Iranian Journal of Child Neurology (IJCN)
Volume:5 Issue: 2, Spring 2011

This review summarizes some patterns of pre-surgical evaluation of epilepsyin children with medically refractory seizures. Epilepsy surgery is a valuabletherapeutic option for many children with intractable epilepsy. The most effectivetreatment for intractable partial epilepsy is a focal cortical resection with excisionof the area of ictal onset and initial seizure propagation (the epileptogenic zone).EEG monitoring continues to prove indispensable in pre-surgical planning ofrefractory epileptic patients by defining the epileptogenic zone. Technologicalprogresses in both structural and functional neuroimaging techniques have ledmany medical centers to consider surgical treatment of epilepsy. In childrenundergoing pre-surgical evaluation, the goals of neuroimaging studies includethe identification of structural abnormalities in the brain, eloquent regions of thebrain including language, memory, and sensorimotor functions, and the relationof these regions to the potential epileptogenic region. Neuropsychological testingplays a crucial role in assessing the potential impact of surgery on cognitivefunction of the patients and helps in lateralizing the cerebral hemispheredominant for verbal and nonverbal function in older children. The Wada testis an invasive procedure to determine language dominance and can be usedto assess the risk of postoperative memory deficits in children with temporallobe epilepsy. Some children scheduled for resection still need to undergofurther precise localization of the epileptogenic zone and functional mappingwith invasive EEG monitoring through intracranial subdural grid and/or depthelectrodes. Epilepsy surgery has the potential of changing the life quality ofchildren by improving or eliminating seizures in carefully selected patients.

ObjectiveThe primary objective of this study was to evaluate pediatric residentsknowledge about the milestones of growth and development in chlidren.Materials and MethodsSeventy-one pediatric residents from 3 main Children's Centers in Tehran weresurveyed to evaluate their knowledge on growth and development of children.Included in the survey was based upon filling up a questionnaire containingquestions about four different categories of development (motor, cognitive,speech and social interactions). The scores that the residents got from the testwere summed up and were compared together.Results71 resident participants completed the questionnaire. The distribution ofresidents by year was 26 post graduate year 1 (PGY1), 26 PGY2, and 23 PGY3.The knowledge of 14 (19.7%) of the participants about child's development wasranked as "Less than expected", 47 (66.2%) as "Unacceptable", 10 (14.1%) as"Acceptable" and none (0%) as "Ideal", according to the total score they gotfrom their correct answers to the questions.ConclusionThis study describes that most pediatric residents lack the acceptable knowledgeon children's growth and development. It seems that their knowledge did notimprove through their years of residency.

In this study, we investigated the changes of the serum levels of thyroidhormones including Thyroxine (T4), Triiodothyronine (T3), T3 resin uptake andThyroid stimulating hormone (TSH) in epileptic children during treatment withanti-epileptic drugs (AEDs) including carbamazepine (CBZ), primidone (PRM),phenobarbital and valproic acid (VPA).Materials and MethodsThis study consisted of four case-series comparisons, was conducted on 115epileptic children (37 girls and 78 boys with an age range between 2 monthsand 15 years, mean: 62.06 ± 44.97 months). These children were divided into4 groups who took either phenobarbital (n=29), PRM (n=28), CBZ (n=29), orVPA (n=29) for 3 months. Thyroid hormone levels (T3, T3 resin uptake, T4 andTSH) were measured at the beginning and three months after starting the study.ResultsAt first, all patients were euthyroid and there were no clinical or laboratoryfindings suggestive of hypothyroidism. Regarding thyroid hormones before andafter the administration of phenobarbital, carbamazepine, valproic acid andprimidone, there were no significant changes in serum T3, T4, T3 resin uptakeand TSH levels.ConclusionOur findings showed that short term therapy with phenobarbital, carbamazepine,valproic acid and primidone had no effect on thyroid function etsts.

ObjectiveInfantile Spasms (IS) is one of the catastrophic epileptic syndromes of infancy.The purpose of this study was to evaluate clinical efficacy and safety oftopiramate (TPM) as the first -line drug in the treatment of infantile spasms.Materials and MethodsIn a quasi- experimental study, efficacy and safety of TPM in rteatment of fortychildren with IS who were referred to pediatric neurology clinic of ShahidSadoughi University of Medical Sciences in Yazd, Iran, from September 2008to 2010 was evaluated.ResultsTwenty two girls (55%) and 18 boys (45%) with a mean age of 9.2 ± 3.9 months(range= 3-20 months) were evaluated. Ninety percent of the patients hadsymptomatic IS. At the end of three months of TPM treatment, 40% of thepatients became seizure free, 27.5% had more than 50% reduction in seizurefrequency, 27.5% had no notable change in seizure frequency and 5% had anincreased frequency of seizures.Transient and mild side effects, which were seen in 32.5% of the patients,included drowsiness in 15%, hypotonia and hyperthermia (each one) in 7.5%and anorexia and weight loss in 2.5%. All side effects disappeared in two orthree weeks of treatment.ConclusionTopiramate is an effective and safe drug in the treatment of IS and could beconsidered as the first line of treatment.

Objective Febrile convulsion (FC) is a common cause of seizure in young children, with an excellent prognosis. In addition to genetic predisposition and infections,FCs are generally thought to be induced by metabolic and elemental changes during fever such as Zinc (Zn) deficiency. Regarding the high prevalence of febrile convulsions and the role of Zn deficiency, we investigated the role of Zn in FC patients in Isfahan, Iran. Materials and Methods In a controlled cross sectional study, 90 patients aged 9 months to 5 years were studied in a period of 12 months. They were assigned to three groups. Thirty patients were included in the Febrile Seizure group, thirty febrile children without convulsion or previous history of convulsion were included in the febrile group and thirty afebrile healthy ones were enrolled as controls. Venous blood was obtained and Zn concentration in serum was measured using Graphite Furnance Atomic Absorbance Spectrophotometering (GF-AAS). Results Patients and the control groups had no difference in either mean age or sex distribution. No significant relationship was observed between serum Zn level and age or sex among patients in the FC group and two other control groups. Conclusion Our findings showed that Zinc level was significantly lower in the febrile seizure group compared to two other groups. We tried to categorize various conditions in a more practical form. Also, Zinc is in close relationship with socioeconomic level of the individuals which was well considered in the current survey.

ObjectiveClub-foot is one of the most prevalent congenital anomalies of the lowerextremities. Since there is no epidemiologic study on the prevalence of thisdisease in Iran, we decided to assess it in a sample population in Tehran.Materials and MethodsWe assessed all neonates who were referred to the emergency department ofmofid children hospital between October 2007 and November 2008, due to apaediatric emergency problem. None of the patients had chief complaints oflower extremity deformity.ResultsDuring this time period (13 months), we could examine 682 neonates. Noneof the parents of these neonates had complaints regarding anomaly of lowerextremities of their neonates at the time of the emergency referral. Of them, 371(54%) were female and 311 (46%) were male. The age of the mother at the timeof pregnancy was < 20 yr in 124 (18%) neonates, between 20 and 35 yr in 472(69%) neonates and > 35 yr in 86 (13%) neonates. There was a previous historyof clubfoot in the siblings of one of the neonates who was under orthopaedictreatment.Among all these neonates, we found two cases of clubfoot (0.3%), with bilateralinvolvement. In one of these cases, the older sibling also had clubfoot.ConclusionThe incidence of clubfoot has been reported between 0.39 and 6 cases in 1000live birth in the literature. In the present study, we found a prevalence of 0.3 forclubfoot in every 1000 neonates.

Rubinstein-Taybi Syndrome is a rare genetic disorder with characteristic featuresincluding downward slanting palpebral fissures, broad thumbs and halluces,and mental retardation. Systemic features may involve cardiac, auditory,ophthalmic, endocrine, nervous, renal and respiratory systems. This syndromeis sporadic in nature and has been linked to microdeletion at 16p 13.3 encodingCREB-binding protein gene (CREBBP). We report a 15-years-old girl, a knowncase of chronic renal failure, with downward slanting palpebral fissures towardthe ears, hypertelorism, short stature, beaked nose, micrognathia, strabismus,dental anomalies, large toes, broad thumbs, and mental retardation.