Review Article

Cognitive Prerequisites in Development of Childhood Anxiety: An Integrative Literature Review and Thematic Analysis

Samaneh Behzadpoor, Hamidreza Pouretemad, Saied Akbari zardkhaneh

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 9-27

It has been suggested that cognitive development affects the emotional experience of children, including anxiety. However, an evidence review is needed to extract cognitive prerequisites that contribute to the development of anxiety in children. The purpose of the study is to explore evidence on cognitive prerequisites involved in experiencing anxiety in children. Four electronic databases of Scopus, OVIDPsycINFO, PubMed, and ScienceDirect were comprehensively searched for 1900 to 2018, yielding 4,618 articles. According to the Preferred Reporting Items for Systematic Reviews and Meta- Analysis (PRISMA) and inclusion and exclusion criteria, 25 articles were found as eligible. The analysis of literature identified 3 themes, including threat perception, future thinking, and generalization. It is suggested that these cognitive abilities may underlie anxiety. These results have important implications for better understanding the effect of cognitive prerequisites in anxiety phenomena and also could shed light on the explanation of anxiety in some disorders characterized by deficits in cognitive development.

Research Article

Comparison Of Chloral Hydrate Solution, Hydroxyzine Syrup And Lidocaine/Prilocaine Cream As Premedication For Lumbar Puncture In Children, A Double Blind Study

Hossein esfahani, Iraj Sedighi, Maryam Sahraei Ardakani, Mohammad Ali Seif Rabie, firozeh hosseini

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 29-34


This study aimed to compare the clinical effectiveness of oral hydroxyzine and chloral hydrate to topical lidocaine/prilocaine 2.5% cream as premedication in pediatric leukemia patients.

Material & Methods

This double-blind clinical trial study was conducted on 70 leukemic and non-leukemic patients aged 3-11 years old. The patients were divided into 4 groups as follow: in the group A, choral hydrate solution was given in 18 patients, in group B hydroxyzine syrup was used in the same number of patients, in group C, chloral hydrate solution and Lidocaine/Prilocaine cream were used in 17 patients, and in group D hydroxyzine syrup and Lidocaine/Prilocaine cream were given in the same number of patients. These groups were assessed and judged based on visual analog scale (VAS). Side effects of the drugs were also recorded.


In this study, 54.3% (38) of cases were girls and 45.7% (32) were boys. Patients’ selection was included as follow: ALL in 77%, AML in 7.2% and non-leukemic cases in 15.8% of cases. The VAS test showed no difference in these four groups. Non-Traumatic lumbar puncture (RBC<50) were seen in 97.1% of cases.


Although the use of pre-medications for lumbar puncture with hydroxyzine syrup and chloral hydrate solution was not statistically effective, it did lead to increased patient and parent satisfaction. Also, adding  the Lidocaine/Prilocane cream does not improve the effectiveness of the drugs.


Effect of Curcumin on Pediatric Intractable Epilepsy

Mina Erfani, Farah Ashrafzadeh, Hamid Reza Rahimi, Seyed Ali Ebrahimi, Keivan Kalali, Mehran Beiraghi-Toosi, Elnaz Faraji Rad

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 35-45

Epilepsy is the most prevalent chronic neurologic disorder in children. One-third of patients with epilepsy do not respond to antiepileptic drugs. This condition is known as intractable epilepsy. Previous studies have shown the beneficial effects of curcumin in the treatment of epilepsy. There are no randomized controlled clinical
trials assessing the use of curcumin in epilepsy. This study aimed to evaluate the effects of nanomicelle curcumin on intractable pediatric epilepsy.

Materials & Methods

This double-blinded randomized crossover clinical trial was performed by a consecutive sampling to select 22 patients with intractable epilepsy divided into two groups. Patients received a daily dose of 4 mg/kg of curcumin or placebo as add-on therapy for 4 weeks. After a 2-week washout period, the treatment was replaced, and the new treatment was given for another 4 weeks. The SPSS software version 16 was used for statistical analysis. The study was approved by the Ethics Committee of Mashhad University of Medical Sciences, Iran


A total of 22 children were enrolled in this study, 11 of which were boys. The mean age of the patients was 4.28±5 years. A female patient taking a placebo was excluded in the first week of the trial due to parental dissatisfaction. The most common type of seizure among our patients was a generalized myoclonic seizure (42.9%). The mean number of seizure attacks among the subjects was 68.76±69.26 preintervention and 39.85±39.41at the end of the intervention, which represents a statistically significant difference (P=0.01).


Nanomicelle curcumin reduced the number of seizures significantly. Our results imply that curcumin treatment can help treat patients wit intractable pediatric epilepsy.

The Efficacy of Addition of Atomoxetine to Speech Therapy on the Stuttering Severity of Children Aged 4-12 Years; a Double-Blind Controlled Randomized Clinical Trial

Farzad Ahmadabadi, Abdullah Motamedi , ghazal zahed, Akram motamedi, Farshid Shahriari, Farhad Pourfarzi, Narjes Jafari, Mohammad Mehdi Hosseini, Mohammad Mehdi Hosseini

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 47-56



Stuttering is a common problem at all ages and it is thus required to treat this problem since childhood. Atomoxetine is currently used for the treatment of attention deficit hyperactivity disorder (ADHD) and can also be effective for the treatment of stuttering due to its selective inhibition of norepinephrine reuptake and dopaminergic properties. Therefore, this randomized clinical trial (RCT) aimed to evaluate the effect of Atomoxetine on children’s stuttering.

Materials & Methods

Children aged 4–12 years, diagnosed with stuttering, who referred to pediatric neurology clinic, were randomly divided into experimental (N=50) and control (N=50) groups. One group received atomoxetine plus speech therapy and the other group only speech therapy. Both groups completed the Stuttering Severity Questionnaire (SSI4) at baseline (on the first visit) and three months after the intervention.


Most (67%) were boy; 24% aged <60mo,46% 60–95mo,and 30% >95mo. About half (52%) had a positive family history of stuttering. Stuttering severity was highest at ages of 60–95mo, in left–handed children,those who used formula,and those who felt insecure in the family; but was not different based on child’s sex, concomitant ADHD, multilingualism, facial or movement tics, based on sleeping hours, and using teats. Mean stuttering severity reduced in both groups (P<.001) with a greater decrease in the experimental group, compared to the control group (P=.011).


Atomoxetine,plus speech therapy,is effective for the treatment of children’s stuttering and can be used as a complementary treatment strategy in these patients.

The effect of low-grade intraventricular hemorrhage on neurodevelopment of very low birth weight infants

Omid Yaghini, Maede Majidinezhad, Azam Ghehsareh Ardestani , Maryam Riahinezhad, Mohammad Reza Maracy

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 57-65



Intraventricular hemorrhage (IVH) is a significant concern for premature very low birth weight (VLBW) neonates worldwide. Recently, the popular theory of the benign nature of low-grade IVH has been argued with uncertain outcomes. This study has aimed to assess the effect of low-grade IVH on neurodevelopment of VLBW neonates.

Materials & methods

This was a six-month follow-up cohort study conducted on VLBW neonates with and without grade I-II IVH diagnosed through brain ultrasonography. The participants were neurologically examined at birth and within six months. Their neurodevelopment was assessed using Bayley-III questionnaire evaluating cognition, receptive language, expressive language, fine motor and gross motor performance.


100 VLBW neonates including 40 cases with grade I-II IVH diagnosed through brain ultrasonography and 60 controls were recruited. The cases and controls were similar considering gestational age, body birth weight, hospitalization duration, gender distribution and age at Bayley-III evaluation (P>0.05). The neurological assessments at birth showed no significant difference between the two groups (P=0.20), while controls were significantly showed better results in the sixth month of age (P =0.004). Assessing different neurodevelopmental indices, after controlling for demographic characteristics and respiratory-related variables at the time of Bayley-III evaluation, There was a higher performance in cognition and gross motor aspects in controls compared with cases (P= 0.04 and 0.03, respectively).


The low-grade IVH affected the sixth-month neurological examination and gross motor performance of the VLBW newborns. Notably, cognition and gross motor were the two affected subscales in the presence of low-grade IVH, independent of demographic factors.


Given the importance of having a continuous performance for the academic and social life of children with attention-deficit/hyperactivity disorder (ADHD), in this study, a continuous performance test was used to compare the effect of long-acting methylphenidate and modafinil on attention and impulsivity of these children.

Materials & Methods

A randomized clinical trial was conducted on 50 children aged 6 to 12 years with ADHD in the child and adolescent psychiatric departments of Imam Hossein and Mofid hospitals, Tehran, Iran. The children were selected by availability sampling and randomly divided into two groups of 25. One group was treated with long-acting methylphenidate and the other with modafinil for 14 days. The continuous performance test was carried out before and after the treatment. The obtained data were analyzed by F and t tests


Long-acting methylphenidate and modafinil were both effective in improving attention and impulsivity in children with ADHD. There was no significant difference between the two drugs in terms of effectiveness on attention and impulsivity.


The findings of this study showed that long-acting methylphenidate and modafinil are equally effective in improving attention and impulsivity in children aged 6 to 12 years with ADHD.

Development and psychometric evaluation of the Persian version of the Phoneme Recognition Test A central auditory processing measure

Sahar Shomeil Shushtari, Farzaneh Fatahi, Nematallah Rouhbakhsh, Nader Saki, Shohreh Jalaie, Ehsan Negin, Mojtaba Tavakoli, Majid karimi

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 79-93



The present study evaluating the psychometric properties of the Persian version of the Phoneme Recognition Test (P-PRT) in normal subjects and cochlear implant (CI) users.

Material & Methods

This study includes developing the Persian phoneme recognition test (PRT), determining its validity and reliability, and comparing the results of a control group versus CI users. The test reliability was examined through a test-retest with an approximately five-week interval. In the present survey, 363 subjects were investigated in three stages. The face validity evaluation stage was conducted on 40 subjects. The psychometric properties of the P-PRT were evaluated in 323 individuals (225 normal subjects and 98 CI users). The test-retest reliability was examined in all the 225 subjects in the control group
and 40 CI users.

The results confirmed the face validity of the P-PRT. No significant differences were observed between the two genders in terms of performance in the P-PRT. Significant differences were observed between the control and CI groups. Evaluating the test-retest reliability suggested perfect reliability (r>0.9) in both groups. Significant differences were observed in the P-PRT between the adults and the 7-year-old subjects compared to other age groups.


The P-PRT can be used as a valid and reliable test for clinically evaluating phoneme recognition abilities and monitoring the rehabilitation progress

Prevalence of Psychiatric Disorders among Children and Adolescents: A Study from Khuzestan

Frough Riahi, Mohammad Reza Mohammadi, Maryam Izadi, Ali Khaleghi, Zahra Hooshyari

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 95-107



This cross-sectional study aimed to study the prevalence rate of psychiatric disorders in children and adolescents in Khuzestan province.

Materials & Methods

A community sample consisting of 1028 (51.6% female) children and adolescents aged 6-18 years was selected using a multistage cluster sampling method. Data were gathered using the Kiddie-SADSPresent and Lifetime Version (K-SADS-PL) and a demographic questionnaire (i.e., gender, age, level of education, place of residence, parent’s education, and parent’s Job)


Nearly 22.6% (22.3% of boys and 23% of girls) of all participants suffered from at least one psychiatric disorder. There was no significant difference in the prevalence of psychiatric disorders based on gender, age, father’s education, mother’s education, mother’s job, and father’s job (all p>0.05). Psychiatric disorders were significantly
more prevalent among children and adolescents in urban areas compared to rural places (2.9% vs. 8.1; p<0.001). The most prevalent category was anxiety disorders (15%). Also, the most common disorders were specific phobia (7%), separation anxiety disorder (6.3%), and enuresis (5.2%). The most common comorbid disorders were mood disorders and anxiety disorders (56.3%), followed by anxiety disorders and elimination disorders (32.1%).

Psychiatric conditions are prevalent in children and adolescents living in Khuzestanian. The study’s findings have important implications for providing effective psychiatric services.



Evaluating the Correlation between Brain Ultra Sonographic, Brain MRI, and Electroencephalography Findings and the Severity of Asphyxia and Neurodevelopment in Infants with Hypoxic-ischemic Injury

Ahmad Enhesari, Habibe Nejad Biglari, Mohammad Shafie’ei, Mahsa Sirooee Nejad, Zahra Daei Parizi, Rana Eftekhar Vaghefi

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 109-119

Hypoxia-ischemia-induced brain injury is a major cause of acute mortality and chronic neurological disability in infants and children.
Imaging plays a vital role in diagnosing and treating hypoxicischemic encephalopathy (HIE) and as an adjunct to acute conditions and provides valuable information on long-term prognosis.
Materials & Methods
Our study was prospective with 50 neonates aged 34 weeks and older with HIE. Cerebral ultrasound and MRI were performed on the infants, and the pattern of lesions was recorded. A pediatric neurologist examined the infants, and their developmental status was assessed and recorded with electroencephalography (EEG) findings.
The data were analyzed.

The sonography pattern was normal in 26 (76.5%) term neonates, and also, the PVL pattern was observed in 10 term neonates. The incidence of observing an edema pattern (17.6%) was significantly different between the term and pre-term infants (P-value = 0.001).
MRI findings were normal in 20 (58.8%) term neonates and 11 premature neonates. However, the PVL pattern was observed in MRI performed in six term neonates (6.6%). The watershed pattern (17%) showed that these differences were significant between the term and pre-term infants (P-value = 0/001).

Normal sonography was significantly higher in neonates with normal neurodevelopment than in patients with normal MRI and EEG findings but with poor neurodevelopment. Also, the probability of having normal MRI results was lower in neonates with moderate to severe asphyxia compared to ultrasound and EEG.


Study of changes in rs2283265 polymorphisms in dopamine receptor D2 and rs27072 in dopamine transporter gene (SLC6A3) in patients with attention-deficit hyperactivity disorder

Parvin Safavi, Hossein Soleimani Farsani, Effat Farrokhi, Afsaneh Malekpour Tehrani, Nika Khoshdel, Abolfazl Khoshdel

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 121-132

Attention-deficit hyperactivity disorder (ADHD) is one of the most common psychiatric disorders in children that lead to numerous complications. This study examined the changes in rs2283265 polymorphisms in the dopamine receptor D2 (DRD2) and rs27072 in the dopamine transporter gene (SLC6A3) in ADHD patients.
Materials & Methods
This descriptive-analytical study was performed on children aged 4-12 years with ADHD. In this study, 100 patients in the ADHD group (according to DSM-IV-TR criteria and diagnosed by interview by a child and adolescent psychiatrist) and 100 children in the control group (including patients referring to the pediatrician without hyperactivity) were enrolled.
Two polymorphisms rs2283265 and rs27072 in two groups were comparatively investigated using PCR-RFLP method and restriction enzymes. Data were analyzed using SPSS 17.

There was a significant correlation between gender and ADHD, and the disease was more common in boys (P=0.021). In this study, there was no significant relationship between ADHD types and frequency distribution of rs2283265 (DRD2) and rs27072 (SLC6A3) polymorphism genotypes (P<0.05). However, there was a significant correlation between distribution of rs2283265 (DRD2) and rs27072 (SLC6A3) polymorphisms and ADHD (P<0.05).

It seems that the changes in DRD2 and SLC6A3 genes are associated with ADHD, and study of these genes can be helpful in diagnosis and genetic screening.

Patterns of Antiepileptic Drug Reactions in Children: A Multicenter Study

parinaz sedighi, Neda Khalili, Nastaran Khalili, Amin Doosti-Irani, Atefeh Moradi, Samira Moghadam, Meshkat Nemati, Sobhan Mohammadi Jorjafki, Reza Shervin Badv, Iraj Sedighi

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 133-143

Antiepileptic drugs are among the most common triggers of cutaneous adverse reactions. About 5–17% of epileptic patients develop idiosyncratic skin reactions at some point during their treatment course, most of which occur within the first two months of drug initiation. This study aimed to investigate the pattern of cutaneous drug reactions associated with anticonvulsant use among the pediatric population in Iran to identify high-risk individuals.

Materials & Methods
In this retrospective descriptive study, medical records of children aged two months to 14 years, who were diagnosed with drug reactions due to anticonvulsant drugs between April 2007 and March 2018, were reviewed, and relevant information were extracted. This multicenter study was conducted in several provinces of Iran.
A total of 186 cases with a final diagnosis of the antiepileptic druginduced
eruption were evaluated. The median age of participants was
36 months (range: 2-168), and 56% were male. In approximately
70% of the children, phenobarbital was the culprit. The median time
interval between initiation of the causative drug and development of
rash and fever was 10 and 7 days, respectively. The most common
rash type was maculopapular rash (69%). Overall, 33% of the patients
only received antihistamines after discontinuation of the causative

Similar to previously published studies in Iran, phenobarbital was
the main cause of cutaneous drug reactions to antiepileptic drugs,
indicating the necessity of paying more attention when prescribing
phenobarbital for Iranian pediatrics.

Assessment of neuropsychiatric indicators in children and adolescents with primary brain tumors and other brain space occupying lesions before and after surgery

Samira Nasehi, Ayyoub malek, Ali Meshkini, Ali Reza Shafiee-Kandjani, Shahrokh Amiri, Firooz Salehpoor, Farhad Mirzaei, Hasan Shahrokhi, Saeed Dastgiri, Sara Farhang

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 145-156



Cognitive abilities may be impaired due to brain lesions in children and adolescents. This study aimed to investigate neuropsychiatric indicators in children and adolescents with primary brain tumor and other brain space occupying lesions (SOL) before and after surgical procedure.

Methods & materials

the current study is a pre and post study which was conducted on 81 patients with brain space occupying lesions aged less than 18. Patients with metastatic brain tumors were excluded. The study was performed between 20 December 2016 to 20 December 2017 on patients hospitalized in neurosurgery ward of Imam Reza university hospital, Tabriz, Iran. Before and after surgical procedure, Digit span forward and backward Task (to assess working memory), Stroop task and Trail Making Task A & B (to assess attention) and Rey Osterrieth Complex Figure Test (to assess Visual Spatial Memory) were done. Then, scores of tests were compared with normal values as well as the post-surgery scores.


the most prevalent type of space occupying brain lesion was medulloblastoma and the most common region of involvement was posterior fossa tumor. Scores of all tests after surgery comparing to before surgery were significantly improved (P<0.05). In assessment of Digit span forward and backward Task with standard scores, there was no significant difference among scores of patients before surgery with the standard value (P>0.05). Regarding scores of various stages of Rey Osterrieth Complex Figure Test, the  scores of immediate recall stage was significantly low (P<0.05). Among Trail Making Task A & B and stroop task, before surgery, just the Trail Making Task A & B was significantly increased (P<0.05). Scores of Trail Making Task A was significantly higher in patients with medulloblastoma and anatomically in left temporal tumors which indicate greater damage of attention field (P<0.05). In addition, in cerebellar tumor, scores of immediate recall stage of Rey Osterrieth Complex Figure Test was significantly lower (P<0.05).


Visuo-Spatial Memory and attention in pre-surgery assessments was significantly impaired comparing to general population (P<0.05). Working memory, Visuo-Spatial Memory and attention showed improvement comparing to pre-surgery. Deficits in attention domain was greater in medulloblastoma.

Seizures are the most common neurological illness in the pediatric population and account for 1% of all emergency department (ED) visits and 2% of all visits to children’s hospital EDs. Pediatric epilepsy presents with various diagnostic challenges. Neuroimaging, especially structural neuroimaging and preferably MRI brain, plays an essential role in diagnosing, managing, and guiding pediatric epilepsy treatment.
The study aimed to estimate the clinical spectrum of seizures in children and examine the neuroimaging findings in children with seizures.

Materials & Methods
The study was a hospital-based retrospective observational study. The hospital case records of all children belonging to the age group 1 month to 12 years with ‘seizures’ were reviewed for 5 years from Jan 2016 to Dec 2020. Clinicodemographic profiles and neuroimaging (CT/MRI) findings were obtained, and descriptive statistics were
A total of 838(11%) children in the age group 1 to 144 months (mean±SD: 32.57±32.65) presented with seizures, of whom 515(61.5%) were boys and 323(38.5%) girls. Of 596(71.1%) children under five years, 409(68.6%) had febrile seizures. Generalized onset-motor seizures were the predominant type of seizures seen in 666(79.4%) children, of whom 434(65.1%) had febrile seizures.
Neuroimaging (CT/MRI) was normal in 335(40%) and abnormal in 124(14.8%) children. Perinatal insult (7%) was the most common abnormality, followed by CNS infections (2.8%).
Neuroimaging, preferably MRI brain, is the most helpful tool for the
etiological diagnosis of afebrile seizures. In our study, seizures secondary to perinatal insult/hypoxic insult followed by infections were major causes. Improvement in peripartum and perinatal care coupled with a targeted Tuberculosis control program may help in reducing these potentially preventable causes

The preventive Effectiveness of Rituximab in Pediatric Autoimmune and Inflammatory CNS Diseases Relapse: an Iranian Experience

Mohammad Mahdi Nasehi, Mohammad Ghofrani, Aydin Tabrizi, Fatemeh Abdollah Gorji, Bakhtyar Khosravi

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 167-182

To determine the effectiveness of Rituximab (RTX) therapy as the first therapeutic choice for the long-term prevention of secondary relapse in children with Autoimmue Neurological Disease (AIND) had relapse after primary treatment with immunosuppressive agents other than RTX.
Materials & Methods

We conducted a single-center retrospective study of 9 consecutive pediatric patients (≤ 18 years old) registered on Autoimmune and Demyelinating Disorders Database (ADDD) of Mofid Children Hospital, from 2012 to 2016 and experienced relapse following therapeutic interventions with immunosuppressive agents other than RTX.
A remarkable reduction of 94.13% (p=0.015) occurred in annualized relapse rate (ARR) as a clinical indicator of therapeutic efficacy comparing before and after initiating RTX therapy.
Rituximab is an effective drug in relapse prevention of AIND whenadministrated to patients for whom initial treatment with other immunosuppressive agents fail. POWER OF EVIDENCE: This study represents Class IV evidence that RTX therapy significantly reduces ARR in pediatric AIND including DDCNS.


Evaluation of brain CT scan, sonography and EEG findings in neonates with seizure

Mirhadi Mussavi, Shokoufeh Khanzadeh, Shahram Sadeghvand, Eslam Hasanpour, Morteza Akbari , ,

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 183-191

This study aimed to determine the frequency of different types, causes, and abnormal findings of brain computed tomography scan (CT scan) and ultrasonography (US) and multichannel- electroencephalography
(EEG) in neonates with seizure. The ability of brain CT scan was also compared with US in terms of detecting the underlying causes of neonatal seizures.
Materials & Methods
In this cross-sectional retrospective study, the medical records of 90 neonates younger than 28 days with the definite diagnosis of seizure were reviewed. The ability of brain CT scan was also compared with US in terms of detecting the underlying causes of neonatal seizures.
Totally, 90 newborns (M: F = 1.5:1) with mean age of 63.11 ± 32.8 days were enrolled. 35.5% of newborns were born before the 37th week of pregnancy. In this study, 45.6% of EEG findings, 22% of brain CT scan findings, and 12.5% of US findings were abnormal. The automatisms (38.9%) and benign idiopathic neonatal seizure (70.7%) were the most common seizure type and cause respectively. The hypoxic Ischemic encephalopathy was the most common abnormal finding (30%) in brain CT scan.

Given the accuracy of EEG in detection of brain pathologies, where available, all neonatal seizures should be initially confirmed using EEG. Radiologic investigations (CT scan, US) off the head/ craniumshould be done to detect the cause of neonatal seizure. The capacity of brain CT scan to detect underlying causes of neonatal seizures is more than US


Neurometabolic Disorder Articles

Infantile Neuroaxonal Dystrophy in Two Cases: Siblings with Different Presentations

Behnaz Ansari, Jafar Nasiri, Hamide Namazi, Maryam Sedghi, Mahdieh Afzali

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 193-198


Infantile neuroaxonal dystrophy (INAD) is a rare recessive neurodegenerative disorder manifested by symptoms, including hypotonia, extrapyramidal signs, spastic tetraplegia, vision problems, cerebellar ataxia, cognitive complications, and dementia, before the age of three. Various reports evaluated the relationship with the incidence of INAD and different mutations in the PLA2G6 gene. We describe cases of two children with INAD whose diagnoses were challenging due to misleading findings and had a mutation in the position C.2370 T>G (p. Y790X) in the PLA2G6 gene based on NM_001349864 which has been reported previously.

Case Report

A Spontaneous dissecting posterior cerebral artery aneurysm in a 10-month-old female infant: a case report

Seyed Hossein Aghamiri, Sohrab Salimi, Atefe Sepehrirad, Mohammad Sistanizad, Elham Pourheidar

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 199-203


Posterior Cerebral Artery (PCA) aneurysms are a rare group of intracranial aneurysms. Pediatric aneurysms account for approximately 4% of all aneurysms and commonly are associated with an underlying predisposing disorder such as trauma. In contrast to traumatic aneurysms, spontaneous dissecting aneurysms are pointed out as a rare cause of pediatric aneurysms. aimed to present one case of a spontaneous dissecting aneurysm in a 10-month-old female infant.


Intracranial angiomatoid fibrous histiocytoma: report of a rare case

Alireza Tabibkhooei, Leila Asgarzadeh, Alireza Sadeghipour, Mohammad Vafaee Shahi

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 205-212

One rare and low-grade soft tissue tumor with intermediate malignant potential is angiomatoid fibrous histiocytoma (AFH) و which occurs mainly in children and adolescents. The tumor naturally tends to local
recurrence and recurrent hemorrhage but rarely to remote metastasis.
AFH has been reported in different organs; however, there are rare reports of primary intracranial AFH. The diagnosis of AFH may be difficult due to its occurrence at multiple unusual anatomic sites and its spectrum of morphologic patterns; thus, it is especially importantto diagnose it correctly because of the small risk of metastasis and death. The lesion is simply confused with a hematoma, soft tissue hemangioma, or malignant fibrous histiocytoma from clinical and radiographical aspects.
We report a case of intracranial AFH in a 5-year-old boy. The tumor is a heterogeneous intra-axial with a size of 78*73mm at the right front temporal. There was also an extra-axial mass measured 8*12mm at the left superior frontal lobe in favor of metastasis. The diagnosis was confirmed using radiographical, immunohistochemical, and molecular tests.
AFH is a rare tumor with a high probability of misdiagnosis. Surgeons must be aware of the presence of AFH and conduct a careful followup

Delayed hypoxic encephalopathy: a rare complication of methadone poisoning in two cases

Saeed Abdollahifard, Fatemeh Kheshti, Soroor Inaloo

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 213-217


Methadone is a kind of opioid that is used to reduce the pain of addicts who decide to withdraw drugs. Sometimes due to a lack of appropriate cautions, this drug will be accessible to children, andpoisoning might occur. Methadone poisoning usually presents with the loss of consciousness and pinpoint pupils. Herein, we present two cases of delayed hypoxic encephalopathy that had been poisoned by methadone


Ewing’s sarcoma is a highly malignant bone tumor commonly affecting diaphysis of long bones & pelvic bones. It belongs to a spectrum of neoplastic disease known as the Ewing sarcoma family of tumors (EFT) that have the same histologic, immunohistochemical characteristics and chromosomal translocation. It commonly affects children between the ages of 5-15 years. Spinal involvement might be primary or metastatic. Primary spinal involvement is very rare. We report a 7-year female with acute paraplegia caused by dorsal osseous Ewing’s sarcoma that her presentation mimics other causes of acute lower limb weakness in children.  Early detection of this rare case and prompt treatment will ensure better outcomes for the patient. 

Hypertonia, Microcephaly And Hyperkalaemia In A Neonate: Coexistence Of Neurodevelopmental Disorder And Adrenal Insufficiency

Usha Devi R, Nirmalan Dhakshanamoorthy, Prakash Amboiram, Umamaheswari Balakrishnan

Iranian Journal of Child Neurology, Vol. 16 No. 3 (2022), , Page 223-228


In neonates with more than one clinical abnormality, we always look for a unifying diagnosis that explains the entire clinical presentation. In rare instances, two conditions can co-exist. Here we report a neonate born out of consanguineousmarriage presenting at 48 hours of life with microcephaly, encephalopathy, hypertonia. He had excessive weight loss,persistent hyperkalaemia, shock and 17- hydroxyprogesterone was elevated. Steroids were started for adrenal insufficiency. Magnetic  resonance imaging (MRI) of the brain revealed T2 hyperintensity of cerebral white matter and cerebral atrophy. Clinical exome sequencing (CES) revealed a pathogenic homozygous missense variation of CYP21A2 gene responsible for congenital adrenal hyperplasia and also a homozygous missense variant of unknown significance (VUS) of VARS gene implicated in Neurodevelopmental disorder with microcephaly, seizures, and cortical atrophy (NDMSCA). Baby was neurologically abnormal at discharge. In the setting of consanguinity, there is a possibility of two genetic conditions. Clinical exome sequencing is useful in demystifying the diagnosis in complex clinical presentation.