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In this article, we conducted a case–control study to determine the effects of migraine on quality of life (QOL) in children. Participants, aged between 6 and 18 years, were assigned to migraine (n = 70) and control (n = 70) groups. We used a translated version of Pediatric Quality of Life Inventory 4.0 questionnaire. From the children's perspective, the overall QOL scores in all domains were lower in children with migraine. Children in the migraine group had significant lower scores in the social, emotional, and physical functioning (p-value

Background Cerebellar ataxia, mental retardation, and dysequilibrium (CAMRQ) syndrome is a rare and early‐onset neurodevelopmental disorder. Four subtypes of this syndrome have been identified, which are clinically and genetically different. To date, altogether 32 patients have been described with ATP8A2 mutations and phenotypic features assigned to CAMRQ type 4. Herein, three additional patients in an Iranian consanguineous family with non‐progressive cerebellar ataxia, severe hypotonia, intellectual disability, dysarthria, and cerebellar atrophy have been identified. Methods Following the thorough clinical examination, consecutive detections including chromosome karyotyping, chromosomal microarray analysis, and whole exome sequencing (WES) were performed on the proband. The sequence variants derived from WES interpreted by a standard bioinformatics pipeline. Pathogenicity assessment of candidate variant was done by in silico analysis. The familial cosegregation of the WES finding was carried out by PCR‐based Sanger sequencing. Results A novel homozygous missense variant (c.1339G > A, p.Gly447Arg) in the ATP8A2 gene was identified and completely segregated with the phenotype in the family. In silico analysis and structural modeling revealed that the p.G477R substitution is deleterious and induced undesired effects on the protein stability and residue distribution in the ligand‐binding pocket. The novel sequence variant occurred within an extremely conserved subregion of the ATP‐binding domain. Conclusion Our findings expand the spectrum of ATP8A2 mutations and confirm the reported genotype‐phenotype correlation. These results could improve genetic counseling and prenatal diagnosis in families with clinical presentations related to CAMRQ4 syndrome., A novel mutation (c.1339G>A; p.G447R) in ATP8A2 gene is responsible for cerebellar ataxia, mental retardation, and dysequilibrium syndrome type 4 (CAMRQ4) syndrome. WES could be a diagnostic approach in rare diseases with heterogeneous clinical presentations like CAMRQ4.

Factors influencing the choice of the subject could strengthen the knowledge and motivation to enter the field to promote the health of society. This study explores factors influencing specialty choice of Ahvaz Jundishapur University of Medical Sciences pediatric department residents In this cross-sectional study all 38 pediatric residents were was undertaken using a questionnaire designed to assess their reasons with regard to pediatric course choice. The nameless questionnaire with sixteen questions was completed separately. The reliability and validity of the questionnaires were verified by experts and Cronbach's alpha 0.810. From 38 pediatric residents, including 26 female (71%) and 12 male (29%), twenty six (70.3%) were married. Mean age was 33.1±4.2 years. participating in the study mentioned factors such as "helping patients", "raising knowledge and skills" and "the ability to do a better service" with the average of 4.32, 4.26 and 4.18 respectively as high priority; and factors such as "lack of heavy duty," "comfortable and effortless" and "lack of urgency", respectively, with an average of 2.18, 2.24 and 2.37 had a low priority in choosing pediatric specialty course. According to the t-test, statistically significant differences were observed between gender, marital status and factors influence specialty choice of pediatric course (P

The common diagnosis of loss of consciousness and clonic movements in children is seizure or epilepsy, but in a number of patients these symptoms could also be due to syncope. Over interpreted electroencephalography is misleading in a number of patients; therefore, in addition to a detailed and thorough history, a reliable test is needed to differentiate between these conditions. The aim of the study was to evaluate the utility of the head upright tilt test to differentiate between seizure-like events and syncope in children. A chart review descriptive study was conducted in a tertiary medical center in Ahvaz, Iran. We selected sixteen children (nine boys and seven girls) with convulsions of any type who were first diagnosed as epileptic based on the event description by their parents or caregivers to undergo the head upright tilt test. The main findings were the reproduction of previously presyncopal or syncopal symptoms in the tilted position. Fourteen children showed positive results after conducting the head upright tilt test, and their heart rates were significantly decreased compared to baseline at the onset of the syncopal or presyncopal manifestations. Systolic and diastolic blood pressures were significantly reduced in patients with positive results. In three children who initially had negative head upright tilt tests, intravenous isoproterenol was administered, and all three showed presyncopal and syncopal symptoms. The results indicate that the head upright tilt test could differentiate presyncopal and syncopal events in children who present with seizure-like movements but their history has clues for conclusive syncope.

More than 25% of seizures in children are refractory to anti-seizure medications and need other modalities to control. Recently, herbal agents with anticonvulsive effects on seizure models in animals have received significant attention, but studies that show their impact on seizures in children are scarce. In a randomized, double-blind placebo-controlled cross-over design, we tried to demonstrate the efficacy of a mixture of Nigella sativa and Thymus vulgaris extracts (Epistop) on seizures in a group of children with refractory epilepsies. Twenty-two children with refractory epilepsies were randomly assigned into two sequences to receive Epistop or placebo in a cross-over design. Neither Epistop nor placebo had effects on the seizure frequency and duration over the study period. Only four children experienced clinically substantial seizure frequency (more than 50%) reduction after Epistop administration. These results indicate that a mixture of Nigella sativa and Thymus vulgaris extracts does not affect the seizure frequency and duration in children with refractory seizures. However, a minority of highly selected children with refractory seizures might benefit from the effects of a mixture of these agents’ extracts.

Momen AA, Shakurnia A, Momen M. Eleven-year surveillance of acute flaccid paralysis in southwestern Iran. Turk J Pediatr 2019; 61: 544-551. The purpose of the study was to report the results of acute flaccid paralysis (AFP) surveillance in the southwestern Iran in the 11 years' period. This is a retrospective descriptive study based on WHO guidelines. The collected data includes all pediatric AFP cases reported between of January 2006 to December 2016 by all health centers of Khuzestan province in Southwest of Iran. During an eleven-year period, 274 cases of AFP were reported in Khuzestan Province. Among them, 56.9% were younger than five years of age, and 55.9% were male. None of these cases was confirmed as poliomyelitis. The annual incidence of non-polio AFP ranged from 1.46/100,000 to 3.11/100,000 (Mean: 2.04 cases/100,000). The incidence rate of AFP in children under five years of age was significantly higher compared to older children (p=0.001), and the most common cause of paralysis (74.5%) was Guillain-Barre syndrome. All performance indicators met the WHO-specified targets. The follow-up of patients with AFP is essential to the eradication of poliomyelitis.

Introduction: Seizures are the most frequent neurologic disorder seen in childhood. Epilepsy is a group of disorders that includes an abnormally increased susceptibility to seizures. Aim: To examine the effectiveness of SPECT (Single Photon Emission Computerized Tomography) in detecting seizure foci in 21 Iranian children who had medically refractory epilepsy. Materials and Methods: Children between 2 to 15 years of age with uncontrolled seizures were investigated using SPECT scan as a standardized protocol. Results: In 16 cases (76.2%), likely seizure foci were evident, as were seen in the form of decreased regional blood flow, while in 5 cases (23.8%), SPECT scan results were normal. Left temporal lobe was the most common area which had decreased regional blood flow. Conclusion: SPECT scan can potentially be used to investigate children with uncontrolled seizures.

Background: Guillain-Barré syndrome (GBS) is an acute inflammatory polyneuropathy, which has become the most common cause of acute flaccid paralysis. An accurate estimation of GBS occurrence would be useful for investigating the potential causal relationships between risk factors and GBS. The aim of the study was to analyze the incidence, annual time trend, and some epidemiological aspects of GBS in children in the Southwest of Iran. Methods: This was a retrospective study conducted by the Department of Pediatrics of Ahvaz Jundishapur University of Medical Sciences from January 2006 to December 2015. We extracted data from the national database of Acute Flaccid Paralysis Surveillance System. Results: A total of 184 subjects with GBS were assessed. The mean age of subjects was 5.43 ± 4.07 years. The average annual incidence rate of GBS was 1.51 per 100,000 children under 15 years old (95% CI: 1.29-1.73). There was no significant statistical difference in GBS incidence rate between girls and boys (p = 0.376). The highest and lowest proportions of the GBS occurrences were in autumn (32.2%) and summer (14.7%), respectively. Conclusions: The findings indicated that the annual incidence rate of GBS in this study was similar to those in other studies in this area.

OBJECTIVES: Investigations into the epidemiology of acute flaccid paralysis (AFP) are an essential strategic component of the Global Poliomyelitis Eradication Initiative of the World Health Organization (WHO), and are part of the certification process for polio eradication worldwide. This is an epidemiological report of AFP incidence in children less than 15 years old in southwest Iran. METHODS: This was a retrospective cohort study, carried out based on WHO guidelines, in which we reviewed non-polio AFP cases recorded from January 2006 to December 2010 in different regions of Khuzestan Province, southwest Iran. In this study, the records of all children under 15 years old with AFP were evaluated. RESULTS: During a 5-year period, 137 cases of AFP were reported (incidence rate, 2.21 per 100,000 children

The aim of this study was to evaluate the efficacy and safety of intramuscular midazolam in controlling convulsive status epilepticus in children, by comparing it with rectal diazepam.In this randomized trial, 100 children (50 in each group) with convulsive status epilepticus aged 1 month to 16 years were enrolled and randomly assigned into two groups to receive either 0.3 mg/kg intramuscular midazolam or 0.5 mg/kg rectal diazepam. Main outcome measure was stopping of all motor activity after drug administration. Another measures were times between patient's arrival to emergency department till drug administration, between drug administration to seizure cessation, and between patient's arrival to seizure cessation.Both medication were effective for seizure control and no significant difference was found between successful treatments after administering the medication (P = 0.061). In the midazolam group, in 96% (48/50) of cases treatment was successful and in the diazepam group, in 94% (47/50) of cases treatment was successful. Time from arrival to administering the medication was significantly shorter in midazolam group (P = 0.017). The majority of seizures in midazolam group were stopped in less than 66 s (median) compared to 130 s (median) for diazepam group, (P 0.001). No serious adverse effects were seen in both groups.IM midazolam is not superior but may be at least as effective as rectal diazepam for controlling of status epilepticus in children. Midazolam via IM route could be one of the choices in children with convulsive status seizures who have difficult IV access.

Introduction: Headache is a common complaint in children, and it has a significant effect on their daily living activities. Having a definite diagnosis for the cause of the headache is very important for its appropriate management. Case Presentation: We report a 12-year-old boy complaining of chronic headache, with a positive family history of migraine. The only abnormal finding in his general and neurological examinations, and CT scan evaluation of his brain and paranasal sinuses, was a retention cyst in his sphenoid sinus. The child’s symptoms did not improve after receiving migraine medications or treatment for sinusitis. Further investigations revealed positive serological results for brucellosis infection. His symptoms were relieved, and imaging evidence of sinus involvement disappeared after brucellosis treatment. The child remained headache free in later follow-ups. Discussion: In evaluating the cause of children’s headaches, brucellosis must be considered in differential diagnosis.

Background. Developmental disorders are failure or inability to acquire various age-specific skills at expected maturational age, which affects about 5–10% of preschool children. One of the most important methods for evaluation of developmentally delayed children is neuroimaging, especially, brain magnetic resonance imaging (MRI) that provides useful information regarding brain tissue structures and anomalies.Method and Material. In this study, hospital records of 580 developmentally delayed children (aged 2 months to 15 years) who admitted in pediatric ward of Golestan Hospital from 1997 to 2009 were selected. Information such as age, MRI findings were collected in the questionnaire and statistically analyzed.Results. Total, 580 children including 333 males (57.4%) and 247 females (42.6%) were studied. Abnormal brain MRI was observed in 340 (58.6%) cases (204 Males, 136 females). The finding includes nonspecific in 38 (6.6%), congenital and developmental anomalies of brain in 39 (6.7%), recognizable syndromes in 3 (0.5%), neurovascular diseases or trauma in 218 (37.6%), and metabolic or neurodegenerative diseases in 42 (7.2%) cases.Conclusion. Because 60% of all study groups showed abnormal brain MRI, using this method could be effective in diagnosis, management, and almost prognosis determination processes.

Aim: We aimed to determine the relative frequency of febrile convulsion in children with major thalassemia to theorize that higher serum iron levels could reduce the incidence of febrile convulsion. Background: Febrile convulsion is the most common type of seizure in childhood that its causes are not fully understood. However, some risk factors have been cited such as the serum iron level. Materials and methods: Three hundred and fifty-nine children aged more than 5 years with major thalassemia who were receiving blood were enrolled as the case group. The control group consisted of 357 children without thalassemia aged 4–7 years (151 boys, 206 girls) who were referred to healthcare centers for routine health monitoring. Included data were the history of febrile convulsion, age of onset and type and the frequency of convulsions. Results: Children in control group significantly experienced more febrile convulsions than thalassemic children [4/359 (1.1%) in the thalassemic children and 14/357 (3.9%) in the control group had experienced febrile convulsions ( P = 0.017)]. Conclusion: The frequency of febrile convulsion in children with major thalassemia is less than that of normal children. Children with thalassemia major may have higher serum levels of iron and such high serum iron levels might have a protective role in the children who have a vulnerability for febrile convulsions.

Objective Approximately 5–10% of preschool age children are considered developmentally disabled. Brain Magnetic Resonance Imaging (MRI) plays a key role in the diagnostic evaluation in these children. Many congenital or acquired brain anomalies are revealed with MRIs. Although the majority of these abnormalities are sporadic but patients with subcortical band heterotopia or double cortex syndrome have sex-linked inheritance. We are going to present the first case in Iran from Ahvaz city, which was presented with status epilepticus associated with developmental delay and finally diagnosed as double cortex syndrome, because band heterotopia cases especially for continuous or generalized form is rare. A 4.5-year-old developmentally delayed girl was admitted for generalized tonic clonic seizure attack of 1 hr, upward gaze, locked mouth, and urinary incontinence (status epilepticus) in the child neurology ward. She had a history of recurrent seizures that started as febrile seizures since she was 12 months of age and had frequent admissions for having recurrent seizure attacks. She was the only child of consanguineous parents with negative family history of any neurologic problems. She was a product of uneventful term pregnancy, vaginal delivery with a low Apgar score at birth who was admitted for six days in the neonatal ward for hypotonia and cyanosis. At 4.5 years of age, she had HC: 45cm (