Iranian Journal of Child Neurology (IJCN)
Volume:10 Issue: 1, Winter 2016

Febrile seizure is the most common neurologic problem in children between 3 months to 5 years old. Two to five percent of children aged less than five yr old will experience it at least one time. This type of seizure is age dependent and its recurrence rate is about 33% overalls and 50% in children less than one yr old.The prophylactic treatment is still controversial, so we conducted a randomized controlled clinical trial to find out the effectiveness of continuous phenobarbital versus intermittent diazepam for febrile seizure. This clinical trial was conducted in the Department of Pediatric Neurology, Babol University of Medical Sciences, Babol, Iran between March 2008 and October 2010. All children from 6 month to 5 yr old referred to Amirkola Children’s Hospital, Babol, Iran were enrolled in the study. Children with febrile seizure that had indication for prophylaxis but did not receive any prophylaxis previously were enrolled in the study. For prophylactic anti convulsion therapy, patients were divided randomly in two groups. One group received continuous phenobarbital and another treated with intermittent diazepam whenever the children experienced an episode of febrile illness for up to one year after their last convulsion. Of all 145 studied cases, the recurrent rate in children under prophylaxis with diazepam was 11/71 and in phenobarbital group was 17/74. There was no significant difference in the recurrence rate in both groups. There was no significant difference in the effectiveness of phenobarbital and diazepam in prevention of recurrent in febrile seizure and we think that in respect of lower complication rate in diazepam administration, it cloud be better choice than phenobarbital.

Breath holding spells (BHS) are common involuntary reflexes in infancy and early childhood. Differential diagnosis should embrace Long QT Syndrome (LQTS) and paroxysmal abnormalities of rhythm. The aim of this study was to compare QT dispersion (QTd) in children with breath holding spells and normal controls. QT dispersion and Corrected QT(QTc) dispersion were measured in 12 lead surface electrocardiograms in 56 patients with BHS and compared with healthy children of the same age referred to the clinic for regular checkup visits. The most common type of BHS was cyanotic (83.9%). Seven patients (12.5%) had pallid and two patients (3.5%) had mixed spells. There was a history of breath holding spells in 33.9% of the children. QT dispersion was 61.6± 22.5 and 47.1±18.8 ms in patient and control groups, respectively. QTc dispersion (QTcd) was 104 ± 29.6 and 71.9 ±18.2 ms, respectively. There was a significant difference between patient and control groups in terms of QTd and QTcd (P<0.001). QTd and QTcd were increased in children with BHS. Therefore, the evaluation of EKG for early diagnosis of rhythm abnormalities seems reasonable in these children.

Due to excessive production of free radicals and antioxidants evolved mechanisms against oxidative stress, infants are very vulnerable. As there was a significant relation between antioxidant levels and birth weight, we aimed verify this relationship. In this descriptive analytical study we evaluated the antioxidant status of 40 healthy term newborns (gestation age 38-42 wk) with weight >2500 g (AGA) and 40 healthy term newborns (gestation age 38-42 wk) with LBW babies (weight < 2500 g) (SGA) in Ardabil Buali Hospital, Ardabil, northwest Iran in 2014. About 15 Ml of cord blood was collected after the second stage of labor.The levels of vitamin A, E, and C, catalase, glutathione peroxidase (GPX), bilirubin and serum uric acid were measured by standard methods. Informed consent was obtained from newborn mothers and study protocol was approved by university Ethics Committee. Data were analyzed using SPSS.19. The mean levels of bilirubin, vitamin C, E, catalase and GPX in AGA group were significantly higher than SGA group but the mean of serum uric acid in SGA group was more than AGA. In addition, the mean of vitamin A was similar in two groups. There was a significant relation between antioxidant levels and birth weight in term newborns. In line with other studies the amounts of antioxidant levels except serum uric acid in AGA group was significantly more than SGA group.

Cerebral palsy (CP) is a non-progressive CNS disorder due to an insult to the growing brain, usually occurring in the first two years of life. During the recent years, its etiology has been changed; perinatal and postnatal insults are not considered as its main causes in developed countries any more. The aim of this study was to evaluate the causes of CP in children in southern Iran. Overall, 200 children with CP aged 1-12 yr old referring to Pediatric Neurology Clinic affiliated to Shiraz University of Medical Sciences, Shiraz, Iran between 2012 and 2013 were enrolled. In addition, 200 healthy age and sex-matched children were considered as the control group. Exclusion criteria were isolated movement disorders with no other evidence of CP, progressive neurologic disorders, metabolic disorders, and incomplete or uncertain past history. After collecting the data on pregnancy period, prenatal history and past medical problems, they were analyzed with appropriate statistical methods. Maternal age, medical problems during pregnancy period, route of delivery, head circumference at birth, neonatal admission, neonatal jaundice, and prematurity were the main risk factors for CP. The distribution of risk factors of CP is different from that of developed countries in our region. Pre- and peri-natal etiologies are still among the common causes of CP in Iran.

Perinatal asphyxia may result in hypoxic damage in various body organs, especially in the central nervous system. It could induce cascade of biochemical events leading to the cell death and metabolic changes, eventually may increase plasma ammonia levels. The purpose of this study was to determine the prevalence of hyperammonemia in neonates with asphyxia and to find the relationship between ammonia levels and severity of asphyxia. In this cross-sectional study, we included 100 neonates with perinatal asphyxia in the Neonatal Intensive Care Unit of Ali-Asghar Hospital, Iran University of Medical Science, Tehran, Iran in 2010-2011. All full term patients diagnosed of asphyxia were enrolled. The relationship between plasma ammonia levels and sex, gestational age, birth weight and severity of asphyxia were determined.Data were analyzed using SPSS software. Fifty six percent of neonates were male. The mean gestational age was 38.0± 1.2 wk. Mean plasma ammonia level was 222 ± 100 μg/dl and 20% of the neonates had hyperammonemia. It was not associated with gender, gestational age, birth weight, and asphyxia severity. Six patients died and mean plasma ammonia levels was 206±122 μg/dl. In this group, there was no significant relation between plasma ammonia levels and severity of asphyxia. No significant different was seen between plasma ammonia in dead and lived neonates. According to high prevalence of hyperammonemia in neonatal asphyxia, measurement of plasma ammonia levels, is suggested to improve management of asphyxia.

We aimed to assess the effect of body mass index (BMI) on reducing the risk of refractory seizure due to lipoid tissue factors. This matched case-control study, consisted of cases (Patients with refractory epilepsy) and controls (Healthy children) referred to 17 Shahrivar Hospital, Guilan University of Medical Sciences, Guilan, Iran during 2013-2014. Data were gathered by a form including demographic characteristics, type of epilepsy, predominant time of epilepsy, therapeutic approach, frequency of epilepsy, time of disease onset and anthropometric indices. We measured anthropometric indices and transformed them into Z-scores. Data were reported by descriptive statistics (mean and standard deviation) and analyzed by Pearson correlation coefficient, paired t test and multinomial regression analysis test using SPSS 19. There was no significant difference between sex groups regarding anthropometric indices. Generalized and focal types of epilepsies were noted on 57.5% and 38.75% of patients, respectively. Daytime epilepsies happened in 46.25% of patients and 33.75% noted no predominant time for epilepsies.Clinicians indicated poly-therapy for the majority of patients (92.5%). The most common onset times for epilepsies were 36-72 months for 32.5% of patients. Lower onset time indicated lower frequency of refractory epilepsies.Although, there was significant difference between Zheight and predominant time of epilepsies but no significant relation was found between types of epilepsies and frequency of epilepsies with anthropometric indices. Using multivariate regression analysis by backward LR, Zweight and birth weight were noted as the predicting factors of refractory epilepsies. This effect may be because of leptin. Therefore, researchers recommend further investigations regarding this issue in children with epilepsy.

Simple Febrile Seizure (SFS) is the most common seizure disorder in childhood, and is frequently described as inoffensive disorder. Nevertheless, there is evidence suggesting the association between neonatal febrile seizures and hippocampal abnormalities in adulthood. This study was conducted at evaluating the hippocampal expression of pro-apoptotic Bax and anti-apoptotic Bcl-2 proteins following SFS induction in rat neonates. Febrile seizure was modeled by hyperthermia-induced seizure in 22-dayold male rats by a hot water bath. The animals were divided into two groups based on the presence or absence of seizure behaviors: Hyperthermia without seizure (n=10) and hyperthermia with seizure (n=10). To control the effects of environmental stress a sham-control group was also added (n=10). The rats’ hippocampi were dissected 2 or 15 days after hyperthermia. The expression of Bax and Bcl-2 proteins were measured using Western Blotting technique. The hippocampal expression of Bcl-2 protein was significantly lower in the hyperthermia with seizure animals than that of the sham-control and hyperthermia without seizure groups. The expression of pro-apoptotic Bax protein also significantly increased in the hippocampus of hyperthermia with seizure group rats compared to the sham-control and hyperthermia without seizure animals. The simple febrile seizure markedly disturbed the hippocampal expression of both Bcl2 and Bax proteins, resulting in apoptosis promotion in hippocampi of juvenile rats, which were measurable for at least 15 days.

Adrenoleukodystrophy disorder is one of the x-linked genetic disorders caused by the myelin sheath breakdown in the brain. In this study, we present 4 yr experience on this disorder. The patients diagnosed as adrenoleukodystrophy in the Neurology Department of Mofid Children’s Hospital in Tehran, Iran from 2010 to 2014 were enrolled into the study. The disorder was confirmed by neuroimaging and clinical findings along with genetic and neurometabolic assessment at Reference Laboratory in Germany. We assessed age, gender, past medical history, developmental status, clinical manifestations, and neuroimaging findings of populous family with adrenoleukodystrophy. All of the patients were one populous family with high rate of consanguineous: marriages. This disorder was confirmed by genetic assessment, VLCFA and brain MRI.c.253_254insC, p.R85Pfs112* was found in heterozygote state and the VLCFA assessment showed the typical pattern for adrenoleukodystrophy/ adrenomyeloneuropathy. This diagnosis was in agreement with the family history and the clinical history of the patient. Since there have been a number of cases in patient’s family in the past, so intensive follow-up on the family especially detection the female members of the family of childbearing age was recommended. The amount of C-26, C24/C22 and C26/C22 was elevated. All patients with the same genotype had wide ranges of clinical presentation. Early diagnose of this disease might help us for early intervention and prenatal diagnosis for the disease in next siblings.

Guillain-Barre syndrome (GBS) is the most common cause of acute weakness in children. It has multiple variant forms with different presentations. A rare initial sign is ptosis. In this study, we present a 10-year-old girl with bilateral ptosis without opthalmoplegia followed by a weakness in extremities with a favourable response to intravenous immunoglobulin. Due to the patient’s initial eyelid levators, myasthenia gravis was ruled out by a Tensilon test and electrophysiological studies. Our report highlights the possibility of GBS as a cause of isolated ptosis, especially in cases without ophthalmoplegia.

characterized with decreased compound muscle action potentials (CMAP) and absence of demyelinating findings in electrophysiological studies, is a subtype of Guillain-Barre Syndrome (GBS). A 4 yr-old male patient presented with ascending weakness, dysarthria and dysphagia to İstanbul Dr. Lütfi Kırdar Kartal Training and Research Hospital Neurology outpatient for three days to in 2012. Dysphonia, restricted eye movements, flaccid tetraplegia and areflexia were found in neurological examination. There were motor conduction blocks in all peripheral nerves in electrophysiological studies.According to these findings the patient was diagnosed as Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP). Reduction of CMAP amplitudes in posterior tibial nerve, absence of CMAPs in median, ulnar and peroneal nerves and loss of motor conduction blocks were found in following electrophysiological studies. According to these findings, patient was diagnosed as AMAN. Motor conduction blocks may appear in early stage of AMAN and they disappear in later examinations.That’s why electrophysiological studies must be repeated in patients with GBS.