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"Iranian Journal of Child Neurology" is a quarterly research journal published by Iranian Child Neurology Society and Child Neurology Department & Research Center of Shahid Beheshti University of Medical Sciences.

ISSN:1735-4668

Online ISSN:2008-0700

This Journal is a member of the Committie on Publucation Ethichs (COPE).

 

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Neonatal seizure: how sure about diagnosis and treatment? A mini review of previous knowledge

Mahmoud Mohammadi, Zahra Rezaei

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 9-16

Seizure is the most common neurologic event in the neonatal period when the immature, growing brain is vulnerable to various injuries. Seizure might be present as an atypical feature in neonates, which makes diagnosis a challenge. A vast majority of seizures are symptomatic due to an underlying cause, searching for etiology to remove them leads to more effective therapy. However, there is doubt about the diagnosis of seizures and the best treatment for them. Here, we reviewed articles related to diagnosis and treatment of neonatal seizures to evaluate the available evidence. The results showed that despite numerous studies on the topic, neither an implicit diagnosing method nor a therapeutic regime was proposed. It was also observed that phenobarbital prescription was reduced while newer anti-seizure medication such as levetiracetam was further prescribed. Seizure, the most common neurologic disorder in neonates, is a challenging topic for both neonatologists and neurologists. Since patients are critically ill, designing a randomized clinical trial appears not to be easy for neonates admitted to a neonatal intensive care unit. Moreover, both the diagnosis and treatment of seizures should be reevaluated based on neonatal characteristics. In the recent decade, new less harmful anti-seizure medications are being replaced with old ones.

Autistic Feature as a Presentation of Inborn Errors of Metabolism

Farzad Ahmadabadi, Hamid Nemati, Amirmohammad Abdolmohammadzadeh, Adel Ahadi

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 17-28

Autism spectrum disorder (ASD) is a category of neurodevelopmental disorders characterized by social and communication impairment and restricted or repetitive behaviors. The pathogenesis of ASD is not well understood and it’s proved that genetic is strongly associated with ASD in 5 to 25% of cases. Inborn errors of metabolism(IEMs), defined by a vast array of disorders that are caused by specific enzyme deficiencies or transport protein defects, is as frequent as in 1 in 800 births. IEMs can manifest several psychiatric or behavioral manifestations such as self-injuriesincreased activity and aggression, personality changes, paranoia, depression, catatonia, and psychosis. IEMs underlie autistic symptoms in less than 5% of cases. The literature on the association between ASD and respiratory chain abnormalities is growing, including complex III/IV deficiency and MELAS (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes) syndrome, as well as glucose-6-phosphate dehydrogenase deficiency. Google Scholar, Pubmed, and SCOPUS databases were searched using a combination of the following keywords: “autism spectrum disorder”, “autism spectrum”, “autistic feature” and “inborn error of metabolism”, “ IEM”, “congenital error of metabolism”. Initially, 655 articles were found and our expert and methodologist altogether selected 187 articles based on the titles, relevance, and text language.
After reading full texts, 37 studies were selected for review. We think it’s best to consider IEMs in children with syndromic ASD and/or if there is a strong familial history of autism or parental consanguineous marriage.

The Effect of the combination of active vestibular intervention and occupational therapy on Balance in Children with Bilateral Spastic Cerebral Palsy: A pilot randomized Controlled trial

Mehdi Rassafian, Nazila Akbarfahimi, Seyed. Ali Hosseini, Soheila Shahshahani, Masoud Karimlou, Farhad Tabatabai Ghomsheh

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 29-42

Objective


This study aimed to examine the effect of the combination of active vestibular interventions and occupational therapy on balance, and the relationship between balance changes and Activity of Daily Living in school-aged children with cerebral palsy (CP).


Materials & Methods


Twenty-four children with Spastic CP, at level I and II according to the “Gross Motor Function Classification System aged 7-12 years were enrolled and randomly assigned into control and intervention groups. Pediatric Balance Scales, Bruininks-Oseretsky Test of Motor Proficiency II were employed to assess the functional balance changes, as well as Force Plate (during eyes closed and open) to assess the parameters of balance changes (e.g., center of pressure excursion), Activity of Daily Living was assessed by “Activity Scales for Kids (performance version)”. Participants in the intervention group received active vestibular intervention for 20 min and regular occupational therapy program for 25 min, and the control group received regular occupational therapy program for 45 min. Interventions were provided 3 d/week for 6 weeks in each group. The participants were assessed in three stages: baseline, immediately after and eight weeks after intervention. Data were analyzed by ANOVA and linear regression.


Results


The results demonstrated that only functional balance according to Pediatric Balance Scales scores was significantly increased in the active vestibular interventions group (p=0.02). There was no significant relationship between functional balance and Activity of Daily Living (P>0.05).


Conclusion


The combined administration of active vestibular interventions and occupational therapy could improve the functional balance in children with spastic CP. It may be related to the reorganization of the vestibular system with a controlled and precise application of stimuli.

Evaluation of the Efficacy of Levetiraceteam Plus Iron in Comparison With Iron Alone in Controlling and Reducing the Frequency of Breath-Holding Spells in Children Aged 6 Months to 5 Years

Ezatollah Abbasi, Ahad Ghazavi, Masoud Hassanvand Amouzadeh, Mohammad Valizadeh, Masoud Matinkhah

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 43-53

Ojective


A breath-holding spell (BHS) is defined as an apnea attack following an initial stressful event like anger, sadness, and fear, a painful event like falling or head trauma or any stressful psychology event. This study was designed to assess the comparative efficacy of levetiracetam plus iron and iron alone in reducing the BHS frequency in children aged 6 months to 5 years


Materials & Methods


This study was designed as a double-blinded randomized clinical trial. Sixty patients aged 6 months to 5 years were assigned into two groups, with the first group (A) receiving only iron and the second group (B) receiving levetiracetam plus iron. At the end of the study, the efficacy of therapy was analyzed comparatively in these groups


Results


In this study, the mean number of attacks was 3.94 ± 2.69 before treatment and 1.71 ± 1.99 after treatment in the group A, while it was 6.39 ± 5.7 before treatment and 0.37 ± 1.03after treatment in the group B. The mean number of attacks after treatment was lower in group B than in group A. In fact, there was a significant difference between the two groups in terms of the number of attacks after treatment (P = 0.003).


Conclusion


Levetiracetam plus iron is more effective than iron alone in reducing breath-holding spells in children aged 6 months to 5 years.

Early screening of Autism among 18 to 24 months old toddlers using Quantitative-Checklist for Autism in Toddlers (Q-CHAT)

Mohammad Reza Mohammadi, Ghazal Zahed, Hadi Zarafshan

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 55-62

Objective


The aim of the present study was to screen the toddlers who are suspected to be autistic in their well-child visits at age 18 to 24 months via the Quantitative-Checklist for Autism in Toddlers (Q-CHAT).


Materials & Methods


After the screening, the screen-positive cases were clinically assessed
by a child psychiatrist and a child developmental psychologist. The total sample included 2467 toddlers; the mean total score of Q-CHAT was 30.64 (SD: 9.133).


Result


According to the clinical examinations, 6 cases met the criteria for autism (equal to 5.8% of screen-positive cases and 0.25% of the total sample), and 18 cases had a high probability of autism or other
neurodevelopmental disorders.


Conclusion


This study showed that the toddlers who were at risk of autism could be screened in PHC. Also, Q-CHAT could be used as a screening tool in Iran.

Social Determinant of Health, Maternal Involvement and Child Development: Direct and Mediated Path ways

Firoozeh Sajedi, Mahbobeh Ahmadi Doulabi, Roshanak Vameghi, Alireza Akbarzadeh Baghban, Farzaneh Rashidi Fakari, Mohammad Ali Mazaheri

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 63-76

Objectives


In the process of child development, a variety of factors are at play. In this regard, social determinants of health play a determining role in the development and growth of the child.


This study aimed to design and test the model for social determinants of health for the development of 36-6o-month-old children in Tehran with the mediation of maternal Involvement.


Materials & Methods


This cross-sectional study was conducted among 1067 mothers and their 36-60-month-old children in childcare centers in Tehran, using multistage sampling.


Data gathering tools consisted of a demographic questionnaire for mothers and children, a questionnaire on unhealthy behaviors, Ages and Stages Questionnaire, Economic and Social Status Questionnaire, Perceived Social Support Questionnaire, Perceived Stress Questionnaire, Spielberger Anxiety Inventory, Beck Depression Inventory, ENRICH: Marital Satisfaction Scale, and Participation Scale for Parents and Mothers.


Results


Model fit measures were suitable and goodness of fit (RMSEA = 0.031, GFI = 1) was satisfactory. In addition, the results of path analysis indicated that the participation of mothers in the development of children had a direct positive (ß = 0.089) and increasing effect.


Conclusion


Findings indicated that depression, anxiety, stress, and marital satisfaction have both direct and indirect effects on the participation of mothers and child development. Moreover, the model fit measures indicated the utility and high proportionality of the model, as well as the logic of the adjusted relationships of variables based on the
conceptual model.

Efficacy of Topiramate Alone and Topiramate Plus Vitamin D3 in the Prophylaxis of Pediatric Migraine: A Randomized Clinical Trial

Razieh Fallah, Saeedreza Sarraf Yazd, Seid Mojtaba Sohrevardi

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 77-86

Objective


Topiramate is effective in the prevention of pediatric migraine, and studies show that vitamin D3 supplementation might also be useful in the treatment of adult migraineurs with a normal vitamin D3 level.
The present study aimed at comparing the efficacy and safety of topiramate plus vitamin D3 and topiramate alone in the prophylaxis of pediatric migraine.


Materials & Methods


In a single-blinded, randomized, clinical trial, 5-15-year-old children with migraine headaches, referred to the Pediatric Neurology Clinic of Shahid Sadoughi Medical Sciences University, Yazd, Iran from January 2016 to January 2017, were randomly allocated to receive 2 mg/kg/day of topiramate or 2 mg/kg/day of topiramate plus one 500,000 IU vitamin D3 pearl weekly for two consecutive months.


Primary outcomes were the reduction of monthly frequency, severity, duration, and the disability score of migraine, and the secondary outcomes included a good response to treatment (more than 50% reduction in monthly headache frequency) and a lack of clinical adverse events.


Results


Totally, 31 female and 26 male children with the mean age of 10.02±2.11 years were evaluated. Both drugs were effective in the reduction of monthly frequency, severity, duration, and disability for headaches. Nevertheless, the combination of topiramate and vitamin D3 was more effective than topiramate alone in reducing the monthly headaches frequency (6.12±1.26 vs. 9.87±2.44 times, P=0.01) and disability score (19.24±6.32 vs. 22.11±7.91, P=0.02). Good response to treatment was observed in 60.7% and 75.9% of the subjects in the topiramate alone and topiramate plus vitamin D3 groups, respectively, and topiramate plus vitaminD3 was more effective (P= 0.01). Transient mild side effects were observed in 14.3% and 17.2% of the subjects in the topiramate alone and topiramate plus vitamin D3 groups, respectively (P=0.8).


Conclusion


A combination of Topiramate and vitamin D3 might be considered safe and more effective than Topiramate alone in the prophylaxis of pediatric migraine.

A case report of congenital myasthenic syndrome caused by a mutation in the CHRNE gene in the Iranian population

Zahra Farjami, Negar Khodaienia, Neshat Ebrahimi, Gholamreza Zamani Ghaletaki, Amirhossein  Ashnaei, Mohammad Galehdari, Mehdi Moradyar, Massoud Houshmand

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 87-94





Congenital myasthenic syndrome (CMS) refers to a heterogeneous group of inherited disorders, characterized by defective transmission at the neuromuscular junction (NMJ). Patients with CMS showed similar muscle weakness, while other clinical manifestations are mostly dependent on genetic factors. This disease, caused by different DNA mutations, is genetically inherited. It is also associated with mutations of genes at NMJ, involving the acetylcholine receptor (AChR) subunits. Here, we present the case of a five-year-old Iranian boy with CMS, undergoing targeted sequencing of a panel of genes, associated with arthrogryposis and CMS. The patient had six affected relatives in his genetic pedigree chart. The investigations indicated a homozygous single base pair deletion at exon 12 of the CHRNE gene (chr17:4802186delC). This region was conserved across mammalian evolution and was not submitted to the 1000 Genomes Project database. Overall, the CHRNE variant may be classified as a significant variant in the etiology of CMS. It can be suggested that the Iranian CMS population carry regional pathogenic mutations, which can be detected via targeted and whole genome sequencing.





Agenesis of the Corpus Callosum: a Rare Association with Ehlers-Danlos Syndrome

Motahareh Afrakhteh, Mostafa Almasi-Dooghaee, Fahimeh Haji-Akhoundi

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 95-99

Ehlers-Danlos syndrome (EDS) is a rare congenital disorder of connective tissues which involves the skin and musculoskeletal system. There are also some reports for the involvement of the central and peripheral nervous systems. We want to present a very rare coassociation of EDS, spondylolisthesis, and Agenesis of the corpus callosum in an Iranian lady.

Congenital spinal dysraphism with infected sacrococcygeal sinus tract: need for improved awareness amongst clinicians

Shameem Ahmed, Deep Dutta, Siba Prosad Paul

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 101-105

Spinal dysraphism (SD) includes a group of developmental anomalies resulting from failure of fusion of parts along dorsal aspect of midline structures lying along spinal axis from skin to vertebrae and spinal cord. There are two types of SD, open and closed. Close SD, also known as spina bifida occulta, can present with diagnostic challenges in resource limited settings where awareness regarding the condition and specialist radiological investigations, including Magnetic Resonance Imaging (MRI), may not be easily available. Undiagnosed cases can potentially lead to long term morbidities. We report the case of a 13-year old boy with closed SD presenting with recurrent infections of the sacrococcygeal sinus tract which were treated with oral antibiotics for what was considered to be localized infection. Following neurosurgical assessment and spinal MRI a diagnosis of SD was made. He underwent surgical excision of the sinus tract and closure of the defect with good outcome. The case emphasizes the need for awareness regarding SD in children who have sinus tracts in the intergluteal fold with symptoms of recurrent discharge and infection.

Case report partial seizure due to COVID19 infection in an infant

Saeed Anvari, Alireza Nateghian

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 107-109

We live at the time of the coronavirus pandemic in the world (1, 2). The symptoms of COVID19 are similar in children and adults. However, children with confirmed COVID19 have generally shown mild symptoms (3). The symptoms in children include cold-like symptoms, such as fever, runny nose, and cough, vomiting, and diarrhea. In this study, we describe an eight-month-old boy with recurrent partial seizure and mild diarrhea. It was later revealed that he was COVID19 positive

Succinate Dehydrogenase Deficiency, a Treatable Neurometabolic Disorder

Parvaneh Karimzadeh, Mohammad Keramati Pour, Arezou Karamzade, Elham Pourbakhtyaran

Iranian Journal of Child Neurology, Vol. 14 No. 4 (2020), 10 October 2020 , Page 111-116

Succinate dehydrogenase (SDH) deficiency is a rare autosomal recessive neurometabolic disorder that causes brain insult, neurodevelopmental delay, exercise intolerance, and cardiomyopathy. A 25-month-old boy was referred to our neurometabolic center due to developmental regression after injecting the influenza vaccine when he was 10 months old. Magnetic resonance imaging (MRI) showed high signal changes in the brain white matter, and magnetic resonance spectroscopy (MRS) detected a high succinate peak at 2.4 parts per million (ppm). The evaluation of urine organic acids showed a significant elevated succinic acid and whole exome sequencing, confirming SDH. Treatment with a mitochondrial cocktail was initiated, and remarkable improvement was observed. SDH deficiency as a treatable neurometabolic disorder should be considered in any patients with developmental disorders, accompanied by hyperintensity in white matter (as similar to leukodystrophia). Further evaluation is recommended since outcomes depend on early diagnosis and treatment.


 

 

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