Iranian Journal of Child Neurology
Year:2018 | Volume:12 | Issue:4|Papers Count:17

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.

Objectives Constraint-Induced Movement Therapy (CIMT) is an intervention method that can enhance cerebral palsy (CP) children’ s hand function. CP is a pervasive and common disorder which affects many aspects of a child life. Hemiplegic CP affects one side of a child’ s hand and has great effect on child’ s independence. We investigated the CIMT’ s studies conducted in Iran, and indicated the effectiveness of CIMT on duration and children age? Materials & Methods This systematic review was conducted using the electronic databases such as Medline PubMed, CINAHL, etc. performed from 1990 to 2016. Iranian and foreigner famous journals in the fields of pediatrics such as Iranian Journal of Pediatrics (IJP), Iranian Rehabilitation Journal (IRJ) and Google scholar with some specific keywords such as CP, CIMT, and occupational therapy were searched. Results Overall, 43 articles were found, from which, 28 articles were removed because of lack of relevancy. Ten article were omitted because of duplication and exclusion criteria, so finally 15 articles were included. Conclusion CIMT is effective compared to no intervention but there are some inconsistencies regarding some parts of CIMT effectiveness such as its effectiveness on muscle tone and protective extension.

Objectives We aimed to answer the question whether or not previous antiepileptic drugs with combination of omega-3 and risperidone are 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). Material & Methods In a randomized clinical trial (IRCT201604212639N18), participants referred to Pediatric Neurology Clinic of Shahid Sadoughi Hospital, Yazd, Iran from Jun 2015 were distributed randomly into two groups. 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. Results Overall, 23 girls and 33 boys with mean age of 9. 24± 0. 15 yr (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 (31%) in omega-3 (95% CI: 47. 83%-14. 17%) group, which showed that omega-3 was more effective in seizure control. (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). Conclusion Omega-3 might be used as an effective and safe drug in seizures control of children with refractory epilepsy and ADHD.

Objective This research was an experimental study that was aimed to detect differences response of tactile sensory stimulus between normal children and children with sensory brain development disorders such as Autism Spectrum Disorder (ASD). Materials & Methods A total of 134 children, in two groups including 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) 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. Results Autistic children were more sensitive to palpation and pain than children who were not autistic. Furthermore, the value of SSP was also significantly higher (P<0. 05) in autistic children, which means that they always responded to all categories in the SSP questionnaire than children who are not autistic. Conclusion Autistic children are more sensitive to tactile sensory stimulus and all categories of SSP than children who are not autistic.

Objectives Headache and sleep problems are commonly reported in children, and both can adversely impact the child’ s life. We aimed to compare the sleep quality and intensity of headache between school-age children with migraine as well as tension headache and healthy children. Materials & Methods In this cross-sectional study, 198 children 6-12 yr old in three groups were enrolled from Aug 2015 to Mar 2016. Migraine and tension headache groups from the Outpatient Clinic of Imam Khomeini of Hamadan, western Iran 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 by descriptive statistic and multivariate ANOVA, one way ANOVA, Chi-square, Kruskal-Wallis and linear regression tests. Results 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<0. 05). There was a significant difference in mean scores of sleep quality among three groups (P<0. 001). Conclusion 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.

Objectives Given the positive effects of stimulation with breast milk odor and nonnutritive sucking (NNS) on preterm feeding skills, we examined the effect of NNS and milk odor, on the time of achieving independent oral feeding in preterm infants. Materials & Methods This study was conducted at two Neonatal Intensive Care Units of Tehran, Iran in 2016. Overall, 32 neonates with gestational ages of 28-32 wk were enrolled in two groups; NNS with and without olfactory stimuli (breast milk odor). The stimulations were performed in both groups during the first five minutes of gavage, three times per day, and over ten consecutive days. Weight gain, time of achieving oral feeding and chronological age at discharge were as measures of the effectiveness of the interventions. The results of the interventions were analyzed and compared using SPSS. 18. Results NNS with breast milk odor resulted to a lower post-menstrual age at the first oral feeding, independent oral feeding and discharge from the hospital, but had no effects on their daily weight gain and weight at the time of discharge. Conclusion These results show the effectiveness of combining milk odor and NNS as two important stimuli in achieving oral feeding and earlier discharge from the hospital.

Objectives Caregivers of children with cerebral palsy (CP) have to spend a long time to take care of their children. We aimed to develop a user-friendly webbased intervention for training parents of children with CP and evaluate the process of development using modified CeHRes roadmap. Materials & Methods The study was conducted from September 2016 to September 2017 in Tehran, Iran. We did it in four main steps including determining the needs of users, content development, design, operational development and evaluation. Results The website for caregiver training provided nine general topics and had the possibility that the caregivers could determine their educational priorities. Moreover, the users could share their experiences with other users and could ask questions from an expert. Ten caregivers completed a usability questionnaire after four weeks of use. The average score of 70. 5 out of 100 was shown among caregivers. The average score of all statements was above three on a Likert scale between 1 and 5. Conclusion 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.

Objectives 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 with status epilepticus, referred to emergency ward from Mar to Nov 2013. Materials & Methods In this randomized clinical trial, registered with the code number IRCT2015051722300N1, 80 children aged 6 months to 10 years with prolonged seizure and with no response to one dose of diazepam (0. 2 mg/ kg) administered through IV injection during the five min were selected. Children were randomly divided into two groups, intervention, and control through permutation blocks. In intervention group, intravenous sodium valproate (20 mg/kg) and in control group, intravenous phenobarbital (20 mg/kg) were 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, Chisquare and Independent t-test. Results Two groups were the same in terms of age and gender and had no statistically significant difference, but they were different in terms of seizure type. In valproate group, 18 patients (45%) and in phenobarbital group, 32 patients (80%) had positive response to the treatment and the chi-square test showed the significant difference. Conclusion With regards to this point that in phenobarbital group, patients had more rapid response to drug in comparison with patients in sodium valproate group, phenobarbital is a suitable and effective drug for controlling of seizure in children.

Objective The main aim of the current research was evaluation of concurrent validity of the Goodenough– Harris Draw-A-Man Test (GHDAMT) with the problem-solving subscale of ASQ2 among children between 54-60 months old in Tabriz City, northwestern Iran. Materials & Methods In this cross-sectional study, 136 males and 105 females were selected by simple random sampling from nursery schools in Tabriz City, northwestern Iran in 2014 and tested with GHDAMT and ASQ2 to compare the concurrent validity of these tests in evaluation of cognition. Data were analyzed using Pearson or Spearman correlation coefficients and SPSS. 16. Results The mean Intelligence Quotient (IQ) in girls was 128± 18. 18 and in boys 118± 18. 50, and the difference was statistically significant P<0. 001. There was no statistically significant correlation between GHDAMT and ASQ2. The statistical correlation was significant between IQ and mental age among children who had-2SD score in problem-solving subscale, but there was no statistical correlation between children who had-1SD score P<0. 002. There was no statistically significant correlation between problem-solving subscale of ASQ2 and mental age and IQ. Conclusion GHDAMT did not have acceptable validity and concurrent validity of the test was less than 0. 3. So GHDAMT cannot be used as a substitute of ASQ questionnaire. However, the correlation of two tests in children with intellectual and developmental disability was significant. After doing morestudies in further research, it is possible to use GHDAMT as a proper tool for cognition evaluation of these children.

Objective 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. We aimed to evaluate drugs that are more effective on neonatal seizure. Materials & Methods Patients admitted to neonatal wards & NICUs (level IIa&IIb) in Qom hospitals (2015-2017), central Iran with presentation of seizure, were enrolled in this clinical trial study. After ruling out electrolyte imbalance and hypoglycemia these neonates were managed by intravenous phenobarbital, then if no response was seen we added intravenous phenytoin and for remaining neonates with refractory seizure we applied oral levetiracetam as add on therapy. The study was registered as code number of IRCT2016051527896N1. Results Initially, 245 neonates were enrolled. According to exclusion criteria, 12 cases were excluded, and phenobarbital was prescribed to the remaining patients. Out of these, 86 patients did not respond, and phenytoin was prescribed for them. Forty two patients who were not responding to phenytoin were finally treated with oral levetiracetam. Finally, 95. 3% of seizures were controlled with oral levetiracetam but 4. 7% were not cured. Conclusion: When the intravenous form of levetiracetam is not available and neonatal seizure does not respond to first line classic drugs, oral levetiracetam as add on therapy maybe effective.

Objectives 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, we investigated a family with a new mutation in ATM, confirmed by molecular genetic test. Materials&Methods Four members of a family including a symptomatic AT patient, his parents and sibling were examined for ATM gene defects at Kerman University Hospital, Kerman, Iran in 2016. 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. Results 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. Conclusion 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.

Objectives 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. Materials & Methods 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 (UTI) in Qazvin, central Iran in 2015-2016. The age of children was between 6 months to 5 yr. Results Among 165 children with febrile seizure, 25 (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). Conclusion Urinary tract infection could be a risk factor for febrile seizure. Therefore, all patients with febrile seizure are examined in terms of urinary tract infection.

Objectives 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 was to analyze the normal development of pointing and reaching gestures and their relationship in 12-16-month-old children speaking Farsi. Materials & Methods In this prospective, observational and longitudinal study the gestures of 11 monolingual Farsi-speaking children (7 boys and 4 girls, from Oct 2015 to Jan 2017 in the homes of participants across Tehran, Iran) 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. Afterward, the data were coded and statistically analyzed for this purpose Repetitive measured; independent t-test and Pearson correlation were used. Results The mean of the pointing gesture increased significantly from 12 to 16 months (P<0. 05). However, this was not significant for the reaching gesture. Moreover, there was no relationship between pointing and reaching gestures. Conclusion 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 study provided rich details of common gestures that children use to signal their intentions before verbal communication.

Objectives Emotion is a key component in language processing, but emotional words processing in children is still controversial. We aimed to investigate the relationship between emotional dimensions, arousal and valence, word familiarity, comprehension, use, and emotional content recognition. Eventually, a list of emotional content words for this age was prepared in Persian. Materials&Methods The study was conducted in selected elementary schools in Tehran, Iran from April to June 2017. Emotional words, from adult emotional words list, were categorized into 5 groups according to their arousal and valence scores, including neutral, happy, calm, anxious and sad. Evaluation of familiarity, use, comprehension and emotional content recognition of the list was conducted with a checklist in 60 first grade children by speech and language pathologist. Results 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. There was a significant difference between all word groups in four aspects (P-value<0. 001). There were no significant differences between boys and girls in four aspects. Conclusion Neutral words are better comprehended and expressed than emotional words. Valence is more effective than arousal in emotional words. 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.

Objectives Hearing loss is one of the most important disabilities in neonates. Delay in the detection of hearing loss leads to impaired development and may prevent the acquisition of speech. We aimed to determine the risk factors associated with hearing loss in neonatal patients aged more than 6 months with a history of hospitalization in Neonatal Intensive Care Unit (NICU). Methods In this case-control study, screening for hearing loss was carried out on 325 neonates aged 6-12 months referred to Pediatric Neurology Office of Vali-e-Asr Hospital, Tehran, Iran up to 2011. Hearing loss was confirmed using Auditory Brainstem Response screening test (ABR). Results The prevalence of mildly and moderately hearing loss in neonates was determined as 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. Conclusion It seems to quantitative auditory system screening using ABR is necessary for all neonates; because rehabilitation support such as speech therapy and hearing training in this age period is more effective than older ages.

A 7-yr-old patient was referred to pediatric orthopedic clinic of Imam hospital (2016) with the diagnosis of cerebral palsy (CP). His parents were concerned about some inconsistency of his disease progression. After initial evaluations, the diagnosis of CP was incorrect. The true diagnosis was suspected and confirmed with molecular genetic analysis. A rare autosomal recessive disorder-Cockayne syndrome-was diagnosed. Although untreatable, it can be prevented by appropriate prenatal diagnostic tests for their future children.

Gram-negative meningitis can occurssubsequent to dura-arachnoid barrier disruption because of trauma, surgery and rarely an infected dermoid cyst. Association of neurosurgical procedures with Gramnegative meningitis was described for the first time in 1940. Intracranial infections from gram-negative bacilli like Enterobacter are serious and difficult to treat as many antibiotics fail to achieve bactericidal concentrations in the cerebrospinal fluid. Here in, we report a rare case of pediatric Enterobacter meningitis in a patient with a dermoid cyst that had been manipulated. She was managed with antibiotic therapy plus surgical removal of the infected cyst.