SUMMARYThe ketogenic diet (KD) is an established, effective nonpharmacologic treatment for intractable childhood epilepsy. The KD is provided differently throughout the world, with occasionally significant variations in its administration. There exists a need for more standardized protocols and management recommendations for clinical and research use. In December 2006, The Charlie Foundation commissioned a panel comprised of 26 pediatric epileptologists and dietitians from nine countries with particular expertise using the KD. This group was created in order to create a consensus statement regarding the clinical management of the KD. Subsequently endorsed by the Practice Committee of the Child Neurology Society, this resultant manuscript addresses issues such as patient selection, pre-KD counseling and evaluation, specific dietary therapy selection, implementation, supplementation, follow-up management, adverse event monitoring, and eventual KD discontinuation. This paper highlights recommendations based on best evidence, including areas of agreement and controversy, unanswered questions, and future research.

We report the identification of a recurrent 520-kbp 16p12.1 microdeletion significantly associated with childhood developmental delay. The microdeletion was detected in 20/11,873 cases vs. 2/8,540 controls (p=0.0009, OR=7.2) and replicated in a second series of 22/9,254 cases vs. 6/6,299 controls (p=0.028, OR=2.5). Most deletions were inherited with carrier parents likely to manifest neuropsychiatric phenotypes (p=0.037, OR=6). Probands were more likely to carry an additional large CNV when compared to matched controls (10/42 cases, p=5.7×10-5, OR=6.65). Clinical features of cases with two mutations were distinct from and/or more severe than clinical features of patients carrying only the co-occurring mutation. Our data suggest a two-hit model in which the 16p12.1 microdeletion both predisposes to neuropsychiatric phenotypes as a single event and exacerbates neurodevelopmental phenotypes in association with other large deletions or duplications. Analysis of other microdeletions with variable expressivity suggests that this two-hit model may be more generally applicable to neuropsychiatric disease.

Background: There are few studies on neonatal cerebral sinovenous thrombosis (SVT). Objectives: To describe the presentations, treatments, and outcomes of neonatal SVT and to assess infarction as a predictor of outcome. Design: Retrospective chart study. Setting: A tertiary pediatric hospital in Indianapolis, Ind. Patients: Forty-two children with neonatal SVT identified using International Classification of Diseases, Ninth Revision code searches from 1986 through June 2005 and review of neurology clinic records. Interventions: None. Main Outcome Measures: Cognitive impairment, motor impairment, and epilepsy at last clinic visit. Results: Gestational or delivery complications or risk factors and comorbid conditions such as dehydration, sepsis, and cardiac defects were common (gestational/ delivery factors in 82% [31 of 38 with available maternal data]; comorbid conditions in 62% [26 of the 42]). Twenty-four (57%) presented with seizures. Twentyfive (60%) had infarcts, which were hemorrhagic in 22. Only 27 (64%) of 42 received prothrombotic evaluations; none had persistent deficiencies of protein C, protein S, or antithrombin III. Three (7%) received heparin sodium. All other children received only supportive care. One child died. Outcome data were available for 29 (71%) of the 41 survivors; of these, 23 (79%) had impairment(s). Two were known to be in early intervention, and no further information was available. Of the remaining 27, 16 (59%) had cognitive impairment, 18 (67%) had cerebral palsy, and 11 (41%) had epilepsy. Infarction was associated with the presence of later impairment (P=.03). Conclusions: The presentation of neonatal SVT is often nonspecific, the diagnosis can be difficult to make, treatment beyond supportive care is rarely used, and outcomes can be severe. Further work is needed to develop standardized guidelines for the evaluation and treatment of neonatal SVT.

SUMMARYPurpose: To determine if the clinical characteristics of nonepileptic seizures (NES) are different in children younger than 13 years age as compared to adolescents. Methods: Retrospective review of medical records and video-EEGs (VEEG) of all patients with NES confirmed on VEEG monitoring was performed. Results: Sixty-eight (3.5%) of 1,967 patients monitored with VEEG had a clinical diagnosis of NES. Fifty-nine of 68 patients had their habitual event recorded. Mean age at the time of the VEEG diagnosis was 13 years 4 months. Twenty-two patients were less than 13 years (group A) and 37 were 13 years and older (group B). The male to female ratio was equal in group A, with female predominance seen in group B. NES commonly manifested as subtle motor activity in group A (p < 0.01) and prominent motor activity in group B (p < 0.001). Difficulties at school, family discord, and interpersonal conflicts, were frequent stressors in both groups. Sexual abuse was the least frequent. Depression was more common in group B; cognitive dysfunction (p < 0.001) and epilepsy (p < 0.01) were more common in group A. Conclusions: Differences in clinical semiology and predisposing factors may help identify young children and adolescents who might be at risk for the development of NES. KEY WORDS: Pseudoseizures-Epilepsy-Video-EEG.Nonepileptic seizures (NES) are nonepileptic events that may resemble epileptic seizures, but are not associated with abnormal cortical electrical discharges. They should be suspected whenever the attacks are frequent despite appropriate medical management, have atypical clinical features and are exacerbated by stress and when EEGs are repeatedly normal. A variety of terms have been used in the literature to describe these events, including pseudoseizures, psychogenic seizures, hysterical epilepsy, pseudoepileptic seizures, and nonphysiologic or functional seizures. We prefer to use the term NES, because it is more neutral. NES have been described extensively in the literature mainly in adults and less frequently in chil-

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