Iranian Journal of Child Neurology
Year:2016 | Volume:10 | Issue:2|Papers Count:13

Objective In healthcare system, it is necessary to have exact and accurate information in order to address health care needs and requirements of society members as well as expectations of policy makers, planners and decision makers. The aim of this narrative review article was to explain the role of information systems in cerebral palsy management and identify the advantages and barriers to the development of cerebral palsy registry system. Data were collected using databases such as of Science Direct, PubMed, Proquest, Springer, and SID (Scientific Information Database). Overall, 65 sources were selected. One of the biggest challenges for children with physical and motor disabilities in rehabilitation center is access to a system, which provides a comprehensive data set reflecting all information on a patient’s care. Thus, data and information management in children with physical and motor disability such as cerebral palsy facilitates access to data and cerebral palsy data comparison as well as the monitoring incidence rate of cerebral palsy, enhancing health care quality; however, there are always numerous barriers to establish the system. One of the ways to overcome these problems is the establishment of a standard framework of minimum data sets and exact definition of its data components. Reliable standards in the use of applications as well as user-friendly software will ensure patients’ data extraction and registration.

Objective Juvenile myoclonic epilepsy (JME) is supposedly the most frequent subtype of idiopathic generalized epilepsies (IGE). The aim of this study was to determine the prevalence of JME and comparison of patients’ demographics as well as timeline of the disease between positive family history epileptic patients (PFHE) and negative family history epileptic patients (NFHE) among sample of Iranian epileptic patients.Materials & Methods From Feb.2006 to Oct.2009, 1915 definite epileptic patients (873 females) referred to epilepsy clinics in Isfahan, central Iran, were surveyed and among them, 194 JME patients were diagnosed. JME was diagnosed by its specific clinical and EEG criteria. Patients were divided into two groups as PFHE and NFHE and data were compared between them.Results JME was responsible for 10% (194 patients) of all types of epilepsies. Of JME patients, 53% were female. In terms of family history of epilepsy, 40% were positive. No significant differences was found between PFHE and NFHE groups as for gender (P>0.05). Age of epilepsy onset was significantly earlier in PFHE patients (15 vs.22 yr, P<0.001). Occurrence of JME before 18 yr old among PFHE patients was significantly higher (OR=2.356, P=0.007). Conclusion A family history of epilepsy might be associated with an earlier age of onset in patients with JME.

Objective Many studies have supported the role of protein kinase C (PKC) inhibitors in the physiopathology and treatment of bipolar disorder in adults. Tamoxifen is one of the drugs with the effect of PKC inhibition. This study aimed to determine the effect of tamoxifen on the rate of improvement mania symptoms in the sample of children and adolescents with acute mania.Materials & Methods In this randomized, placebo-controlled clinical trial study, registered in www.irct.ir with the code of IRCT201410126418N3, overall 44 patients with bipolar disorder with acute manic episode were randomly assigned into treatment and control groups. The serum levels of lithium and tamoxifen among the participants in the treatment groups were 0.8 -1.1 mg and 20-40 mg per day respectively. Serum level of lithium among participants in the control group was similar. The main comparisons were made based on the Young Mania Rating Scale (YMRS) and Children Depression Inventory (CDI) scores of the participants at baseline and at the end of each study week. The pharmacological side effects of serum level of lithium were examined weekly. Analysis of Covariance (ANCOVA) test was used for the statistical analysis.Results There was no difference in the baseline score of YMRS and CDI in the treatment and control groups while a statistical significant difference (P<0.05) in these scores was found between and within the groups.Conclusion The addition of tamoxifen to lithium causes a significant difference in reducing the symptoms of mania and depression in the treatment group compared to the control group.

Objective In this study, different kinds of memory were evaluated using Rey Auditory Verbal Learning (RAVLT) test and were compared between two groups of typical and gifted students using Digit Span test. Finally, we determined if working memory interfered with scores in different Rey stages or not.Material & Methods This study was conducted in Tehran City, Iran in 2013. Scores on RAVLT were compared with WISC- R digit span results in a sample of 148 male students aged 12-14 yr old divided into two groups including 75 students in typical school (IQ ranging between 90 and 110) and 73 gifted students (IQs ranging between 110 and 130).Results Gifted students obtained higher scores than typical students in both Forward Digit Span (FDS) and Backward Digit Span (BDS) and all 9 stages of RAVLT comparing with typical students (P<0.001). There was no significant difference between different ages (P>0.05). The 14 yr old students in both groups had the highest score. There was a high correlation between FDS and the first stage of RAVLT as well as high correlation between BDS and seventh stage of RAVLT.Conclusion Intelligence has effect on better score of memory and gifted subjects had better scores in memory tests, although the intelligence effect in learning was quantitative rather than qualitative. RAVLT is a comprehensive test, which evaluates short-term memory, working memory and long-term memory and besides Digit span test provides precious information about memory and learning of subjects in order to program different student’s educational schedules.

Objective Guillain-Barre Syndrome (GBS) is an acute poly radiculoneuropathy characterized by progressive motor weakness of limbs and areflexia. In this study, our aim was to evaluate the clinical pattern and prognosis of children with Guillain-Barre syndrome.Materials& Methods This cross-sectional study was conducted in the Pediatric Neurology Unit of Amirkola Children’s Hospital, Babol, Iran during the period of 5 years from October 2008 to September 2013. We assessed the clinical features, results of electro diagnostic tests, functional status, treatment and outcome of 17 children diagnosed with GBS.Results Of 17 (male to female ratio=1.6: 1) children studied, all had motor weakness, 4 children (23.5 %) and cranial nerve palsies. Respiratory paralysis was found in one child requiring assisted ventilation. Antecedent illness preceding GBS was recorded in 7 (41.2%) children. The GBS subtype distribution as per electro diagnostic studies was as follows: acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 12 (70.6%) acute motor axonal neuropathy (AMAN) in 3 (17.6%), acute motor sensory axonal neuropathy (AMSAN) in 2 (11.8%). IVIG constituted the treatment given in all of the patients. Complete recovery was observed in 16 children and the remaining one child was dependent to wheelchair.Conclusion GBS in children is not poor prognostic disorder and our recommendation is administration of IVIG as soon as possible after clinical diagnosis. Except for one child who remained wheelchair bound, there was no mortality or morbidity in long-term observation. Besides, strong limitation of our study was the low number of subjects.

Objective The aim of this study was to translate and validate the psychometric properties of the Quality of Life Kindl questionnaire.Materials & Methods Parents of 4-7 yr-old healthy and ill children referred to Mofid Children Hospital in Tehran in 2013, Iran were sampled randomly in two groups each of which 130 people. After translation, the questionnaie’s validity and reliability was evaluated and was confirmed for face and content validity. Questionnaire was also completed by two (one healthy and one ill) groups for which inclusion criteria included consent of the parents, age of the children being beween 4 and 7 yr, and presence of the child in a nursery school, kindergarten, school or any class at least for one month. Exclusion criteria were inability of the parents in answering the questions accurately. Inclusion criterion for the ill group was having chronic cardiac, neurologic, hematologic, or respiratory diseases, lasting longer than 3 months for which they were followed up in outpatient clinic in the hospital. The reliability of questionnaire was measured by the Cronbach’s alpha.Data were analyzed using factor analysis, Spearman’s correlation coefficient, Mann-Whitney and Chi-square test.Results The reliability was 0.85 and 0.81 in healthy and ill groups, respectively. The results of factor analysis showed that each of eight subscales of questionnaire had acceptable construct validity. Only two of 52 questions of the questionnaire did not have proper correlation coefficient.Conclusion Quality of Life Kindl Questionnaire is a valid and reliable test for assessing healthy and ill children in Iran.

Objective Epilepsy is a chronic neurological disease. Evidence has indicated that epilepsy has an impact on mental and physical health of children. The present study aimed to determine the effectiveness of attribution retraining on health enhancement of epileptic children.Materials & Methods This was an experimental study with a pre-test and a post-test design with a control group. Thirty students with epilepsy (11 female and 19 male students) were selected in convenience from Iranian Epilepsy Association. They were assigned to experimental and control groups and their mothers completed Child Health Questionnaire (CHQ-PF.28) before and after the intervention.The experimental group attended to eleven sessions (each session 45 minutes, twice a week). Subjects were trained by attribution retraining program, but control group was not. Multivariate analysis of covariance (MANCOVA) was used for analyzing the data.Results Health (both psychosocial and physical) of experimental group enhanced significantly after the intervention sessions compared to control group.Conclusion Attribution retraining is an effective intervention to enhance the psychosocial and physical health of epileptic children.

Objective Reduction of pain of invasive procedures in neonates can prevent pain side effects. The purpose of present study was to evaluate the efficacy and safety of a single low dose of intravenous fentanyl in reducing of lumbar puncture (LP) pain in neonates.Materials & Methods In this randomized clinical trial, registered with code number of 2014022616761N150, admitted neonates to Shahid Sadoughi Hospital, Yazd, Iran from August-April 2012 (45 cases) were randomly assigned into two groups to receive 2 mg/kg of intravenous fentanyl or 0.2 milliliter of normal saline, two min before LP. Primary outcome was success rate in reducing of pain during needle insertion to skin (pain score of less than three). Secondary outcomes were clinical side effects and serious adverse events.Results Forty-five neonates including 23 girls and 22 boys were evaluated. Pain reduction was obtained in 39.1% (9 of 23 neonates) of fentanyl group and in 4.5% (one of 22 neonates) of control group. Means of pulse rate (136.41 ± 9.16 vs.148.9 ± 8.99) and pain score during needle insertion (3.41±1.31 vs.5.8±1.12) were lower in fentanyl group. No severe adverse effects were seen in both groups. Side effects such as vomiting [9% (N=2) in control and 4.3% (N=1) in fentanyle group] and mild transient decrease in oxygen saturation in 8.7% (N=2) of fentanyle group were seen. Safety in two groups was not statistically different.Conclusion Intravenous fentanyl might be considered as a safe and effective analgesic drug in LP in neonates.

Objective Premature birth is an important factor for mortality and morbidity of neonates.This study was designed to evaluate the outcome of preterm neonates who needed neonatal intensive care (NICU) hospitalization after 6 yr at their entrance to the school.Materials & Methods This cross sectional study was conducted on premature neonates consecutively hospitalized in NICU of Valie Asr Hospital (the Academic Pediatric Hospital, Zanjan, Northwestern Iran) from September 2001 to September 2003. All children with a history of prematurity and NICU treatment were evaluated at their entrance to the school. Demographic findings, clinical examinations, IQ test, hearing and visual acuity exams were recorded.Results From 179 neonates, 78 (43.6%) survived and were discharged from hospital.Fifty-four of them were available and entered first grade in primary school.Only one case had severe mental retardation. One case had severe retinopathy of prematurity (ROP). Hearing abnormality was not detected in any case. There was no significant relation between IQ score, visual as well as hearing findings and gestational age.Conclusion We did not find significant disability in the outcome of surviving infants. This could be explained by the high mortality rate of neonates during hospitalization.

Objective Metabolic disorders, which involve many different organs, can be ascribed to enzyme deficiency or dysfunction and manifest with a wide range of clinical symptoms. This study evaluated some of the demographic and clinical findings in pediatric patients affected by organic acidemia.Materials & Methods This cross-sectional study was part of a larger study conducted in patients with metabolic disorders during a period of 7 years from 2007 to 2014 in Isfahan Province, Iran. Our study covered a wide range of cases from newborn infants (one-week old) to adolescents (children up to the age of 17 years). This study evaluated patients’ demographic information, history of disease, developmental and educational status, clinical and general conditions. Phone and in-person interviews were used to gather information.Results Out of 5100 patients screened in this study, 392 patients were affected by one of the different metabolic disorders and 167 individuals were diagnosed as organic acidemia. Propionic acidemia/methyl malonic acidemia (PA/MMA) was the most prevalent form of this metabolic disorder. The frequency of consanguinity was 84.7% in the group of patients. The mortality rate was 18.8% in patients with organic academia.Conclusion Each of the metabolic diseases, as a separate entity, is rare, nevertheless, in aggregate they have a somewhat high overall prevalence. These diseases result in mental and developmental disorders in the absence of quick diagnosis and initiation of treatment. Furthermore, more mutations should be identified in societies affected by consanguinity. Further research should also be conducted to determine worthwhile and more-efficient screening methods as well as long term neurological prognosis.

Objective The Hereditary Sensory and Autonomic neuropathy (HSAN) is a rare group of neuropathies that affects the Sensory and Autonomic nervous system. The patients do not have the ability of sensing different sensations such as pain and temperature, which tends to lead to different injuries. In addition, due to autonomic involvement, the patients suffer from fluctuation in body temperature periodically and lack of precipitation. HSAN is divided into 5 types according to the age of onset, clinical features, and inheritance. Our case was a 9-yr old boy from cousin parents. He had some developmental delay and history of recurrent fever and convulsion in the first year of his life. Gradually, other symptoms added to patient’ feature such as multiple painless skin ulcers, tooth loss, destruction of toes and fingers. In electro diagnostic study, we found decreased amplitude of sensory nerves, while the other studies were normal. Laboratory test and imaging studies were also normal. All clinical and paraclinical findings were in favor of HSAN type IV. There is no cure for such patients, as a result, these patients and their families need receiving appropriate education and timely rehabilitation services.

Objective Angelman syndrome (AS) is a neurodevelopmental disorder presented by jerky movement, speech delay and cognitive disability epilepsy as well as dysmorphic features. It occurs due to an expression deletion in 15q11-q13 chromosome. In this article, we present an eight yr boy referred to Pediatrics Neurologic Clinic Mashhad, Iran for speech delay. He had abnormal behavior ataxia unusual laughing facial expression intellectual disability and mandibular prognathism.Metabolic screening tests and brain MRI were normal. Genetic analysis was pathognomonic for AS.

Objective Conversion disorder (CD) is a mental disorder in which patient displays neurological symptoms such as blindness, mutism, paralysis and seizure. It starts when our mind converts our mental stress into a physical symptom. A 15-year-old single white female with chronic cough, which had begun 5 months ago, was brought to our clinic. She had no history of hospitalization. His daily cough was without sputum production or fever, rhinorrhea and stopped during sleep. There was no recent exposure to tobacco smoke or a person with a chronic productive cough. Laboratory tests were normal. She had engaged 4 months ago.Doing sex during engagement is prohibited in her culture but and had anal sex, because of her spouse’s trend. Psychotherapy was done and complete recovery was accomplished.