estimated a global incidence of 39.9 million measles cases, 777,000 deaths, and 28 million disability-adjusted life years. The World Health Organization regions of Africa and Southeast Asia had 70% of incident cases and 84% of measles-related deaths; 11 countries alone (Afghanistan, Burkina Faso, Democratic Republic of the Congo, Ethiopia, India, Indonesia, Niger, Nigeria, Pakistan, Somalia, Uganda) account for 66% of deaths. This approach quantifies the measles burden by considering country-specific indicators, which can be updated, permitting an assessment of country, regional, and global changes in the burden associated with measles infection.The World Health Organization (WHO) estimated that about 875,000 children died of measles in 1999 [1]. This is 56% of all estimated deaths from vaccine-preventable diseases of childhood for that year, making measles the leading cause of vaccine-preventable child mortality. In 1989 the World Health Assembly set specific goals for the reduction in measles morbidity and mortality [2]. The continued commitment and effort is shown in the recent Strategic Plan for Measles Mortality and Morbidity Reduction [3].Because measles is both an epidemic and endemic

In this case report we assess the occurrence of cortical malformations in children with early infantile epilepsy associated with variants of the gene protocadherin 19 (PCDH19). We describe the clinical course, and electrographic, imaging, genetic, and neuropathological features in a cohort of female children with pharmacoresistant epilepsy. All five children (mean age 10y) had an early onset of epilepsy during infancy and a predominance of fever sensitive seizures occurring in clusters. Cognitive impairment was noted in four out of five patients. Radiological evidence of cortical malformations was present in all cases and, in two patients, validated by histology. Sanger sequencing and Multiplex Ligation‐dependent Probe Amplification analysis of PCDH19 revealed pathogenic variants in four patients. In one patient, array comparative genomic hybridization showed a microdeletion encompassing PCDH19. We propose molecular testing and analysis of PCDH19 in patients with pharmacoresistant epilepsy, with onset in early infancy, seizures in clusters, and fever sensitivity. Structural lesions are to be searched in patients with PCDH19 pathogenic variants. Further, PCDH19 analysis should be considered in epilepsy surgery evaluation even in the presence of cerebral structural lesions. What this paper adds Focal cortical malformations and monogenic epilepsy syndromes may coexist. Structural lesions are to be searched for in patients with protocadherin 19 (PCDH19) pathogenic variants with refractory focal seizures.