VITAMIN B6 & TREATMENT OF INFANTILE SPASMS: A COMPARISON WITH STANDARD STEROID THERAPY

BackgroundConsidering the inadequacies of current therapeutic regimens for infantile spasms (IS), and the frequent and serious side effects of  Some regimens, the ongoing search for more enhanced protocols is understandable.Materials and Methods:We have compared the therapeutic and adverse effects of vitamin B6 given in high doses with those of prednisolone in a randomized controlled clinical trial. Vitamin B6 (40mg/kg/24hr) and prednisolone (1.5mg/kg/day) were given to in 22 and 15 patients respectively, and the patients were followed for at least 6 months.Results:Response to treatment was slightly better in the prednisolone group but the difference was not significant (p=0.4). On the other hand adverse effects were also seen more frequently with prednisolone.Conclusion:We conclude that high dose vitamin B6 should be considered as an alternative method of treatment; it seems that it can be safely used where there is contraindication to use other antiepileptic drugs or where they have failed; even in newly diagnosed cases of IS.Keywords:Vitamin B6, prednisolone, infantile spasm

Intractable Seizure Disorders: Efficacy of The Classic Ketogenic Diet

 ObjectiveThe ketogenic diet is a high-fat, low carbohydrate, adequate protein diet,developed in the 1920s for the management of intractable seizure disorders in children. To evaluate efficacy and tolerability of the classic  ketogenic diet, we analyzed records of the children started on the diet from 1999 to 2006 at the Mofid children's hospital.Materials & Methods The subjects were 87 children, mean age 55 months. Before initiation of the diet, 55% of the patients had seizures, at least 1-4 times per day, 36% - 5 or more per day and 9% - 2 to 4 times per week. Mean number of Anti Epileptic Drugs (AEDs) tried for them was 8 and 67% were receiving three or more drugs.Results The ketogenic diet showed drastic improvement, with at least 50% reduction in seizure frequency in 87% of our patients, 39% of whom showed complete seizure control in the third month. After one year, in 80% of the patients who returned, improvement  continued, with 26% of them being seizure free; besides, 23% had one AED decreased, 36% had two or three AEDs decreased, and 25% (one child) had all AEDs discontinued. Of the 30 improved cases, 20%, at the end of the first year, had improved behavior as  well, and 23% of them had become more alert. The median diet duration of the improved group was 15 months.Conclusion The improvement in our patients, low  side effects, and the duration of diet by families reveal that the ketogenic diet can still be a very useful alternative therapy in certain epileptic children.

Sodium Valproate and Phenobarbitol: Weight Complications of Treatment in Epileptic Children

Objective The aim of this study was to evaluate and compare the effects of Na Valproate and Phenobarbital on changes in the weight of epileptic patients following treatment for their condition using the drugs mentioned.Materials and methodsSixty epileptics were assigned into two groups of 30 patients each, the case and controls. The diagnosis was made on the basis of the International League Against Epilepsy (ILAE) characteristics. BMI was defined. In the case group, the patients received 20mg/kg/day of Na Valproate, while the 30 controls received 5mg/kg/day of Phenobarbital for 6 months. Using the Mc Nemar and Chi-2 tests, BMI changes were compared after 6 months between the groups. Fisher's exact test was used to evaluate the role of age, sex, and primary weight on the weight increase due to Na Valproate usage.ResultsThere were no specific changes in age, sex, primary BMI and fatness between the 2 groups; in the case group, 20 patients(66.7%) and in the controls 4(13.3%) gained weight (PConclusionThe results indicate that epileptic children, aged over 10 years, and those who are overweight have more chances of gaining weight or becoming fatter, following treatment with Na Valproate. Further studies investigating the issue are warranted

Primary Central Nervous System Lymphoma

ObjectivePrimary central nervous system lymphoma (PCNSL) is an extremely rare condition in childhood. We report the first case of PCNSL in a child in Iran.Clinical presentationA nine-year-old boy was referred to Mofid Hospital with the history of headache of four months and seizure of 2 months duration. Magnetic resonance imaging of the brain revealed a hyper-intense lesion in left fronto-parietal area with secondary satellite lesions. Biopsy of the brain mass was performed. Pathologic findings showed brain lymphoma and immunohistochemistry confirmed this diagnosis. The treatment started with intrathecal and systemic chemotherapy in combination with radiotherapy

Relationships between Locus of Control and Adherence to Diabetes Regimen

Abstract Background: Adequate self-care in diabetes causes quality of life promotion and decreases the number of inpatient cases. The health locus of control theory is used to assess adherence to diabetes regimen in some studies in developed countries. The purpose of this study was to determine the status of diabetes locus of control in a sample of diabetic patients in Iran and investigation of it's relationship to adherence to diabetes regimen. Methods: This analytical and cross-sectional study was carried out on 120 patients referred to Yazd Diabetes Research Center. The Iranian versions of Diabetes Locus of Control scale and Diabetes Selfcare Activities scale were used for data collection. Results: Men were more internal locus of control and women were more chance locus of control. The attributions of external locus of control increased by age, while the internal locus of control increased by education level and chance locus of control decreased by education level. A positive association between internal locus of control and adherence to diabetes regimen was found and there was a negative association between chance locus of control and adherence to diabetes regimen. Conclusion: Findings suggest that interventions aimed at improving internal locus of control may improve adherence to diabetes regimen but different diabetic patients have different attribution styles and interventional programs to enhance diabetes self-care will be more successful if patient's locus of control is addressed

Reporting a rare form of myopathy, myopathy with extrapyramidal signs, in an Iranian family using next generation sequencing: A case report

Background: Myopathy with extrapyramidal signs (MPXPS) is an autosomal recessive mitochondrial disorder which is caused by mutation in mitochondrial calcium uptake 1 (MICU1) gene located on chromosome 10q22.1. Next Generation Sequencing (NGS) technology is the most effective method for identification of pathogenic variants with the ability to overcome some limitations which Sanger sequencing may encountered. There are few reports on this rare disease around the world and here in this study we first revealed genetic identification of two affected individuals in an Iranian family with a novel mutation. Case presentation: The proband was a 5-year-old girl from consanguenous parents. She was first clinically suspicious of affected with limb-girdle muscular dystrophy (LGMD). Muscle biopsy studies and autozygosity mapping, using four short tandem repeat (STR) markers linked to 6 genes of the most prevalent forms of LGMD, ruled out calpainopathy, dysferlinopathy, and sarcoglycanopathis. DNA sample of the proband was sent for NGS. Whole exome sequencing (WES) revealed a novel mutation c.1295delA in exon 13 of MICU1 gene. This homozygous deletion creates a frameshift and a premature stop codon downstream of canonical EF4 calcium binding motif of MICU1. According to the American College of Medical Genetics and Genomics (ACMG) guidline for sequence interpretation, this variant was a pathogenic one. Sanger sequencing in all family members confirmed the results of the WES. Conclusions: This study was the first report of MPXPS in Iranian population which also revealed a novel mutation in the MICU1 gene. © 2020 The Author(s)

Prediction of response to treatment in children with epilepsy

Abstract 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. Key Words: Pediatric, Anti-seizure drug, Response to treatment, Children, Epileps