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Alexander disease is a rare, sporadic leukoencephalopathy characterized by white matter abnormalities with frontal predominance and clinically as a rule associated with megalencephaly, seizures, spasticity and psychomotor deterioration. We describe a boy who was diagnosed as affected by anorexia nervosa because his refusal to eat, progressive weight loss and psychological disturbances. The observation of a hyperintense lesion on T2-weighed MR images was initially explained as a pontine and extrapontine myelinolysis related to malnutrition. Following MRI and DNA analysis we diagnosed a juvenile type of Alexander disease.

Background: Status epilepticus is a neurological emergency that require a prompt treatment following steps of more aggressive therapy. We analysed efficacy of treatment protocol in paediatric SE. Methods: We present a retrospective study in 29 patients (age range: 3mm-18yy) affected by status epilepticus (SE) of whom 16 patients had a previous diagnosis of epilepsy (symptomatic or idiopathic) while, in 13 cases, SE was the first epileptic event. Protocol of treatment suggests a first treatment with intravenous Diazepam followed by a bolus of Phenytoin (PHT) in case of non-response. All patients were carefully monitored, electroencephalographic activity (EEG) and vital signs, particularly respiratory depression. When the patients were classified as refractory or suffered from respiratory insufficiency, they were transferred into ICU where anesthetic drugs ( propofol or pentothal) could be administered. Results: Seventeen children were treated in neurological unit: 7 (24%) children responded to intravenous Diazepam. 10 (35%) children required an additional bolus of Phenytoin at the variable dose of 15-20 mg/Kg/bolus. 12 patients (41%) were admitted to Intensive Unit Care (ICU): 3 for respiratory insufficiency and 9 for treatment with anaesthetics drugs. Conclusions: In our experience so as in literature there is no Gold Standard in the treatment of SE. Nearly half of patients needed ICU support thus, the best approach still remains multidisciplinary.

We describe clinical features and Magnetic Resonance Imaging findings of an adolescent affected by Restrictive Anorexia Nervosa. This first report on ischemic lesion in Anorexia Nervosa suggest the importance of brain MRI in order to demonstrate the brain sufferance and to understand how cerebral tissue responds to blood flow droop in AN.

PURPOSE: The aim of the present study was to assess serum lipid levels before and after treatment with oxcarbazepine in children with epilepsy. METHODS: We measured total cholesterol (TC), triglycerides (TGs) and high-density lipoprotein cholesterol (HDL-C) in 28 patients whereas only TC levels in 11 patients, during baseline period and at 3 months after the beginning of therapy with oxcarbazepine. RESULTS: During baseline period, median values were: 4.38 (IQR=4.12-5.03) mmol/L for TC levels, 1.72(IQR=1.42-2.01) mmol/L for HDL-C levels and 1.54 (IQR=1.29-1.96) mmol/L for TGs levels. At three months, median values were: 4.38 (4.10-4.95) mmol/L for TC levels (p

To evaluate the efficacy and tolerability of levetiracetam or oxcarbazepine as monotherapy in children with newly diagnosed benign epilepsy with centrotemporal spikes (BECTS). Twenty-one children (11 males, 10 females), aged between 5 and 13 years (mean 10.5 years), and 18 (10 M, 8 F), aged between 3.3 and 14 years (mean 8.4 years), were randomised to receive monotherapy with levetiracetam or oxcarbazepine, respectively. LEV was titrated up to 20-30 mg/kg/once or twice a day, and OXC up to 20-35 mg/kg once or twice a day. Thirty-nine consecutive children (21 males, 18 females), aged between 3.3 and 14 years (mean 10.7 years), were recruited into the study. Twenty-one were randomised on LEV (11 male, 10 female; mean age 10.5 years), and 18 on OXC (10 male, 8 female; mean age 8.4 years). After a mean follow-up period of 18.5 months (range 12-24 months), 19 out of 21 patients (90.5%) on levetiracetam, and 13 out of 18 (72,22%) on oxcarbazepine did not have further seizures. Mean serum level of LEV was 4.1 microg/ml (range 1.3-9.0), and of OXC was 15.2 microg/ml (range 4.2-27.5). Adverse side effects on LEV were reported in 3 children (14.3%), represented by mild and transient decreased appetite (2) and cephalalgia (1). They were reported on OXC in 2/18 (11.1%), including headache (1), and sedation (1). These preliminary data from an open, parallel group study suggest that levetiracetam and oxcarbazepine may be potentially effective and well tolerated drugs for children with BECTS who require treatment.

Levetiracetam is a new antiepileptic drug reported to be effective and well-tolerated in adults and children affected by epilepsy. Its lack of hepatic cytochrome metabolism is the theoretic basis for the absence of interactions with other drugs that follow this pathway. We present a 14-year-old girl who underwent orthotopic heart transplantation, followed by antirejection therapy including cyclosporine. Symptomatic occipital lobe epilepsy developed that was successfully treated with oxcarbazepine, but cyclosporine plasma levels decreased to below the antirejection threshold. Oxcarbazepine was replaced by levetiracetam. Levetiracetam did not affect the metabolism of cyclosporine, and cyclosporine plasma levels have remained in the therapeutic range up to now. The patient is still seizure-free and does not complain of any side effects after a 1-year follow-up. Further studies are necessary to confirm the lack of interactions between these drugs, which would make levetiracetam a useful therapeutic option in managing seizure control during antirejection therapy with cyclosporine.