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The majority of epileptic seizures are controlled with antiepileptic drugs (AEDs). Whether or not to start treatment following an initial unprovoked seizure should be decided on an individualized basis; and, the risk of seizure recurrence versus possible side-effects and social consequences of chronic AED treatment should be evaluated. Main risk factors for seizure recurrence can be summarized as the presence of focal seizure types, organic brain lesion, focal neurologic abnormality, previous neurodevelopmental retardation, specific EEG abnormality and positive family history of epilepsy. The AED chosen for initial therapy should be one that is highly effective for a particular seizure type and epilepsy syndrome, as also, that is safe and well tolerated. Monotherapy is the goal in epilepsy treatment. If the first two monotherapies fail, combinations of AEDs should be tried. Withdrawal of AED therapy should be considered in most children after two years without seizures. AEDs should be tapered slowly rather than halted abruptly. [ABSTRACT FROM AUTHOR]

Objective: This study has planned to investigate the role of electroencephalography (EEG) and cranial magnetic resonance imaging (cMRI) in the evaluation and diagnosis of neurological disorders combined with autism in children. Method: A total number of 121 autistic children ranging from 3 to 18 years of age and who had applied to our hospital's clinics between January 2010 and January 2011 were included. The sociodemographic properties, time of birth, birth history, weight at birth, age at onset of walking and language development were investigated. By means of a reevaluation of cMRI, sleep EEG and other examination findings, additional neurological diagnoses, if any were recorded. Children for whom, a cMRI/EEG evaluation was carried out and for whom such an evaluation was not carried out was compared statistically as to certain risk factors separately for additional diagnoses and neurological disorders. In addition, the relationship between cMRI and EEG findings and additional neurological disorder was examined. Results: Autistic children (Male/Female: 92/76) aged 9.30±4.2 years have been diagnosed neurological disorder additionally with a percentage of 40%. The most common of these was the epileptic seizure as 33%. No data was obtained about an additional neurological disorder in 22% of cases who performed cMRI and in 34% of cases who performed EEG. Ratio of presence of a pathological finding in cMRI was high in patients with cerebral palsy, whereas it was not found to be meaningful in patients with epileptic seizures. Analyzing risk factors for neurological disease, birth history of perinatal problems and gait disorders were seen more frequently in patients who required cMRI as compared to those in patients who did not require cMRI. Gait age was older in patients who required cMRI (18±8 months) as compared to patients without cMRI (14±4 months). Conclusion: In our autistic children, cMRI and EEG examinations had been extensively used for the investigation of additional neurological disorders. Both cMRI and EEG examinations had been requested more frequent in the presence of perinatal problems. The other risk factors for additional neurological disorders were history of premature birth to request EEG and older age at onset of walking, walking problems to request cMRI. However with detailed clinic evaluation of autistic children and clarification for investigations criteria unnecessary EEG and cMRI should be avoided. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to investigate health-related quality of life (HRQoL) in children with neurofibromatosis type 1 (NF1) and to determine the factors affecting HRQoL in these children, with particular emphasis on NF1-specific findings and complications. Materials and Methods: The patient group included 60 NF1 patients aged 3-18 years that were evaluated at our pediatric neurology outpatient clinic between January 2001 and January 2011. The control group included 96 age-matched patients without chronic disease and whose mothers had similar levels of education as the mothers of those in the patient group. All the mothers completed the Pediatric Quality of Life Inventory PedsQoL-Parent Form and the PedsQoL scores in the 2 groups were statistically compared. In addition, the effects of sociodemographic variables, as well as NF1-specific findings and complications (skin findings, neurofibromas, Lisch nodules, arterial hypertension and/or cardiac pathology, short stature, macrocephaly, orthopedic problems, hyperintense lesions on cranial MRI, epileptic seizures, psychiatric disease, and cognitive involvement) were statistically analyzed. Results: All PedsQoL domain scores were lower in the patient group than in the control group (P = 0.0001). HRQoL decreased, and the number of NF1-specific findings and complications increased as age increased in the patient group (P = 0.013). Short stature, neurofibromas and/or plexiform neurofibromas, bone lesions, and psychiatric problems were factors specifically related to low-level HRQoL in the NF1 patients. Conclusion: NF1 negatively affected HRQoL in the patient group. We think that multidisciplinary evaluation and, in particular, psychiatric management of NF1 patients are necessary to improve patient HRQoL. [ABSTRACT FROM AUTHOR]

A 3-month-old boy with early myoclonic epilepsy owing to nonketotic hyperglycinemia developed acute encephalopathy after - vigabatrin was presented. Due to the use of vigabatrin, the elevated gamma-aminobutyric acid (GABA) concentration in the brain together with the elevated levels of glycine enhanced the encephalopathy. Our observations indicate the risk of using vigabatrin in patients with nonketotic hyperglycinemia. [ABSTRACT FROM AUTHOR]

Neuroleptic malignant syndrome is a rare, but potentially fatal idiosyncratic reaction to antipsychotic drugs that manifests itself consistently with hyperthermia, muscle rigidity, akinesia, altered mental state, autonomic instability, high creatine phosphokinase levels, and leukocytosis. We report here a case of risperidone-induced neuroleptic malignant syndrome in a 2.5-year-old boy who was given by mistake 2.5 mg risperidone instead of 0.25mg, as it is the youngest case reported in the literature. [ABSTRACT FROM AUTHOR]

Aim: In this study; clinical and laboratory findings of 15 cases with benign childhood acute myositis are presented to look over pathognomonic findings of the disease. Material and Method: Fifteen typical cases with benign childhood acute myositis referred to our Pediatric Neurology Clinic because of inability to walk, from 15th of January to 15th of March 2011, were enrolled into this study. Eighty percent of cases were male and their mean age was 6.3 years. Guillian-Barre's syndrome was the most common initial diagnosis (47% of the cases). Results: Prodromal period lasted 2- 10 days and common symptoms were sore throat, fever and cough. When they were referred to our clinic, the patients complained of pain in both calves and inability to walk. In three days period; the symptoms resolved fast and spontaneously. Leucopenia at 87%, thrombocytopenia at 47%, mildly high alanine transaminase at 87% of the patients were detected. Serum creatinin kinase level ranged from 580 to 8250 U/L and it regressed to normal level within one week. Conclusions: In the patients of whom clinical and laboratory findings are suggestive of benign childhood acute myositis, it is important to think early diagnosis, which prevents unnecessary laboratory evaluations and therapeutic approaches. [ABSTRACT FROM AUTHOR]

Aim: In this study; clinical and laboratory findings of 15 cases with benign childhood acute myositis are presented to look over pathognomonic findings of the disease. Material and Method: Fifteen typical cases with benign childhood acute myositis referred to our Pediatric Neurology Clinic because of inability to walk, from 15th of January to 15th of March 2011, were enrolled into this study. Eighty percent of cases were male and their mean age was 6.3 years. Guillian-Barre's syndrome was the most common initial diagnosis (47% of the cases). Results: Prodromal period lasted 2- 10 days and common symptoms were sore throat, fever and cough. When they were referred to our clinic, the patients complained of pain in both calves and inability to walk. In three days period; the symptoms resolved fast and spontaneously. Leucopenia at 87%, thrombocytopenia at 47%, mildly high alanine transaminase at 87% of the patients were detected. Serum creatinin kinase level ranged from 580 to 8250 U/L and it regressed to normal level within one week. Conclusions: In the patients of whom clinical and laboratory findings are suggestive of benign childhood acute myositis, it is important to think early diagnosis, which prevents unnecessary laboratory evaluations and therapeutic approaches. [ABSTRACT FROM AUTHOR]

Benign familial neonatal convulsions (BFNC) is a rare monogenic subtype of idiopathic epilepsy exhibiting autosomal dominant mode of inheritance. The disease is caused by mutations in the two homologous genes KCNQ2 and KCNQ3 that encode the subunits of the voltage-gated potassium channel. Most KCNQ2 mutations are found in the pore region and the cytoplasmic C domain. These mutations are either deletions/insertions that result in frameshift or truncation of the protein product, splice-site variants or missense mutations. This study reveals a novel missense mutation (N258S) in the KCNQ2 gene between the S5 domain and the pore of the potassium channel in two BFNC patients in a Turkish family. The absence of the mutation both in the healthy members of the family and in a control group, and the lack of any other change in the KCNQ2 gene of the patients indicate that N258S substitution is a pathogenic mutation leading to epileptic seizures in this family. [ABSTRACT FROM AUTHOR]

Objectives: Systemic lupus erythematosus (SLE) is a chronic inflammatory disease characterised by the presence of various autoantibodies. Mild cognitive impairment developing in patients without significant neuropsychiatric (NP) symptoms was thought to be the result of immune-mediated myelinopathy. We aimed to determine the role of myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in the neurological manifestations of childhood-onset SLE (cSLE) and if there is a correlation between various metabolite peaks in magnetic resonance spectroscopy (MRS) and myelinopathy., Methods: MOG-Ab levels were studied in all healthy subjects (n=28) and in all patients with (NPSLE=9) and without (non-NPSLE=36) overt neuropsychiatric manifestations. Twenty patients (all had a normal-appearing brain on plain magnetic resonance) in non-NPSLE and 20 subjects in healthy group met the MRS imaging standards for evaluation in which normal appearing brain on plain MR., Results: A total of 45 cSLE (36 non-NPSLE and 9 NPSLE) subjects and 28 healthy children were recruited to the study. The mean age of the SLE patients at study time was 16.22±3.22 years. MOG-Ab was not detected in cSLE or in healthy group. There was no significant difference between the non-NPSLE group and healthy subjects in terms of choline, N-acetyl aspartate (NAA), creatine, NAA/creatine, and choline/creatine., Conclusions: There was no association of MOG-Ab with cSLE, whether NP manifestations were present or not. A causal relationship between immune-mediated myelinopathy and cognitive impairment could not be suggested, since there has been no patient with positive MOG-Ab and there has been no difference in choline, choline/creatine between groups.