Iranian Journal of Child Neurology
Year:2018 | Volume:12 | Issue:3|Papers Count:15

Objective: Primary immunodeficiencies (PID) are a heterogeneous group of disorders with a variable clinical spectrum of manifestations. The central nervous system may be involved in PID with symptoms which may present initially or develop at later stages. The purpose of this study was to review the neurological manifestations of different PID syndromes. Materials & Methods: We focused on 104 selected studies on PID with certain neurological abnormalities which may accompany these disorders or may later signify a PID in their course. Results: Diverse neurological deficits accompanying certain PIDs may be mild or they may greatly influence the course of the disease with major impacts on the quality of life of these patients. Conclusion: Early recognition and treatment is important to prevent or reduce future irreversible neurological sequelae. Therefore physicians should be aware of the neurological features accompanying PID.

Objectives This case-control study was carried out to compare serum total antioxidant capacity (TAC) in the newly diagnosed children with epilepsy and that of a control group of healthy children at the same age and probable effects of antiseizure medications prescription on it. Materials & Methods Overall, 130 participants (65 in each group) aged between 1 and 17 yr old were enrolled. The study was conducted in Children’ s Medical Center, the Pediatrics Center of Excellence, Tehran, Iran in 2015. Serum TAC test was done for both control and patients group before antiseizure medications therapy and after 3 months of monotherapy with sodium valproate, carbamazepine and phenobarbital in patients. Serum TAC values were measured based on Erel’ s method using an automated commercial kit. This method is based on the bleaching of the characteristic color of a more stable 2, 2’ ‑ azinobis‑ (3‑ ethylbenzothiazoline‑ 6‑ sulfonic acid) radical cation by antioxidants. The results were expressed in mmol Trolox equivalent/l. Results Serum TAC values were significantly lower in the patients group before drug administration [mean (SD): 1. 31 (0. 19) mmol/L] in comparison with the control group [mean (SD): 1. 46 (0. 21) mmol/L] (P<0. 001). In the patient’ s group, no differences were found in the serum TAC before and 3 months after antiseizure medications monotherapy. Conclusion Reduced serum TAC and an increased vulnerability to oxidative stress should be considered as an etiologic factor in the children with epilepsy.

Objectives Preterm birth is considered as a risk factor for developmental disabilities, which can lead to long-term effects on the nervous system of children. The aim of this study was to determine the effect of multi-sensory stimulation on neurodevelopment of premature infants. Materials and Methods In this two-group double-blind clinical trial in Jahrom Hospital, Jahrom, Iran from Jun to Aug 2016, 80 preterm infants were randomly divided. The intervention group received multisensory stimulation for 12 min per session, 5 sessions per wk along with routine NICU care and the control group received ward’ s routine care. Neuromuscular maturity for each infant was assessed by New Ballard Score. Data were analyzed using independent t-test. Results Based on ANOVA with repeated measures, New Ballard score significantly changed in the intervention group before and after intervention (P= 0. 001). This change was also significant in the control group (P=0. 04). However, the changes in New Ballard score were significantly different before and after intervention between the two groups (P=0. 001). Conclusion Multi-sensory stimulation can have beneficial effects on the development of neuromuscular in premature infants.

Objectives Comprehensive ICF Core Set of cerebral palsy (CP) includes a set of functions of children with CP has been created recently. This study determined the content validity of this version based on Iranian Occupational Therapists’ perspectives to explore whether the ICF Core Sets for CP include the areas of function of CP in Occupational Therapy practice. Materials & Methods This qualitative study conducted from Feb 2015 to Apr 2016 in Tehran, Iran. Experts were the academic staffs selected through convenience sampling. Content validity of comprehensive ICF-Core Set of CP with 135 ICF categories was done by them. Delphi survey was used for generating consensus on the final version. Participants were 50 clinical Occupational Therapists invited via email from across Iran. An agreement of 75% was considered as the cut-off for inclusion of each code-category. Results About 60% of the code– categories of comprehensive version of ICF Core Set of CP were approved by Occupational Therapists. In the final version, 82 code-categories were listed that included 21 code-categories for Body Functions, 40 for Activity/Participation, and 21 for Environmental Factors. Conclusion The validity of the Iranian ICF Core Set for children with CP aged 0– 6 yr was supported by Iranian Occupational Therapists. It could be the basis for evaluation of this population in Occupational Therapy.

Objective: Neonatal seizures are common, difficult to diagnose and treat, and associated with a great mortality rate and long-term risk of neurodevelopmental impairments. We aimed to determine the etiology, clinical presentation, and neurodevelopmental outcome of neonatal seizures. Materials and Methods In this cross-sectional study, 88 neonates, aged < 28 days, admitted to Mofid Children’ s Hospital, Tehran, Iran, from September 2011 to 2013 with the initial diagnosis of seizure were enrolled by convenient sampling method. Data, including age, gestational age, birth weight, Apgar of the fifth minute, neonatal intensive care unit (NICU) admission, family history, type, cause, and age of seizure, EEG and paraclinic findings, anticonvulsant used for treatment, neurodevelopmental status, and the final outcome, were collected from medical records and the missed cases were completed by phone calls. The frequency and association of variables were analyzed using SPSS software. Results Among neonates with seizures, 67% were male, 79. 5% were born term, and 72. 7% had normal birth weight. The most common type of seizure was multifocal clonic seizures (45. 5%). The main diagnosis in neonates with seizures was hypoxic-ischemic encephalopathy (HIE) (23. 9%) and hypoglycemia (10. 22%). The mortality rate was 11. 36% during a mean follow-up period of 21. 4± 6. 4 months. Neurodevelopmental assessments showed that 64% were normal, 27% had global delay, and 9% had motor delay. Positive family history of epilepsy (P=0. 006), low Apgar score (P=0. 002) and epilepsy (P<0. 001) were significantly associated with adverse neurodevelopmental outcome. Conclusion Since HIE and hypoglycemia were the most common cause of neonatal seizures, efforts should be made to improve care during delivery and early breastfeeding.

Objective Febrile seizures are the most common type of convulsions. Medicinal prophylaxis is sometimes used for children at high risk of recurrent febrile seizure. In certain circumstances, conventional drugs such as diazepam and phenobarbital cannot be used and the need for alternative medicines is felt. This study compared the effectiveness of topiramate and diazepam in preventing the risk of recurrent febrile seizure in children under 2 yr old. Materials and Methods This randomized controlled trial, in Besat Hospital in Hamedan, Iran from 22 Nov 2013 to 22 Nov 2015 (Registered code: IRCT Number: IRCT2015010120527N1), included 54 patients, at risk of recurrent febrile seizure, inhibited from taking phenobarbital. Samples were randomly divided into two groups. The first group received diazepam treatment during fever episodes and the second group received daily dose of topiramate. A one-year follow-up of recurrent febrile seizure and its complications was also conducted. Results Thirty-four patients (17 patients in each group) completed the one-year course of the trial. Recurrent febrile seizure was not observed in the course of preventive treatment. The prevalence of minor complications was 29. 4% in the diazepam group and 48. 5% in the topiramate group. No major complication was observed in among the subjects Conclusion Topiramate can be recommended for preventing recurrent febrile seizure when the use of frontline medicines is not possible.

Objective Migraine is a common headache associated with structural changes in brain. The purpose of this study was to evaluate brain MRI findings in children with migraine. Materials & Methods This cross-sectional study was conducted at Booali Hospital, Mazandaran University of Medical Sciences, Sari, Iran. Participants with headache and age between 5 and 15 yr were evaluated with MRI and their headache type was diagnosed by the standard criteria. The findings of the MRI were interpreted by a radiologist blinded to the diagnoses. Results Overall, 81 individuals with symptom of headache and the mean age of 9. 56± 3. 25 yr were enrolled. Twenty patients with the mean age of 9. 65± 2. 75 yr were diagnosed with migraine without aura. Among the 54 male patients, 8 patients (14. 8%) were diagnosed with migraine; and among the 27 female patients, 12 patients (44. 4%) were diagnosed with migraine (RR: 1. 5, 95%CI: 1. 07-2. 18, P=0. 004). Ten migraine patients had abnormal MRI findings (50%), including 8 cases with high signal white matter lesion, and 2 cases with empty sella. The occurrence of the high signal white matter lesions was significantly greater in the migraine patients (RR: 3. 91, 95% CI: 2. 10-7. 25, P=0. 001). Conclusion The possibility of occurrence of the high signal white matter lesions in the brain MRI of children with migraine was significantly higher compared with other headache types.

Objectives: Febrile seizure is the most common seizure disorder in childhood. Anemia or failure to thrive can predispose children to febrile seizure by affecting the nervous system function. The current study investigated the association between febrile seizures and anemia or failure to thrive. Materials and Methods: This case-control study was performed on 307 children 6 months to 6 yr old age hospitalized at the Ali Asghar Children`s Hospital, Tehran, Iran from 2011 to 2014 divided into two groups as follows: A case group including 158 children with febrile seizures and a control group including 149 febrile children without seizure. The amount of Hgb, Hct, RBC count, MCV, MCH, and MCHC was recorded and weight-for-age and weight-for-height was calculated based on the WHO Z-Score charts. The data were compared between two groups. Results: There were no differences regarding age and sex between the groups. Statistically significant differences were found regarding the mean RBC count between the case group (4. 38×106 ± 0. 72×106) and the control group (4. 24×106 ± 0. 84×106) (P=0. 013), as well as about the mean MCV that was 78. 73 ± 0. 97 and 76. 78 ± 1. 00 in the case and control groups, respectively (P=0. 005). Anemia was seen in 28. 5% of the cases and 42. 3% of control group which was statistically significant (P=0. 012). There was not statistically significant difference regarding failure to thrive between two groups. Conclusion: In children with febrile seizures, anemia was lower comparing with febrile children without seizure. Moreover, there was not any association between failure to thrive and febrile seizures.

Objective: This study was conducted to predict the response to treatment in patients treated with anti-epilepsy drugs. Material and Methods: This analytical questionnaire-based study was conducted in 2014 among 128 patients with epilepsy admitted to Mofid Children’ s Hospital, Tehran, Iran. The inclusion criteria were children 2 months to 12 yr of age with epilepsy and patients who experienced fever and seizure attacks at least once were excluded from the study. Patients were followed up for 6 months and the response to their treatment was recorded. The good response to treatment was defined as the absence of seizure with two drugs during follow up. Results: Seventy-two patients (56. 3%) were boys. The age of the first seizure was under 2 yr old in 90 patients (70. 3%). History of febrile convulsion, family history of epilepsy and history of asphyxia was found in 16 (12. 5%), 41 (32%), and 27 (21. 1%) patients, respectively. Seizure etiology was idiopathic in 90 patients (70. 3%), and the number of seizures was 1-2 in 36 patients (28. 1%). Overall, 57 patients (44. 5%) had cerebral lesion according to CT scan or MRI, and EEG was abnormal in 101 patients (78. 9%). In 6-month follow-up, 40 patients (31. 3%) responded well to the treatment and 88 patients (68. 8%) responded poorly to the treatment. History of asphyxia (OR = 6. 82), neonatal jaundice (OR = 2. 81) and abnormal EEG (OR = 0. 19) were effective factors in response to treatment. Conclusion: Abnormal EEG is an effective factor in treatment response in the children studied.

Objective: As there were a few studies on the mental disorders resulting from diabetes in children, this study aimed at investigating the prevalence of psychological disorders among children. Materials & Methods We enrolled 323 children with diabetes type 1 aged 5-12 yr old referring to Endocrinology Clinic of Mofid Hospital, Tehran, Iran in 2014-2015. In addition, 317 healthy children were considered as control group. The materials used for data analysis were information form and questionnaire CSI-4 filled out by their parents. The filled questionnaires were rated in that day and then analyzed and diagnosed by the Pediatric Psychiatrist in order to determine the type and intensity of psychological disorder. Results were analyzed using SPSS 20. T tests, Scheffe post hoc test and Pearson’ s correlation test were used for analysis of data. The amounts were significantly different at P<0. 05. Results In terms of Neuro-Evolutionary disorders, Attention-deficit (ADD), hyperactivity (HD) and Attention-deficit hyperactivity disorders (ADHD) in children with diabetes were significantly higher than those in healthy children (P=0. 001). Severe fundamental depressive disorders were higher in diabetic children (P=0. 001). In terms of anxiety disorder, a specific phobia and panic was significantly higher in diabetic children (P=0. 005). Regarding aggressive behaviors, diabetic children were more disobedient and stubborn than the others. Conclusion The prevalence of psychological disorders among diabetic children was higher than that of the others. As psychological disorders will effect on the life quality of children, improvement of life quality of diabetic children and adolescents, on-time diagnosis and treatment of psychological disorders in these patients seems essential.

Beta-ketothiolase deficiency is a rare autosomal recessive disorder characterized by an inborn error of isoleucine catabolism and affecting ketone body metabolism. Clinical features characterized by intermittent keto acidotic episodes are associated with clinical signs and symptoms of toxic encephalopathy such as lethargy, hypotonia, vomiting, tachypnea, and coma in some patients, with an onset during infancy or toddler-hood. A two months old girl presented to pediatric ward of Imam Reza Hospital in Mashhad City, Northwestern Iran in October 2016, with acute episode of fever and toxic encephalopathy with attack of vomiting, hypotonia, lethargy, tonic-clonic seizures and then a day in coma, few days after vaccination. After then similar episodes happened until 7 months age. Bio chemical tests that suggested diagnose of beta ketothiolase deficiency were attacks of ketoacidosis with urinary exertion of 2-methyl-3-hydroxybutyric acid 2-methyl aceto acetic acid tiglylglycine. In genetic assessment, we detected a novel homozygous mutation c. 664A> C (p. Ser 222 Arg) in ACAT gene. This is the first report of beta ketothiolase deficiency confirmed by molecular analysis from Iran. We report on a homozygous variant in the ACAT1 gene and that is a novel mutation. We recommended carrier testing for all informative family members to recognize mutations in asymptomatic family members.

When a child presents with cranial nerve palsy and a bulging fontanel, a pediatric neurologist is often consulted to determine the cause of increased intracranial pressure. This report describes an infant with chronic myelogenous leukemia (CML) referred to Ali-bin-Abitaleb Hospital, Zahedan, eastern Iran in 2013 who presented with seventh cranial nerve palsy and bulging fontanel. Chromosomal analysis and peripheral blood smear confirmed the diagnosis of CML.

Schimke Immuno-osseous Dysplasia (SIOD) is a rare autosomal recessive disease caused by a biallelic mutation in SMARCAL1 gene. Typical findings in SIOD include spondylo-epiphyseal dysplasia, steroid resistance nephrotic syndrome, progressive renal failure, T-cell immunodeficiency, bone marrow failure, and cerebral infarction. In this case report, we describe a 9-yr-old girl who presented with failure to thrive in infancy. Nephrotic syndrome was diagnosed at the age of four years. She had three episodes of admission with cerebral stroke due to moyamoya syndrome. In the last admission at Namazi Hospital, Shiraz, southern Iran, in October 2016, she had new cerebral ischemia, developed seizure, and finally died.

Guillain-Barré syndrome (GBS) belongs to the group of peripheral immune-mediated neuropathies often preceded by an inflammatory episode. GBS is rarely associated with hepatitis A virus (HAV) infection, the latter as a rule antecedent of the neurological disorders. This association is quite rare in childhood, and so far, only isolated cases have been described. We report an unusual case of pediatric GBS which development coincided with the development of HAV IgM (+) viral hepatitis A. From the second to the 14th day after admission to the hospital for mild jaundice of the skin and sclera in a 12-yr-old boy, the following neurological disorders have developed: absent Achilles and knee-jerk reflexes, diminished brachioradialis reflex, moderately decreased muscle power in the upper extremities and more pronounced power loss in the lower extremities. Facial palsy developed bilaterally, more expressed to the right. There was albuminocytologic dissociation of the cerebrospinal fluid and electrodiagnostic study showed findings compatible with the GBS subdivision-Acute inflammatory demyelinating polyneuropathy (AIDP). HAV could trigger GBS in the very beginning of liver inflammation in children. This insight may help wide range of medical professionals to early recognize and treat the peripheral neuropathy.

Dear Editor-in-Chief I wrote this letter for discussing with the author of the paper titled “ Beta-Lactam antibiotics as a possible novel therapy for managing epilepsy and autism, a case report and review of literature” (1). This paper is very interesting because betalactam antibiotics were regarded as a kind of drugs, which could cause epilepsy all the time (2-4)...