Iranian Journal of Child Neurology (IJCN)
Volume:10 Issue: 4, Autumn 2016

Autism is a complex behaviorally defined disorder. There is a relationship between zinc (Zn) levels in autistic patients and development of pathogenesis, but the conclusion is not permanent.
The present study conducted to estimate this probability using meta-analysis method. In this study, Fixed Effect Model, twelve articles published from 1978 to 2012 were selected by searching Google scholar, PubMed, ISI Web of Science, and Scopus and information were analyzed. I² statistics were calculated to examine heterogeneity. The information was analyzed using R and STATA Ver. 12.2.
There was no significant statistical difference between hair, nail, and teeth Zn levels between controls and autistic patients: -0.471 [95% confidence interval (95% CI): -1.172 to 0.231]. There was significant statistical difference between plasma Zn concentration and autistic patients besides healthy controls: -0.253 (95% CI: 0.498 to -0.007). Using a Random Effect Model, the overall Integration of data from the two groups was -0.414 (95% CI: -0.878 to -0.051).
Based on sensitivity analysis, zinc supplements can be used for the nutritional therapy for autistic patients.

Dyslexia is a neurological dysfunction (also known as a learning disability) that characterized by disability in reading in spite of normal intelligence. Bothe genetic and environmental risk factors are contributing into the condition.
Diagnosis of dyslexia is based on examination and investigation of the patient’s memorial, spelling, visual, and reading skills. It is the most common learning disability, affecting 3%–10% of the school population. Modern neuroimaging techniques such as functional magnetic resonance imaging (fMRI) have shown a correlation between both functional and structural differences in the brains of children with reading difficulties. Hence, to address this issue, the auditory brainstem responses of children with dyslexia were investigated.
Fifty two children with dyslexia (30 males, 22 females) were selected after examination by speech therapist. In addition, fifty two control children were included as well. The IPSI and contralateral ABR tests were conducted on both cases and controls. Click stimuli were used at 75 nHL intensity. The study focused on absolute latency of wave V in both groups.
Absolute latency of wave V in contralateral showed differences between children with dyslexia and control group, but no significant results were found in IPSI testing.
The current data provide an evidence for brainstem and its function in signal processing and the role of brainstem nucleus on processing and delivering the information to each hemisphere.

Developmental care comprises a wide range of medical and nursing interventions used in the neonatal intensive care unit (NICU) to mitigate and reduce stressors affecting preterm or ill neonates. Because patient satisfaction survey is a valuable quality improvement tool, we aimed to develop and test the psychometric properties of a tool for measuring parent satisfaction of developmental care in the NICU.
In this psychometric methodological study, the item pool and initial questionnaire were designed based on a comprehensive literature review and exploring NICU parent satisfaction questionnaires. The validity of the designed questionnaire was determined using face, content (qualitative and quantitative), and construct validity. Exploratory factor analysis was performed using responses from 400 parents of infants hospitalized in the NICUs of 34 hospitals in 2015 in Tehran, Iran. The reliability of the questionnaire was identified using Cronbach’s alpha and stability measures.
The initial questionnaire was designed with 72 items in five domains. After testing the face validity, 3 items were omitted. The results of validity testing were acceptable. The exploratory factor analysis was performed on 69 items, and 5 factors (care and treatment with 20 items, information with 15 items, hospital facilities with 9 items, parental education with 7 items, and parental participation with 8 items) were extracted. The reliability was supported by high internal consistency (α = 0.92).
This questionnaire could be valid and reliable tool for measuring parents’ satisfaction.

Electroencephalography (EEG) is the most effective diagnostic tool in distinguishing epileptic seizure. Chloral hydrate (CH) is a sedative hypnotic drug, commonly used as a method of sedation in children agedThis retrospective cohort study was conducted on patients’ records of aged 15-72 months undergone an EEG and required sedation. Overall, 200 children were assessed including 100 patients in group 1 (CH) and 100 patients in group 2 (DH). Data were gathered by a form including age, sex, the cause of EEG, complication, success rate, first dose success, as well as sleep and awake latency.
Data were reported by descriptive statistics (mean, standard deviation, number, and percent) and analyzed by t-test and chi-square using SPSS 19.
Totally, 113(56%) male patients with the mean age of 35.62±14.00 months participated in this study. Vomiting and agitation were the most frequent complications in CH and DH groups, respectively. Most of patients in both group indicated successful sedation. CH indicated higher rate of success by first dose toward DH. In addition, CH mentioned lower sleep latency and significant difference was noted between groups. The mean duration of awake latency was higher in DH groups which showed significant difference.
CH might be a more effective drug in comparison with the DH for sedation. According to the availability and low cost of DH, investigators are advised to perform further investigations.

To describe 12 yr experience of molecular genetic diagnosis of Spinal Muscular Atrophy (SMA) in 460 cases of Turkish patients.
A retrospective analysis was performed on data from 460 cases, referred to Medical Genetics Laboratory, Ege University’s Hospital, Izmir, Turkey, prediagnosed as SMA or with family history of SMA between 2003 and 2014.
The PCR-restriction fragment length polymorphism (RFLP) and the Multiplex ligation–dependent probe amplification (MLPA) analysis were performed to detect the survival motor neuron (SMN)1 deletions and to estimate SMN1 and SMN2 gene copy numbers.
Using PCR-RFLP test, 159 of 324 postnatal and 18 of 77 prenatal cases were detected to have SMN1 deletions. From positive samples, 88.13% had a homozygous deletion in both exon 7 and exon 8 of SMN1. Using MLPA, 54.5% of families revealed heterozygous deletions of SMN1, and 2 or 3 copies of SMN2, suggesting a healthy SMA carrier. Among patients referred for SMA testing, the annual percentage of patients diagnosed as SMA has decreased gradually from 90.62% (2003) down to 20.83% (2014).
Although PCR-RFLP method is a reliable test for SMA screening, MLPA is a necessary additional test and provide relevant data for genetic counseling of families having previously affected child. The gradual decrease in the percentage of patients molecularly diagnosed as SMA shows that clinicians have begun to use genetic tests in the differential diagnosis of muscular atrophies. Cost and availability of these genetic tests has greatly attributed to their use.

Given that several factors involved in the incidence or exacerbation of developmental disorders in children, the present study aimed to investigate the relationship between some of the risk factors affecting mothers’ health and development in children using path analysis.
The present cross-sectional analytical study was conducted on 750 mothers and their children in health centers in Tehran, Iran in 2014 enrolled through multi-stage random sampling. Data were collected using a demographic and personal information questionnaire, the Perceived Stress Scale, Beck’s depression Inventory, Spielberger’ anxiety inventory, the WHO domestic violence questionnaire and an ages & stages questionnaire for assessing children’s development. Data were analyzed using SPSS.19 (Chicago, IL, USA) and Lisrel 8.8.
Developmental delay was observed in 12.1% of the children. The mean stress score was 23.94±8.62 in the mothers, 50.7% of whom showed mild to severe depression, 84.2% moderate to severe anxiety and 35.3% had been subjected to domestic violence. The path analysis showed that children’s development was affected directly by perceived stress (β=-0.09) and depression (β=-0.17) and indirectly by domestic violence (β=-0.05278) and anxiety (β=-0.0357). Of all the variables examined, depression had the biggest influence on development in the children (β=-0.17). The proposed model showed a good fit (GFI=1, RMSEA=0.034).
Children’s development was influenced indirectly by domestic violence and anxiety and directly by perceived stress and depression in mothers. It is thus suggested that more concern and attention be paid to women’s mental health and the domestic violence they experience.

Nowadays, the evaluation of all aspects of infant development is important. However, in practice, some of these assessments, especially those requiring more manipulation on high-risk infants, may impose additional stress on them.
Therefore, sometimes it is essential to utilize the results of a developmental assessment for the prediction of some other aspects of development. This study evaluated the relationship between the scores of the behavioral tests and the motor function test.
This cross-sectional study and was undertaken in the Neonatal Intensive Care Center and Clinic of Shahid Akbar Abadi Hospital, Tehran, Iran. A group of 50 infants with low birth weights was selected based on the easy non-contingency method and the inclusion criteria, and served as the participants. In order to assess the motor function and the behavioral performance, the motor function test (a test of infant motor performance (TIMP)) and the neonatal behavioral assessment scale (neonatal behavioral assessment scale (NBAS)) were used respectively. TIMP has both stimulation and observation sections. The items include habituation, social interaction, motor system, state organization, state regulation, autonomic system, smile, supplementary items, and the reflex.
No significant association was found between the items of the habituation of behavioral testing and the observation of the movement test. There was no statistically significant relationship between the habituation and stimulation sections as well as between the system autonomous of the behavioral test and the observation section of the motor test (P>0.05). The relationship between other variables was statistically significant (PThe scores of some behavioral performance items could be a good predictor of the scores of the motor function items for low birth weight infants in the neonatal period.

Febrile seizure is one of the most prevalent childhood convulsions with the most common age of onset at 14-18 mo old. Fever decreases the brain threshold for seizure. Attention Deficit Hyperactivity Disorder (ADHD) is also a neurologic-behavioral problem defined by attention deficit and hyperactivity according to DSM-IV criteria in which the child must have these signs in two different environments. There is controversy on the possible relation between febrile seizure and ADHD; while some studies approve a strong relation, some exclude any relation and some attribute ADHD to the side effects of other reasons.
This descriptive-analytic study enrolled all children of 3-12 yr old with febrile seizure (according to Nelson Pediatrics Textbook diagnosed by the pediatrician in charge) referring to Amir Kabir Hospital, Arak, central Iran in 2010-2011.
Overall, 103 of them with no corporeal or psychological disorder (like depression, anxiety, schizophrenia and other CNS maternal disease) were compared to 103 children of the same age and gender admitted due to disease other than febrile seizure utilizing DSM IV criteria for ADHD. Data were analyzed using SPSS version 18.
The hyperactivity disorder in the control and case group was 34.3% and 16.7%, respectively, denoted a significant relation between simple febrile seizure and hyperactivity.
Hyperactivity has a significant relation with febrile seizure in male gender, making further investigation in these children prudent for early diagnosis and management.

Chitotriosidase (CT) activity is a useful biomarker for diagnosis and monitoring of Gaucher disease (GD). Its application is limited by some variants in the CT gene. Two main polymorphisms are 24 bp duplication and G102S led to reduce CT activity. The aim of this study was to determine these variants influencing on plasma CT activity.
Blood samples were collected from 33 patients with GD, 15 sibling carriers and 105 healthy individuals serving as controls. CT activity was measured using 4-methylumbelliferyl-β-D-N,N′,N″triacetylchitotrioside substrate in plasma samples. The CT genotypes of 24 bp duplication and G102S variants were determined using PCR and PCR-RFLP.
Untreated GD patients had a significantly higher CT activity compared to treated patients (P = 0.021). In addition, chitotriosidase activity in carriers was higher rather than controls. Allele frequencies of 24 bp duplication in GD patients, sibling carriers and controls were 0.21, 0.266 and 0.29 and for G102S were 0.318, 0.366 and 0.219, respectively. Different G102S genotypes had not significant effect on CT activity. Chitotriosidase activity has a positive correlation with age in normal group, carriers, and negative correlation with hemoglobin in GD patients. Using cut-off level of 80.75 nmol/ml/h, sensitivity and specificity of CT activity were 93.9% and 100%, respectively.
Chitotriosidase activity is a suitable biomarker for diagnosis and monitoring of GD. Determination of 24 bp duplication is helpful for more accurate monitoring the GD patient’s therapy. However, it seems that, specifying of the G102S polymorphism is not required for Iranian GD patients.

Some reports have shown the association between Moyamoya syndrome and autoimmune diseases. Herewith, we present a 3.5 yr old girl with Henoch- Schönleinpurpura (HSP) who was treated with steroids because of sever colicky abdominal pain. However, central nervous system manifestations such as headache, ataxia and vision impairment developed during 6 months of her outpatient follow-up. More evaluation using MRA revealed intracranial stenosis of internal carotid artery and arterial collaterals that were in favor of Moyamoya syndrome. To our knowledge, this is the first report of Moyamoya syndrome following henoch-schönleinpurpura.

The basal ganglia are a group of structures that act as a cohesive functional unit. They are situated at the base of the forebrain and are strongly connected with the cerebral cortex and thalamus. Some speech disorders such as stuttering can resulted from disturbances in the circuits between the basal ganglia and the language motor area of the cerebral cortex. Stuttering consists of blocks, repetitive, prolongation or cessation of speech. We present a 7.5 -year-old male child with bilateral basal ganglia lesion in globus pallidus with unclear reason.
The most obvious speech disorders in patient was stuttering, but also problems in swallowing, monotone voice, vocal tremor, hypersensitivity of gag reflex and laryngeal dystonia were seen. He has failed to respond to drug treatment, so he went on rehabilitation therapy when his problem progressed.
In this survey, we investigate the possible causes of this type of childhood neurogenic stuttering.

We present a 7 year old boy afflicted with super-refractory seizure that responded poorly to antiepileptic drugs and sustained a long course of hospitalization and complications of high doses of medications as well as longstanding stay in hospital. The differential diagnoses were, fever-induced refractory epileptic encephalopathy (FIRES), and infectious and autoimmune encephalitis.
However, work-ups had not revealed any evidence of any specific diagnosis, so we assumed that he was afflicted by viral infectious encephalitis as he had, fever, vomiting, and prodromal symptoms of infectious (most probably viral) disease prior to onset of the seizure attacks.