Iranian Journal of Child Neurology (IJCN)
Volume:12 Issue: 4, Autumn 2018

Macrocephaly is one of the most frequent reasons for referral to a pediatric neurologist. Benign enlargement of subarachnoid space (BESS) in infancy is the most common cause of macrocephaly and characterized clinically with large head circumference, normal or mildly motor and language delay and increased cerebrospinal fluid (CSF) in the subarachnoid space with normal ventricles or mild ventriculomegaly. In this review, we describe the etiology, epidemiology, clinical presentation, pathogenesis, neuroimaging, differential diagnosis, treatment and outcome of this entity from current literature with emphasis on diagnostic work-up.

Constraint Induced Movement Therapy (CIMT) is an intervention method that can enhance cerebral palsy children’s hand function. Cerebral palsy (CP) is a pervasive and common disorder which affects many aspects of a child life. Hemiplegic cerebral palsy affects one side of a child’s hand and has great effect on child’s independence. The aim of this study is to investigate the CIMT’s studies which have conducted in Iran. And indicate that Does CIMT’s effectiveness related to its duration and child’s age? Does CIMT have any effect of muscle tone and protective extension?
Material & This study is a systematic review study. For doing this study the Electronic databases such as Medlin PubMed, CINAHL… Iranian and foreigner famous journals in the fields of pediatrics such as Iranian Journal of Pediatrics (IJP), Iranian Rehabilitation Journal (IRJ)… and Google scholar and some specific key words such as CP, CIMT, and occupational therapy had been used.
Based on the research and key words the 43 articles were found that, from those 43 articles because of lack of relevancy and other issues the 28 articles had been removed. And from obtained 25 articles, 10 of them omitted based on duplication and study’s exclusion criteria, so finally 15 articles included to this study.
This study showed that CIMT is effective compared to no intervention but there are some inconsistencies regarding some parts of CIMT effectiveness such as CIMT effectiveness on muscle tone and protective extension.

This study was done to answer the question that whether or not previous antiepileptic drugs with combination of omega-3 and risperidone is more efficient than previous antiepileptic drugs with risperidone alone in decreasing of seizures monthly frequency of children with refractory epilepsy and attention-deficit/hyperactivity disorder (ADHD).
In a randomized clinical trial, sixty four 7-11 year old children with refractory epilepsy and ADHD who were referred to Pediatric Neurology Clinic of Shahid Sadoughi Hospital, Yazd, Iran from June 2015, were distributed into two groups, randomly. In group I, one capsule of omega-3 daily and 0.5 mg of risperidone was divided into two doses with previous antiepileptic drugs and in group II, 0.5 mg of risperidone was divided into two doses with previous antiepileptic drugs were given. The drugs use was continued for three months and the children were followed up monthly for three consecutive months. Primary outcomes included seizure monthly frequency and good response (more than 50% of reduction in seizures monthly frequency). Secondary outcome was clinical side effects.
23 girls and 33 boys with mean age of 9.24.15(29 children in omega-3 group and 27 children in control group) were evaluated. Omega-3 therapy was effective in decreasing of seizures monthly frequency (10.41±3.92 times vs. 17.01±4.98, P = 0.03). Good response was seen in three children (11.1%) in control (95% confidence interval: 8%-22.8%) and in 9 children (20%) in Omega-3 (95% CI: 47.83%-14.17%) group, which showed that omega-3 was more effective in seizure controlling. (P=0.001) Frequency of side effects was not different in the two groups (14.8 % in control vs. 20.7% in omega-3 groups, P=0.5).
Omega-3 might be used as an effective and safe drug in seizures control of children with refractory epilepsy and ADHD.

This research was an experimental study that was aimed to detect differences respon of Tactile Sensory Stimulus between normal children and children with sensory brain development disorders such as ASD (Autism Spectrum Disorder).
A total of 134 children, in two groups: 67 healthy children (control) and 67 children with autism were studied. Tactile sensory stimulus responses in children were tested directly using a Reflex Hammer. In addition, tactile sensory sensitivity was also assessed via questionnaire Short Sensory Profile (SSP) which was filled out by the child's parents.
All response data were analyzed using Fisher's Exact Test; questionnaire data was analyzed with the Mann-Whitney U Test.
The results showed that autistic children were more sensitive to palpation and pain than children who were not autistic. Furthermore, the value of SSP was also significantly higher in autistic children, which means that they always responded to all categories in the SSP questionnaire than children who are not autistic.
Autistic children are more sensitive to Tactile Sensory Stimulus and all categories of SSP than children who are not autistic.

Headache and sleep problems are commonly reported in children, and both can adversely impact the child’s life. This study aimed to compare the sleep quality and intensity of headache between school-age children with migraine and tension headache and healthy children.
In this cross-sectional study, sample was 198 children 6-12 years old in three groups. Migraine and tension headache groups from the outpatient clinic of Imam Khomeini of Hamadan and healthy group from elementary schools were randomly selected (66 children in each group) Data were collected using demographic questionnaire, Child Sleep Habits Questionnaire (CSHQ), Numeric Scale of Pain Intensity and Wong- Baker Faces Pain Rating Scale. The data were analyzed using SPSS 16 software by descriptive statistic and multivariate ANOVA, one way ANOVA, chi-square, kruskal-wallis and linear regression tests.
The majority of subjects in migraine group (57.6%), tension headache (60.6%) and healthy children (45.5%) were female. Approximately, 45.5% of children with migraine and 37.9% of them with tension headache had experienced severe headache. Only a significant relationship was seen between mean scores of headache intensity and sleep quality in migraine group (p The children with migraine experienced more unsuitable sleep in duration of severe headache. Highlighting the co-morbidity between intensity of headache and sleep problem of children with migraine and tension headache is important to improve treatment strategies and to know the impact of headache on their normal life.

Given the positive effects of stimulation with maternal breast milk odor and non-nutritive sucking on premature infants’ feeding skills, the present study was conducted to examine the effect of combining two stimuli, including non-nutritive sucking and maternal breast milk odor, on the time of achieving independent oral feeding in premature infants.
The present clinical trial was conducted at Mahdieh and Shohada-e Tajrish Hospitals of Tehran in 2016. A total of 32 neonates with a fetal age of 28-32 weeks for whom gavage feeding was started entered the study and were divided into a group of non-nutritive sucking combined with olfactory stimuli and another group of non-nutritive sucking only. The stimulations were performed in both groups at the first five minutes of gavage, three times per day, over ten consecutive days. Weight gain, the time of achieving frequent oral feeding and chronological age at the time of discharge from the hospital were recorded as measures of the effectiveness of the interventions. The results of the interventions were analyzed and compared in SPSS-18.
Findings and Non-nutritive sucking combined with maternal breast milk odor leads to a lower post-menstrual age at the first oral feeding, independent oral feeding and discharge from the hospital in premature infants, but has no effects on their daily weight gain and weight at the time of discharge. These results show the effectiveness of combining maternal breast milk odor and non-nutritive sucking as two separate stimuli in achieving oral feeding and earlier discharge from the hospital.

Caregivers of children with cerebral palsy have to spend a long time to take care of their child. Also taking care of these children is a source of stress for their caregivers. Dealing with stressful situations has a negative impact on the quality of life of caregivers. The aim of the study was to develop a user-friendly web-based intervention for training parents of children with CP and evaluate the process of development using modified CeHRes road map.
The study was conducted in four main steps including determine the needs of users, content development, design, operational development and evaluation.
The website for caregiver training provided nine general topics and had the possibility that the caregivers could determine their educational priorities. Also, the users can share their experiences with other users and can ask questions from an expert. 10 caregivers completed a usability questionnaire after four weeks use. The average score of 70.5 out of 100 was shown among caregivers. The average score of all statements were above three on a Likert scale between 1 and 5.
The website has the possibilities including registering caregivers of children with CP, the possibility to confirm registration with an SMS and the possibility to determine the caregiver educational priorities. It has the usability for training caregivers of children with CP.

Acute prolonged seizure is the most common neurological emergency in children. This research was conducted to compare the effect of intravenous Phenobarbital and sodium valproate in control of seizure in children who were referred to emergency ward in 2013.
In this randomized clinical trial, 80 children with prolonged seizure and with no response to one dose of diazepam (0.2 mg/kg) during the five minutes were selected. Children were randomly allocated into two groups, intervention and control through permutation blocks. In intervention group, intravenous sodium valproate (20mg/kg) and in control group, intravenous Phenobarbital (20 mg/kg) was prescribed. Data such as age, gender, history of previous seizure, seizure type and recovery time after receiving drug was recorded in the form. Data analysis was done through descriptive statistics, Chi-square and Independent T- Test.
Two groups were the same in terms of age and gender and have no statistically significant difference, but they were different in terms of seizure type (p=0.002). In valproate group, 18 patients (45 percent) and in Phenobarbital group, 32 patients (80 percent) have positive response to the treatment and the chi-square test showed the significant difference (p=0.001). The mean of response time to drug in intervention group was 6.17±2.99 and in control group was 4.71±2.38 minutes, but this difference was not statistically significant.
Results of current study showed that the sodium valproate in comparison with Phenobarbital isn’t a suitable and effective drug for controlling of seizure in children

Evaluation of concurrent validity of GHDAMT with the problem solving subscale of ASQ2 among 5 years old children was the objective of present research.
In a cross sectional study 136 boy and 105 girl were selected randomly from childcares and tested with GHDAMT and ASQ2 to compare the concurrent validity of these tests in evaluation of cognition.
Data was analyzed using SPSS.16/win.
The IQ mean difference in girls was 128±18.18 and in boys 118±18.50, which was statistically significant. There was no statistical significant correlation between GHDAMT and ASQ2. It was significant between IQ and mental age among children who had -2SD score in problem solving subscale, but there was no correlation between children who had -1SD score.
These findings revealed that the GHDAMT does not have acceptable validity and concurrent validity of the test was less than 0.3.
In the current research, there is no statistical significant correlation between problem solving subscale of ASQ2 and mental age and IQ. So GHDAMT can̕ t be used as a substitute of ASQ questionnaire; however, correlation of two tests in children with intellectual disability was meaningful. After further studies, it is possible to use GHDAMT as a proper tool for cognition evaluation of these children.

Seizure is the most common signs of nervous system disease in neonates.The first line of treatments in neonatal seizures (after ruling out and emergency treatment of electrolyte imbalance and hypoglycemia) are, phenobarbital and phenytoin. At least, 35%-45% of seizures may not be controlled by first-line drugs, so further treatment would be necessary. Levetiracetam has shown its safety and efficacy in treatment of neonatal seizures. We aimed to evaluate drugs that are more effective concerning neonatal seizure.
Initially, 245 neonates presented with seizure in neonatal wards &NICUs(level IIa&IIb) in Qom hospitals,were enrolled in this clinical trial study(inclusion criteria). The main causes of seizure in 12 neonates were electrolyte imbalance and hypoglycemia,therefore, they were excluded from the study. Phenobarbital and then if needed, phenytoin administered to the remaining neonates, as first line treatment.Eventually, 42 neonates with a diagnosis of resistant seizure to these drugs were challenged with oral levetiracetam. Data was analysed using SPSS ver. 20.(Chicago,IL,USA). The study was registered as code number of IRCT2016051527896N1.
In this study,85.8% of seizures were controlled with initial dosage of oral levetiracetam and 9.5% by increasing the dose but 4.7% were not cured .
when the intravenous form of levetiracetam is not available and neonatal seizure not respond to first line classic drugs,oral levetiracetam as add on therapy is effective.

Ataxia telangiectasia (AT) is a rare autosomal recessive disorder caused by mutation in the Ataxia telangiectasia mutated (ATM) gene. This disorder is characterized by progressive cerebellar ataxia, telangiectasia, immunodeficiency and a predisposition to leukemia/lymphoma.
In this study, four members of a family including a symptomatic AT patient, his parents and sibling were examined for ATM gene defects. DNA was extracted from peripheral leukocytes and the coding regions and exon-intron boundaries of ATM gene were amplified by next generation sequencing technique. The identified mutation was tested in all members of the family.
Molecular analyses identified a homozygous T to G substitution in c.7308-6 position resulting in a novel acceptor splice site in intron 49 of the ATM gene in the index patient. Parents and sibling of the proband were heterozygous for the same mutation.
The variant c.7308-6T>G is predicted to be pathogenic due to impaired splice site causing exon skipping. This newly found frameshift mutation cosegregated as an autosomal recessive trait as expected for Ataxia telangiectasia syndrome.

Febrile seizure is the most common type of seizure among children. Identification of factors involved in febrile seizure is highly critical.
The present study was conducted to determine the association between children’s urinary tract infection and febrile seizure.
In this case-control study, 165 children with simple febrile seizure (case group) were compared with 165 children with fever and without seizure (control group) in terms of urinary tract infection. The age of children was between 6 months to 5 years. The results of both groups were analyzed and compared.
Among 165 children with febrile seizure, 25 subjects (15.2%) had urinary tract infection. In the control group, only 2 patients (1.2%) had UTI. There was significant difference between two groups regarding urinary tract infection (P=0.001). Among 25 children with UTI in the case group, 17 children (68%) had acute pyelonephritis, and the remaining 8 children (32%) had cystitis. The two patients with UTI in control group had cystitis (P=0.055).
The present study showed that urinary tract infection could be a risk factor of febrile seizure. Therefore, it is recommended that all patients with febrile seizure be examined in terms of urinary tract infection.

Human beings can use gestures such as pointing and reaching to communicate with others before they have the ability to use verbal communication to produce speech. Given the importance of children's communication development and the key role of gestures development in communicating, the main purpose of this study is to analyze the normal development of pointing and reaching gestures and their relationship in 12-16-month-old children speaking Farsi.
Methods and materials: In this prospective, observational and longitudinal study the gestures of 11 monolingual Farsi- speaking children (7 boys and 4 girls) were evaluated via non-randomized sampling method. Child-mother interactions were videotaped monthly in a semi-structured context to capture the emergence and consistent use of targeted gestures. Afterwards, the data was coded and statistically analyzed for this purpose Repetitive measured, independent T-Test and Pearson correlation were used.
The mean of the pointing gesture increased significantly from 12 to 16 months (p0.05). Moreover, there was no relationship between pointing and reaching gestures (p>0.05).
The findings of this study suggest that pointing gestures increase with age from 12-16 months in Farsi-speaking children. However, reaching gestures stay the same between 12-16 months of age. The Results are compatible with the other surveys in this area. The study provided rich details of common gestures that children use to signal their intentions before verbal communication.

Emotion is a key component in language processing, but it is controversy how emotional content words process in children. The aim of the present study is investigating the effect of arousal and valence on word familiarity, comprehension, use and emotional content recognition. As secondary goal, a list of emotional content words for this age was prepared in Persian.
Emotional words, from adult emotional words list, were categorized in 5groups according their scores in arousal and valence. Evaluation of familiarity, use, comprehension and emotional content recognition of these words was conducted with a prepared checklist in 60 seven-year-old children by speech and language pathologist.
Neutral words gained the highest score in familiarity, use, comprehension and emotional content recognition (the mean=0.74). Afterward, there were the emotional words with high valence, calm (the mean=0.64) and happy (the mean=0.52). Finally it was found the low score for valence emotional words, sad (the mean=0.46) anxious (the mean =0.43) in end of score rating. Interestingly, there was a significant difference between all word groups in four aspects (p-value0.001).
It was shown that neutral words are better comprehended and expressed than emotional words. More importantly, valence is more effective than arousal in emotional words. It is noteworthy gender was not a determinant factor in all of the aspects. An emotional word list which is comprehensible for children in Persian language was prepared.

Hearing loss is one of the most important disabilities in neonates. Delay in detection of hearing loss leads to impaired development and may prevent the acquisition of speech. The present study was designed to determine the risk factors associated with hearing loss in neonatal patients aged more than 6 months with history of hospitalization in Neonatal Intensive Care Unit (NICU).
In this case-control study, screening for hearing loss was carried out on 325 neonates aged 6-12 months who referred to pediatric neurology office of Vali-e-Asr hospital, Tehran, iran. Hearing loss has confirmed using Acoustic Brain Audiometry (ABR) screening test.
The prevalence of hearing loss in neonates was determined about 3.6%. The most significant risk factors for hearing loss in neonates were neonatal icterus associated with phototherapy, respiratory distress syndrome (RDS) and lower Apgar score.
Considering significant prevalence of hearing loss among studied neonates, screening all newborns is still seems valuable and cost effective.

A 7 years old patient was referred to our pediatric orthopedic clinic with the diagnosis of cerebral palsy. His parents were concerned about some inconsistencies in his disease progression. After initial evaluations, we were convinced that the diagnosis of cerebral palsy was incorrect. The true diagnosis was suspected and confirmed with molecular genetic analysis. A rare autosomal recessive disorder –cockayne syndrome- has been diagnosed. Although untreatable, it can be prevented by appropriate prenatal diagnostic tests for their future children.

Gram-negative meningitis has been reported owing to dura-arachnoid barrier disruption. It can be occurred because of trauma, surgery and rarely infected dermoid cyst.
In the 1940, for the first time, the association of neurosurgical procedures and meningitis due to gram-negative bacillary was described.
Enterobacter is a serious infection with difficult treatment secondary to inability of many antibiotics to reach to adequate concentration in cerebrospinal fluid.
Here in, we report a case of pediatric Enterobactermeningitis in a patient with manipulated dermoid cyst.She was cured with antibiotic therapy and surgery.