Iranian Journal of Child Neurology

Epilepsy, in children, is a common neurological problem which causes referral to child neurology clinics.

The prevalence of nonfebrile seizure in children (under 10 years old),is estimated from 5.2 to 8.1 per 1000. Also, the prevalence of epilepsy in Iran estimated about 5 %; it means 4 million people of Iranian population live with epilepsy in Iran.

Although antiseizure drugs (ASDs) are the essential treatment modalities in most children, more than 30% of epileptic children have intractable seizures or they suffer from drug adverse effects secondary to these medications.

Because only a limited number of epileptic patients benefit from surgical therapy using the additional therapeutic options is inevitable.

There are many available nonpharmacologic proven therapies for refractory seizures that Dietary therapy ( Ketogenic Diet) is one of the important therapeutic options in this group.

In this review, we will discuss the different features of pediatric epilepsy dietary therapies (Especially the Ketogenic Diet) in Iran and also the history of epilepsy in ancient Iran, utilization, effectiveness, side effects, tolerability, and acceptability as well as ongoing and future programs.

Abstract

Objectives

Rett syndrome is an X linked dominant neurodevelopmental disorder which almost exclusively affects females. The syndrome is usually caused by mutations in MECP2 gene, which is a nuclear protein that selectively binds CpG dinucleotides in the genome.

Materials & MethodsTo provide further insights into the distribution of mutations in MECP2 gene, we investigated 24 females with clinical characters of Rett syndrome referred to Alzahra University Hospital in Isfahan, Iran during 2015-2017. We sequenced the entire MECP2 coding region and splice sites for detection of point mutations in this gene. Freely available programs including JALVIEW, SIFT, and PolyPhen were used to find out the damaging effects of unknown mutations.ResultsDirect sequencing revealed MECP2 mutations in 13 of the 24 patients. We identified in 13 patients, 10 different mutations in MECP2 gene. Three of these mutations have not been reported elsewhere and are most likely pathogenic.ConclusionDefects in MECP2 gene play an important role in pathogenesis of Rett syndrome. Mutations in MECP2 gene can be found in the majority of Iranian RTT patients. We failed to identify mutations in MECP2 gene in 46% of our patients. For these patients, further molecular analysis might be necessary.

Objective

The aim of the present study is to explain the correlation between sleep disorders and function in children with spastic cerebral palsy (4-12Y).

Materials & Methods

This cross-sectional study was carried out on 62 children with spastic CP (8.98±1.46Y) who were recruited from rehabilitation clinics of Tehran, Iran 2017. The Activities Scale for Kids, The Sleep Disturbance Scale for Children and the cerebral palsy Quality Of Life questionnaire for Children were utilized in this study. Data were analyzed by SPSS software (version 16)

Results

The results showed that children with sleep disorder and arousal disorders have lower family health, lower quality of life and lower level of independence in their activities (0.05).

Conclusion

These results emphasize on necessity of more attention about sleep disorders and family health problems in children with cerebral palsy.

Objectives

Anorexia Nervosa (AN) as a mental condition is a common eating disorder among young women. This study aims to shed lights on molecular behavior of this serious disorder in terms of protein interacting profile to provide further insight about its complexity.

Materials & Methods

The AN related genes were extracted from STRING database and included in interactome via Cytoscape software. The central nodes of the network were enriched via gene ontology (GO) by ClueGO+CluePedia and the action relationship between the nodes were determined by CluePedia.

Results

Six genes including LEP, INS, POMC, GCG, SST, and ALB were introduced as hub-bottlenecks that among them LEP, INS, and POMC were the super hub-bottlenecks based on further analysis. Action map analysis showed prominent role of hubs relative to bottlenecks in the network. Regulation of behavior, regulation of carbohydrate biosynthetic process, and regulation of appetite are the top associated processes for the identified hub genes.

Conclusion

The topological analysis proposed the five hub-bottlenecks as the most central genes in the network, these genes and their contributing biological terms may suggest additional importance in AN pathogenesis and thereby possible candidates for therapeutic usage.  However, further studies is required to justify these findings.  

Objective: we aimed to estimate possible relationship between vitamin D deficiency and gross motor developmental milestones in Iranian children.

Material and Methods: In this cross sectional study, 186 one-year old healthy children referring to one children hospital in Tehran were studied. The gross motor developmental milestone of children and their serum vitamin D concentration were evaluated.

Results: A total of 186 children, males 92 (49.5%) were studied. 24 children (12.9%) could sit, 40 (21.5%) could stand and 122 (65.6%) could walk. Vitamin D was sufficient in 148 children (79.6%), insufficient in 32 (17.2%) and deficient in 6 (3.2%).  Sufficient vitamin D was significantly correlated with walking ability (p<0.001, OR=3.9, 95%CI=1.9-8.4). 

Conclusion: Considering the significant correlation between vitamin D deficiency and gross motor developmental milestones in this population of children, it can be recommended that children referring with gross motor developmental delay need to be thoroughly evaluated for vitamin D deficiency. 

Abstract

Objectives

This applied research is the first practical study of teaching English as a foreign language (EFL) to students with autism spectrum disorder (ASD) in Iran. We examined the effect of a well-designed foreign language learning setting in facilitation of social skills and willingness to communicate in children with ASD.

Materials & Methods

A mixed-method research design was used. Using stratified sampling, a limited sample of 18 students were chosen from Kerman Province, southeastern Iran in 2014 categorized in three levels of ASD for each group of experimental and control; matched pairs were used to ensure homogeneity of participants in two groups. Each participant received 15 sessions with totaling 67 h of language learning. First 10 sessions were in the form of tutorials and the last 5 sessions were held in the form of paired classes with a peer. Before and after the sessions, caregivers and parents completed a questionnaire on students' social skills; the English instructor also rated participants' willingness to communicate.

Results

Teaching a foreign language had a positive main effect on social skills from caregivers’ and parents’ view compared to those of controls, significantly (P<0.05). From the instructor's view, there was additionally a significant improvement in the students with ASD’s willingness to communicate in classroom settings compared to the control group (P<0.05).

Conclusion

Optimum foreign language pedagogy for students with ASD is applied as an effective context enhancing children’s capabilities in social skills and willingness to communicate, provoked through a motivational foreign setting modulation in a novel environment. Suggestions on enhancing joint attention during the curriculum are provided.

 

Abstract

Objectives

Neurofeedback is a noninvasive treatment that changes brain activity in children with attention-deficit/hyperactivity disorder and thereby improves performance in these children. We examined the effect of neurofeedback on perceptual organization, visual and auditory memory in children with attention-deficit/hyperactivity disorder.

Materials & Methods

This study was quasi-experimental with pre-test, post-test design, and control group. The sample included 20 children with attention-deficit/hyperactivity disorder were selected through convenience sampling in Khorramabad, central Iran in 2017. The sample was divided into control and experimental groups. Pre-test included Rey-Osterrieth complex figure and Wechsler digit span. Rey-Osterrieth complex figure test was used to measure perceptual organization and visual memory. Wechsler digit span was used to measure auditory memory. After conducting pre-test, the experimental group participated in neurofeedback training sessions. Theta/Beta protocol was applied for all participants. The control group did not receive any intervention. Then post-test was conducted on two groups.

 Results

Neurofeedback training significantly improved visual memory (P<0.001) but neurofeedback training had no significant effect on the perceptual organization (P>0.05). Moreover, neurofeedback training enhanced auditory short-term memory and auditory working memory (P<0.05).

Conclusion

Neurofeedback improved neurocognitive abilities in children with attention-deficit/hyperactivity disorder.

Abstract

Objectives

Participation is a complex and context-dependent concept, which several factors can influence it. The aim of this study was assessing the relationship between the upper extremity function level of children with cerebral palsy (all type of cerebral palsy and severity) and their participation in different life areas.

Materials & Methods

This cross-sectional study assessed the relationship between the level of upper extremity function of cerebral palsy children and their participation in different life areas. Participants were 274 parents of children with cerebral palsy of the schools of children with pecial needs and occupational therapy clinics in Tehran, Iran in 2018.

They completed the Manual Ability Classification System (MACS) to determine the level of upper extremity function of children with cerebral palsy and Children Participation Assessment Scale-Parent version (CPAS-P) (to determine the participation level of children with cerebral palsy) questionnaires separately.

Results

The mean age of children was 8 yr and 8 months old (at least 6 yr and maximum 12 yr). The correlation between the level of upper extremity function and the overall score of each dimension of participation is significant (P<0.05) and moderate.

Conclusion

The upper extremity function of children with cerebral palsy has a moderate and significant relationship with the participation of children with cerebral palsy in different life areas and withdifferent dimensions of participation especially parental satisfaction dimension. Therefore, there is a correlation between upper extremity function and participation in occupations, but this relationship is moderate and is not very strong.

Objective: Febrile seizure is the most common seizure disorder in childhood and a common cause of hospitalization in children’s hospitals. The aim of this study was to investigate the prevalence of febrile seizures in children in Zahedan.

Materials and Methods: In this cross-sectional study, 600 children under 7 were examined for positive history of febrile seizure in 2014. The cluster sampling method was used, information was collected using a questionnaire, and data was expressed using descriptive- analytical tests.

Results: The mean age of the children was 2.7±1.8 years. The sample consisted of 290 (48.3%) boys and 310 (51.7%) girls. Of the 600 children studied, 21 experienced febrile seizure, the incidence of febrile seizures was 3.5%. No significant difference was observed in terms of age or gender (p>0.05). Among the patients with history of febrile seizure, 2 (9.5%) had a positive family history of seizure. The age at the first febrile seizure was under one year of age in 13 patients (61.9%) and over one year of age in 8 patients (38.1%).

Conclusion: Results  of present study indicated a moderate incidence of febrile seizure in the studied population. No significant difference was observed in terms of age or gender.

                                                                                         

ABSTRACT

Objectives:

The characteristics of paediatric neuromyelitis optica spectrum disorder (NMOSD) may indicate the degree of disability and identify factors that predict the response to treatment.

Materials & Methods

Among 114 NMOSD patients in an acquired demyelinating syndromes registry at the Sina Hospital, in Tehran, Iran, 10 paediatric NMOSD patients with longitudinal follow-up from 2005 to 2016 were retrospectively identified. The median time between disease onset and diagnosis was 18 months (range 1-108 months).

Results

All patients had a relapsing course, which resulted in disability in six with severe visual impairment and functional blindness in one and impaired ambulation in five patients during follow-up. Azathioprine (AZA) was first drug of choice for prophylaxis, but in five patients new attacks occurred and therapy was switched to rituximab (RTX) with no further relapses after median two years (range 1-3 y) follow-up.

Conclusion:

Paediatric onset of NMOSD was associated with severe attacks and poor response in 50 % of cases to AZA, RTX seemed to decrease the relapse rate.

Abstract

 

Mucopolysaccharidosis type III (MPS III; Sanflilippo syndrome) is a metabolic disorder characterized by a lysosomal enzyme deficiency in the catabolic pathway of heparan sulphate. The patients with mucopolysaccharidosis type III usually present with declined neurocognitive functions such as speech and hearing loss. Subtle somatic features of patients with mucopolysaccharidosis type III can lead to diagnostic delay and consequently a greater neurocognitive deterioration may happen. Herein, we report a 9-year-old boy who had developed normally until the age of 4 years when his symptoms initiated with behavioral disturbances such as auditory agnosia and decreased verbal communication. Progression of his symptoms to seizure and ataxia, brain perfusion scan and electroencephalography features strongly suggested landau-kleffner syndrome; however, results of gene sequencing analysis and high urinary glycosaminoglycan excretion confirmed mucopolysaccharidosis type III as his final diagnosis. This case strongly recommends screening for metabolic disorders such as mucopolysaccharidosis type III in the patients diagnosed as having landau-kleffner syndrome.

 

 

Abstract

 

Juvenile clinically amyopathic dermatomyositis (juvenile CADM) is a rare rheumatologic disease in children defined as presence of the hallmark cutaneous features of dermatomyositis in absence of muscle involvement. In this article we report an Iranian teenage girl presented to rheumatology clinic of Mofid Children's Hospital with cutaneous complaints who was diagnosed with juvenile CADM. Finally, we provide a literature review of previous studies on juvenile CADM.

Acute Spinal Subdural Hematoma (ASSH) is a rarely recognized condition that may result in severe irreversible neurologic complication. We presented a 7-year old girl was transferred to our hospital with limping and pain in lower extremities and acute paraplegia without history of direct trauma. The patient's lower limbs weakened. She was unable to bear weight. Deep Tendon Reflexes(DTR) in lower extremities had increased. Her MRI showed spinal subdural hematoma we reextended from T2 to T6. We performed laminectomy from T2 to T5 and about 70 cc of subdural hematoma was evacuated. After a month after the operation, the patient's neurological deficit resolved completely. The case illustrates the need of attention to clinical manifestation of acute spinal subdural hematoma and early diagnosis to prevent irreversible neurologic complication.