Review Article

Bilirubin Induced Encephalopathy, a Review Article

Parvaneh Karimzadeh, Minoo Fallahi, Mohammad Kazemian, Naeeme Taslimi Taleghani, Shamsollah Nouripour, Mitra Radfar

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 7-19

Hyperbilirubinemia is one of the most common neonatal disorders. Delayed diagnosis and treatment of the pathologic and progressive indirect hyperbilirubinemia lead to neurological deficits, defined as bilirubin induced encephalopathy . The incidence of this disorder in underdeveloped countries is much more than developed areas. All neonates with  the risk factors for increase the blood level of indirect bilirubin are at risk for bilirubin encephalopathy ,especially preterm neonates which are prone to low bilirubin kernicterus .Bilirubin toxicity can be transient and acute(with early ,intermediate and advanced phases)or be permanent, chronic(kernicterus)and lifelong ( with tetrad of symptoms including visual (upward gaze palsy), auditory (sensory neural hearing loss), dental dysplasia abnormalities, and extrapyramidal disturbances (choreoathetosis cerebral palsy).Beside the abnormal neurologic manifestations of the jaundiced neonates ,brain MRI is the best imaging modality for the confirmation of the diagnosis. Although early treatment of extreme hyperbilirubinemia by phototherapy and exchange transfusion can prevent the BIE, unfortunately the chronic bilirubin encephalopathy does not have definitive treatment

Research Article

Well-Being and Coping Capacities of Adolescent Students with Hearing Loss in Mainstream Schools

Narges Adibsereshki, Nikta Hatamizadeh, Firoozeh Sajedi, Anoshirvan Kazemnejad

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 21-30



Coping strategies used by adolescents has an important role in Preventing or decreasing their stresses and also increasing their wellbeings.This study aimed at evaluating the coping capacity and wellbeing of adolescent students with hearing loss in mainstream schools and also the correlations between their coping strategies and positivecharacteristics of well-being (engagement, perseverance, optimism,connectedness and happiness (EPOCH).

Materials & Methods

In this correlational study, 122 adolescent students with hearing loss were randomly selected from mainstream schools. Data collection was done by EPOCH Measure of Adolescent Well-Being and the Ways of Coping Questionnaire (WAYS). The Spearman correlation coefficient was used for determining the correlations between variables.


The mean scores of using different coping strategies varied from 1.36i n problem solving to 1.44 in seeking support. Among the positive characteristics of well-being, happiness had the lowest (11.04) and connectedness showed the highest score (12.33). The findings also showed a significant correlation between all coping strategies and EPOCH, however there was a strong positive correlation between total coping strategy score and perseverance (0.648) and happiness(0.629).


Based on the results, the score of happiness in students with hearingl oss was the lowest among positive characteristics of well-being and also happiness showed a strong association with total scores in coping strategies. Accordingly, interventional studies are needed to examine whether training students with hearing loss to use coping strategies is effective in increasing their happiness and overall well-being.

The Impact of Antiepileptic Drugs on Thyroid Function in Children with Epilepsy: New Versus Old

Hatem Elshorbagy, Naglaa Barseem, Hany Suliman, Eman Talaat, Ashraf Al-shokary, Waleed Abdelghani, Sameh Abdulsamea, Yehia Hamed Abdel Maksoud, Sanaa Mohammed Azab, Akram Elshafey Elsadek, Dalia Mohamed Nour El Din

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 31-41





To investigate the effects of traditional antiepileptic drugs (AEDs) versus newer AEDs on the thyroid hormone profile of children with epilepsy.

Materials & Methods

A total of 80 children with epilepsy were included in this study and were divided into two groups. Group 1 included 40 children with epilepsy on traditional AEDs, and group 2 included 40 children with epilepsy on newer AEDs. Forty healthy children were also included as the control group (group 3). We analyzed the serum levels of free triiodothyronine (FT3), free thyroxine (FT4), and thyroid stimulating hormone (TSH).

Results: Epileptic children treated with traditional drugs showed a statistically significant decrease in the serum fT4 and increase in TSH concentrations compared to the control group (P < 0.001). Whereas epileptic children treated with newer drugs showed no statistically significant changes in the serum fT3, fT4 and TSH concentrations compared to the control group Conclusions: Traditional AEDs have a significant impact in the thyroid hormone profile compared to the newer AEDs.

Serum Zinc level in Children with Febrile Seizure: A Case – Control Study

Ali Nikkhah, Firoozeh Hosseini, Mojdeh Afkhami Goli

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 43-47



Febrile seizure (FS) is the most common seizure disorder in children. FS is a genetic age-limited seizure disorder that occurs only with febrile illness. Nowadays, we know that genetic factors play a major role in occurrence of FS, but some trace elements (for example: zinc) may have important role in occurrence of FS. In this study, we investigate serum level of zinc in patients with FS and in febrile children without seizure as control group.  

Materials & Methods

In this case-control prospective study 41 patients with first febrile seizure (FS) (simple & complex) as case group and 41 febrile children without seizure as age, sex matched control group admitted in Besat hospital in Hamadan between January 2013 to January 2014 were enrolled. Ages of children were 6 months to 5 years. Serum zinc levels were measured by atomic absorption spectrophotometery method in these groups.


The mean serum zinc level was 70.41±20.46 mcg/dl and 92.73±17.62 mcg/dl in the case group and the control group, respectively (P = 0.001). In fact, serum zinc level in children with FS was significantly lower than that of control group.


Although based on results of this study, we found lower serum zinc level in children with febrile seizure, but we can't recommend zinc administration for prevention of FS and we need further fundamental basic studies in futures.   

Relationship Between Parental Communication Patterns and Self-Efficacy in Adolescents with Parental Substance Abuse

Zeinab Hemati, Samira Abbasi, Parastoo Oujian, Davood Kiani

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 49-56


Since an individual’s personality and behavior are mainly shaped in the family environment, parental substance abuse and its consequences can lead to confusion and instability in the family environment and reduce child-parent relationship. This study was conducted to investigate the relationship between different aspects of parental communication patterns and self-efficacy in adolescents with parental substance abuse.

Materials & Methods

 In this descriptive correlational study, a total of 100 adolescents with parental substance abuse were selected via random sampling. The data collection tools included the parental communication patterns scale and self-efficacy questionnaire. Data were analyzed in SPSSversion 20, using linear regression analysis and Pearson’s correlation coefficient test.


 The mean age of the adolescents was 14.5±2.5 years. Pearson’s correlation coefficient showed that the adolescents’ self-efficacy score was directly correlated with the score of conversation orientation dimension (a parental communication pattern) and inversely correlated with the dimension of conformity orientation (P<0.001). 

Conclusion: Development of educational programs to promote communication skills, positive family interactions, decision-making, and flexibility can change the unhealthy family environment into a healthy and productive one, which promotes self-efficacy beliefs in children.

Effect of Midazolam Alone Versus Midazolam with Maternal presence on pain and Anxiety of Lumbar Puncture in 6 to 24-Month-Old Children

Farzad Ferdosian, Rihaneh Esteghamat, Razieh Fallah, Tamkin Shahraki

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 57-64



 Midazolam at a dosage of 0.51 mg/kg induces anxiolytic effects in 90% of children. This study was performed to elucidate whether intravenous midazolam with maternal presence is more efficient than intravenous midazolam alone in the reduction of pain and anxiety of lumbar puncture (LP) in 6 to 24-month-old children.

Materials & Methods

In this not-blinded clinical trial, we included 60 children aged 6 to 24 months old undergoing LP in the Pediatric Ward of Shahid Sadoughi Hospital, Yazd, Iran, from September 2014 to March 2015. The participants were randomly assigned to two groups, and all of them received painless injection of 0.5 mg/kg midazolam five minutes before LP. In group I, LP was performed with maternal presence and in group II, the mothers were absent. The primary outcomes included anxiety and pain scores before LP and during needle insertion to the skin for LP. The secondary outcomes comprised of success rates in the reduction of anxiety (anxiety score of four and more) and pain (pain score of less than three) when the needle was inserted to the skin for LP.


 Twenty-eight girls and 32 boys were evaluated in the two groups. Maternal presence was found to be effective in the reduction of anxiety (2.7±0.65 vs. 3.83±0.87; P=0.001) and pain scores (3.8±1.75 vs. 6.1±1.63, P=0.001). In the maternal presence group, success rate in anxiety (76.7% vs. 16.6%; P=0.0001) and pain reduction (63.3 % vs. 6.7%; P=0.0001) was higher than in the midazolam alone group.                                                                                                                  Conclusion

Maternal presence during lumbar puncture  can reduce pain and anxiety among 6 to 24-month-old children.  

The Effect of “Creating Opportunities for Parent Empowerment” Program on Parents of Children With Epilepsy and Other Chronic Neurological Conditions

marys jahri sheijani, Minoo Mitra Chehrzad, Shadman Reza Masouleh, Ehsan Kazemnezhad Leyli, Elham Bidabadi

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 65-76


Parents taking care of children with epilepsy experience stress in their daily lives, which enhances their anxiety, changes their function, and eventually increases their children's behavioral problems. The present study aimed at investigating the effect of creating opportunities for parent empowerment (COPE) program on parents of children with epilepsy or other chronic neurological conditions.


Material & Methods: A quasi-experimental clinical trial study was conducted on 88 mothers of hospitalized children with epilepsy aged 3 to 12 using gradual sampling and in two intervention and usual care groups. In the intervention group, the COPE program was conducted by the researcher in three phases and the usual care group receiving 'usual healthcare'. The mothers’ anxiety was assessed in three phase, too.


Results of the present study showed that the effect of time and group-time interaction on the state anxiety and trait anxiety was significant in the intervention group; however, the effect of time was not significant in the usual care group (P = 0.12). The differences of state anxiety and trait anxiety were not significant between the two groups (P = 0.136), which depended on the initial level of anxiety before the intervention. With analyzing covariance after controlling the two variables (namely state anxiety and trait anxiety) before the intervention, the score variations in the state anxiety and trait anxiety were significant at all measurement phases.

Conclusions: The results of this study indicated the positive effect of the COPE program on the anxiety level of parents having children with epilepsy. Given that this technique is non-pharmacological, convenient, easy, and cost effective, it can be used to reduce the parents’ anxiety.


Increasing Mothers’ Confidence and Abilities with Creating Opportunities for Parent Empowerment (COPE): A Randomized, Controlled Trial

Reihaneh Askary kachoosangy, narges shafaroodi, mohammad Heidarzadeh, mostafa Qorbani, arash bordbar, mahnaz Hejazi shirmard, fatemeh daneshjoo

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 77-83


Premature neonates are at great risk for cerebral palsy, developmental delays, hearing problems and visual impairments. Interventions to reduce the morbidities and adverse health outcomes in these neonates and improve parent-infant interaction are highly important. This study was conducted to determine the effect of the Creating Opportunitiesf or Parent Empowerment (COPE) program on the perceived mat ernal parenting self-efficacy of premature parents

Materials and Methods: This was a randomized controlled trial with equal randomization (1:1:1 for 3 groups) and parallel group design. Forty-five preterm neonates were randomly allocated into treatment (n=15), supervision (n=15), and control (n=15) groups. COPE program was provided for a 4-phase educational-behavioral intervention program to the treatment and supervision groups. Primary outcome was parental self-efficacy, which was assessed with the Perceived Maternal Parenting Self-Efficacy. All measurements were taken before and after completion of the study with the valid equipment and by the blind assessors.

Results: COPE mothers reported significantly stronger beliefs regarding their parental role and have more confidence to their ability in caring of neonates compared with control mothers (P-value <0.001).

Conclusion: An educational-behavioral intervention program would strengthen mothers’ beliefs and knowledge about their neonates and would remove barriers to enhance their ability for caring of neonates and enhance parent-infant interaction.


Clonidine Versus Chloral Hydrate for Recording Sleep EEG in Children

Mahmoud Reza Ashrafi, Hossein Mohebbi, Mahmoud Mohamadi, Elham Azizi, Gholam Reza Zamani, Alireza Tavasoli, Reza Shervin Badv, Firozeh Hosseini

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 85-92


One of the difficulties for conduct electroencephalography (EEG) in pediatric patient population is that they are not always cooperative during the procedure. Different medications have been used to induce sedation during EEG recording. In order to find a medication with least adverse effects and high efficacy, we aimed to compare clonidine and chloral hydrate as a premedication prior EEG performing in pediatric population.

Materials & Methods

A prospective, randomized, single-blinded, controlled trial was carried out over 198 children (9 to 156 months) to investigate the sedative and adverse effects of clonidine and chloral hydrate. Patients, partially sleep-deprived the night before, were randomly divided in two groups of clonidine (100 patients) and chloral hydrate (98 patients), on an alternative day basis.



The average sleep onset latency was significantly longer in the clonidine group than chloral hydrate group (Mann-Whitney test, p < 0.0001). Sleep duration ranged between 15-150 minutes and it was not significantly different between two groups (Mann-Whitney test p = 0.2). Drowsiness with chloral hydrate terminated faster than with clonidine. Drowsiness after arousal was seen in 58% and 26.1% of patients in the clonidine and chloral hydrate groups respectively that was  significant  (Mann-Whitney test, p = 0.058). EEG results were reported normal in 77 subjects in the chloral hydrate group (77%) and in 69 subjects (69%) in the clonidine group (p = 0.161). Generalized epileptiform discharges  reported significantly  in the clonidine group  (Mann-Whitney test , p = 0.006).


The results of this study showed that both chloral hydrate 5% (one ml/kg)and clonidine (4 μg/kg)could be administered as a pre medication agent for EEG recording in children , although drowsiness after arousal of clonidine is greater than chloral hydrate . However, the yield of generalized epileptiform discharges in the clonidine group was more than the chloral hydrate group.


Attention Deficit Disorder with Hyperactivity symptoms in early-treated phenylketonuria patients

Mayara Thays Beckhauser, Marcella Beghini Mendes Vieira, Betine Moehlecke Iser, Gisele Rozone de Luca, Marcelo Rodrigues Masruha, Jaime Lin, Emílio Luiz Streck

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 93-103



To assess the presence of symptoms consistent with Attention Deficit Disorder with Hyperactivity (ADHD) in all patients with early-treated phenylketonuria (PKU) in the State ofSanta Catarina in southernBrazil.

Materials & Methods

All of the patients diagnosed with PKU by newborn-screening tests, with ages varying from 6 to 18 years and who started treatment before 60 days of life and presented phenylalanine levels consistently below 6 mg/dL throughout treatment, were included. The subjects were invited to complete a questionnaire that collected sociodemographic, gestational and clinical data. ADHD symptoms were assessed using the revision of the Swanson, Nolan and Pelham Questionnaire.


 A total of 34 patients were evaluated, who were 53% male and 94% white and had an average age of 12 years, and 15% were born premature. According to the Swanson, Nolan and Pelham Questionnaire, 13 patients (38%) met the diagnostic criteria for ADHD, with 2 patients having the inattentive type, 6 patients having the hyperactive or impulsive type and 1 patient having the oppositional defiant disorder type.


Although the patients with PKU were regularly treated from birth, there was a high prevalence of symptoms consistent with ADHD. A pathophysiological interface that involves the dopamine metabolic pathway may exist between the two conditions.

Effect of baby walker use on developmental status based on Ages and Stages questionnaire score

Omid Yaghini, Mehrdad Goodarzi, Samin Khoei, Mehrnoosh Shirani

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 105-111



Baby walker is a popular device which parents use for entertainment, keeping infants safe and walking promotion. Some studies showed developmental delay in baby walker users though others reported no significant effect on development in this group. We designed this study to determine whether baby walker usage has any effect on development by using Ages and Stages Questionnaire (ASQ).


 Materials & Methods

We evaluated 107 one-year-old infants in each baby walker user group and non-users (total number of 214 participants) by using ASQ test of 12-month. We re-evaluated 168 infants at age 18-month by using ASQ test of 18-month. The data of these groups was compared.



Girls use baby walker more frequently (p=0.02). Baby walker usage was not significantly associated with parent’s educational state, mother employment, birth rank of infant and delivery method. The starting age of baby walker use was 6.61 ± 1.46 months. ASQ results in area of gross movement and fine movement were not significantly different in users and non- users at age 12 and 18 month.



Most parents believe baby walker can promote earlier walking, but based on current evidences this belief might not be true. Although most studies showed no developmental delay in baby walker users, parents should become aware about their possible negative effects and hazards.

Emotional and Behavioral Disorders in Pediatric Cancer Patients

Ghazal Zahed, Fatemeh Koohi

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 113-121



 Childhood malignancies raise a range of medical, psychological and social concerns. Identifying psychiatric disorders along with providing mental health services to prevent the emergence and aggravation of mental health problems in children seems necessary in pediatric hospitals. We aimed to find out the frequency of probable emotional and behavioral disorders among children and adolescents with malignancy.

Materials & Methods

This was a cross sectional study conducted at Hematology-Oncology Ward of Mofid Hospital, Tehran, Iran in 2017-2018. Emotional and behavioral disorders was assessed in 399 pediatric patients with malignancy, aged 5 to 12 years, using the Parent Checklist of CSI-4. All data analyses performed using the Stata 14 at 95% sig-value.

Results: In all, 89.2% of sample met diagnostic criteria for at least one disorder. The most prevalent psychiatric disorders were special phobia (57%) enuresis (41.9%) Obsessive-Compulsive disorder (45.6%) and Separation Anxiety disorder (30.3%). Our results did not show significant gender and disease type differences in prevalence of psychiatric disorders.

Conclusion: Prevalence of emotional and behavioral disorders in pediatric patients with malignancy admitted in the children’s hospital is common. These disorders affect on the treatment and quality of life of pediatric patients. Therefore, these findings may guide parents, nurses and clinicians to become more careful about identification and management of them.

Neurometabolic Disorder Articles

Utility of Seizure Pattern and Related Clinical Features in the Diagnosis of Neurometabolic Disorders

Narjes Jafari, Asieh Mosallanejad, Asieh Ghobadifar, parvaneh Karimzadeh, robabeh Ghodssi-Ghassemabadi, Mohammadmehdi Nasehi, Marjan Shakiba, Shahrzad Tabatabaee

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 123-132


Objectives: The current study aimed at identifying the role of seizure types and related features in differentiation between neurometabolic disorders and other causes of seizure.

Materials & Methods: The current cross-sectional study was conducted at two referral children hospitals in Tehran, Iran from 2011 to 2018. The study population included 120 patients presenting with seizure due to neurometabolic disorders and 120 cases due to other causes. The types of seizure and related clinical findings were assessed in both groups.

Result: There was a significant difference in the frequency of seizure types in two groups. Tonic and myoclonic seizures as well as infantile spasm were observed more commonly in the patients with neurometabolic disorders, while atonic, partial and generalized tonic-clonic seizures , and lack of seizure were more common in the control group. In addition, frequency of refractory seizure, age at onset of seizure, and pattern of involvement in brain imaging were helpful for seizure differentiation.

Conclusion: The pattern of seizure and related findings vary in patients with metabolic disorders and are helpful for seizure differentiation. Thus, these factors can contribute to early diagnosis and treatment.


Case Report

A Rare Case Report of Acute Necrotizing Encephalopathy of Childhood

Seyed Ahmad Hosseini, Amir Hossein Goudarzian, Fatemeh Sadat Tabatabaei, Mohammad Hadi Molseghi, Hamed Jafarpour, Mahya Ghahramanlou, Arash Rezai Shahmirzadi

Iranian Journal of Child Neurology, Vol. 14 No. 1 (2020), 1 January 2020, Page 133-137

Background: Acute Necrotizing Encephalopathy in childhood (ANEC) is a kind of fast growing illness accompanied with progressive Encephalopathy. The aim of this article is to report a rare case of ANEC in a 4-year-old boy with bilateral thalamic necrosis and a nonfatal outcome. Case Report: The patient was a 4-year-old Iranian boy who had no prior history of health problems and hospitalization except for a period of jaundice and phototherapy as a neonate. In the neurological consultation a brain MRI was requested for the patient to analyze the possibility of brain damage, which showed the involvement of cerebellum, thalamus and the existence of  basal ganglia which led to the diagnosis of ANEC. Conclusion: In conclusion, although ANEC is a rare disease, it should not be underestimated.