Iranian Journal of Child Neurology (IJCN)
Volume:4 Issue: 3, Summer 2010

Epilepsy is a common disorder affecting approximately 1% of the population. It is estimated that about 20- 30% of the patients become refractory to proper medical therapies. Such cases are often termed intractable. Intractable epilepsy (IE) is a serious condition in children, leading to significant impairment in quality of life, as well as behavioral and psychiatric problems.In this review, we tried to define intractability, mention the causes of intractable epilepsy and its predictive factors in children, and outline the management and various treatments of intractable epilepsy.

ObjectiveThe aim of the present study was to evaluate the efficacy and tolerability of a modified Atkins diet for intractable childhood epilepsy.Materials & MethodsTwenty one children with medically intractable epilepsy were enrolled in the study. Inclusion criteria were at least four seizures per month and a trial of at least three anticonvulsants without becoming seizure-free. The subjects received the diet over a 6-month period.ResultsThree months after diet initiation, 15 patients (71.4%) remained on the diet and 12 (57.1%) had >50% seizure reduction. Eleven patients (52.4%) completed the 6-month study and 8 (38.1%) chose to remain on the diet afterward. At 6 months, 9 patients (42.8%) had >50% seizure reduction. The diet was more effective in cryptogenic epilepsy (p=0.032). Most complications were transient and successfully managed by careful follow-up and conservative strategies.ConclusionThe modified Atkins diet is an effective and well- tolerated therapy for intractable childhood epilepsy.

ObjectiveNeuropsychological impairment is an important co-morbidity of chronic epilepsy. The aim of this study was to determine the state of the cognitive and motor development of patients with refractory epilepsy.Materials & Methods We studied 150 consecutive children with epilepsy who were referred to Mofid Children Hospital, a third level public referral University Hospital in Tehran, Iran, from October 2007 to October 2008. Refractory epilepsy was defined as therapeutic failure of three antiepileptic drugs which were used appropriately.Data regarding sex, age, age at which the first seizure occurred, microcphaly, muscle tonicity, EEG findings, kind of treatment for controlling seizures and cognitive and motor development delay were collected from medical recordsDevelopment delay was defined as delay in acquiring cognitive ability and motor skills for age according to the Denver Scale II.Results Of 150 patients 72% were younger than 2 years old and 56.7% were male. About 35.3% were microcephalic while 76% had normal muscular tonicity.Only 2.7% had normal EEGs. About 37.3% showed a good response to anticonvulsive drugs and became seizure free, 13.3% showed a relative response to anticonvulsants but 49.3% did not respond. In the present study, 68% had cognitive developmental delay and 60.7% suffering motor delay. There was a significant difference in response to treatment between patients with cognitive and motor development delay.ConclusionCognitive developmental delay was more frequent in patients with refractory epilepsy, suggesting that early cognitive screening and introduvtion of rehabilitation programs are necessary for patients with refractory epilepsy

ObjectiveEpilepsy is reported in 15-90% of the children with Cerebral Palsy (CP) but its clinical course is not well defined.We conducted a retrospective study on children with hemiparetic CP who were referred to Pediatric Neurology Department of Mofid Hospital. The aim of our study was to evaluate the risk factors and prognosis of epilepsy in children with hemiparetic CP.Materials & MethodsWe evaluated 64 children with hemiparetic CP who were referred to Pediatric Neurology Department of Mofid Hospital between 2006 and 2008.According to our protocol, patients were divided into two groups: 34 children in the case group (hemiparetic patients with epilepsy) and 30 children in the control group (hemiparetic patients without epilepsy).ResultsPrenatal, perinatal and postnatal events, maternal age at the time of delivery, socioeconomic status of the family, familial history of epilepsy, neuroimaging findings, side of the hemiparesia and age at diagnosis of hemiparesis were not considered as risk factors for epilepsy in hemiparetic children, but microcephaly, severity of hemiparetic CP and mental retardation were significantly associated with an increased risk of epilepsy in children with hemiparetic CP.ConclusionOur study showed that microcephaly, severity of hemiparesis and mental retardation were risk factors for developing epilepsy in children with hemiparetic CP; furthermore, they had negative effects on rehabilitation outcome in these patients.

ObjectiveStudies on the effect of various antiepileptic drugs on serum lipids show contradictory results. We aimed to find the effect of Phenobarbital and Sodium Valproate monotherapy on serum lipid profile and liver function tests in epileptic children.Materials & MethodsThis cohort study was conducted in Amirkola Children Hospital. One hundred and ten children with epilepsy were included in this study. Children with hepatic or renal disease, those receiving medications which could alter liver function tests or serum lipid profile were excluded from the study. Patients were allocated into two groups. The first group, including 63 patients, received Phenobarbital and the second group, including 47 patients, received Sodium Valproate, both in divided doses. A venous blood sample was collected after overnight fasting to evaluate serum triglyceride, total cholesterol, LDL, HDL, and liver function tests. Data was analyzed with SPSS version 17.ResultsIn children receiving Phenobarbital, total cholesterol, LDL, HDL, ALP, SGOT and SGPT increased significantly after treatment, but TG level showed no significant changes. In children receiving Sodium Valproate, HDL, ALP, SGOT, SGPT significantly increased after treatment but there were no statistically significant changes in total cholesterol, LDL and TG. In our study, the plasma levels of LPa elevated significantly after treatment with Phenobarbital and Sodium Valproate (P Value=0.0001). This increase was more significant in patients receiving Sodium Valproate.ConclusionOur results suggested a need for monitoring serum total cholesterol, HDL, LDL, and TG levels in patients receiving Phenobarbital and Valproic Acid.

ObjectiveNeuromuscular characteristics in Down syndrome result in generalized muscular hypotonia, developmental delays and sensory integration deficits. The aim of this study was to compare the effects of simultaneous sensory stimulations and current occupational therapy approaches on motor functions development of infants with Down syndrome.Materials & MethodsEighteen infants with Down syndrome, aged 6 -18 months, were evaluated in two groups: intervention group (simultaneous sensory stimulation and occupational therapy) and control group (occupational therapy alone). They attended the program 3 times a week for 6 months and each session lasted 45 minutes. Motor functions were assessed before, during, and after intervention in the two groups, using GMFM test.ResultsMean motor function increased in both groups according to the GMFM test (P = 0.000). Comparison of the changes showed that although the mean difference of this variable was higher in the intervention group, the difference was not statistically significant (P = 0.576).Mean motor deficit reduced in both groups during the period of the study,which was statistically significant (P < 0.05). Comparison of the difference in mean motor deficit in the first and last evaluations showed that this difference was more in the intervention group but statistically insignificant (P = 0.617)ConclusionEarly use of simultaneous sensory stimulations can improve the quality of motor skills in Down syndrome infants. It is suggested that it may be used as an early intervention in association with other methods in the rehabilitation of these patients. However, more studies in this regard are warranted.

ObjectiveThe Lennox-Gastaut syndrome (LGS: the triad of intractable seizures of various types, a slow spike-wave pattern in EEG and mental retardation) is one of the most difficult epilepsy syndromes to treat. The aim of this study was to evaluate the efficacy and safety of zonisamide (ZNS) as add-on therapy in seizures of children with LGS.Materials & MethodsIn a quasi- experimental study, seizure frequency and side effects of 40 children with LGS who were referred to the pediatric neurology clinic of Shaheed Sadoughi University of Medical Sciences, Yazd, Iran, between September 2008 and November 2009 and were on ZNS for six months were evaluated.ResultsTwenty one boys and 19 girls with a mean age of 6.6 3.6years were evaluated. At the end of six months of treatment with ZNS, 25% became seizure free, 25% had > 50% reduction in seizure frequency while 35% did not have a notable change in seizure frequency and 15% experienced an increase in seizure frequency.Drug was effective in 62.5% of the myoclonic and generalized tonic-clonic, 50% of the atonic, 43% of the mixed type and 33.4% of the tonic seizures.Transient side effects were seen in 25% of the patients: drowsiness in 10%, hyperthermia in 5 % and irritability, fatigue, ataxia and anorexia (each one) in 2.5% of the patients. No serious side effects were reported.ConclusionZNS could be considered as an add-on therapy in the management of intractable epilepsy in LGS.

ObjectiveElectroencephalogram (EEG) is an easy and non invasive evaluation method for diagnosis and early prognosis in children. Our aim was to assess the association between EEG and the patient's Developmental Quotient (DQ) level in phenylketonuria.Materials & MethodsIn this study, 94 PKU patients (45 boys, 49 girls; mean age: 8.5 6.2 years) who were diagnosed through newborn screening tests or later were followed. PKU was confirmed with a serum phenylalanine concentration above 6 mg/ dl in untreated newborns. The patients were matched in gender, age and phenylalanine level. The ASQ (Age and Stage Questionnaire) was used for evaluation of the developmental stage of the case (abnormal EEG) and control (normal EEG) groups and the Wechsler Intelligence Scale was used to assess cognitive and intellectual abilities. Finally, one way ANOVA and chi square tests were used for analysis and P< 0.05 was considered significant.ResultsThe case group consisted of 63 patients (67%) with abnormal EEGs and the control group consisted of 31 patients (33%) with normal EEGs. In patients with abnormal EEGs, 34 (53%) had mild, nine (14%) had moderate and 20 (33%) had severe EEG changes. Distribution of high and low DQ levels in these three groups show significant difference (p=0.001). Distribution of DQ level in the abnormal and normal EEG patients showed a significant difference (p=0.001).ConclusionThere was no significant difference between phenylalanine level in case and control groups; therefore, EEG findings may affect patient's developmental scores despite a normal phenylalanine level in PKU patients.

This is the first report of CNS involvement by the new influenza virus (influenza A [H1N1]) in Iran. The patient was a 10-year-old boy with chief complaints of fever, malaise, and cranial nerve involvement, resulting in respiratory muscle paralysis and intubation. This shows that the new influenza virus, as well as the seasonal flu, can cause neurologic complications; however, the severity of the signs and symptoms is less and the disease may resolve without complications in the case of seasonal flu. Therefore, in each patient with neurologic involvement and typical influenza signs & symptoms or a flu-like syndrome, diagnostic tests for H1N1 flu virus should be considered, especially during epidemics, and treatment with oseltamivir should be started.