Iranian Journal of Child Neurology最新文献

Objectives: Migraine is one of the common diseases of children, which can disrupt their quality of life. Some studies have shown the effect of melatonin in reducing migraine headaches. This study aims to investigate the effect of melatonin administration in reducing headaches in children with migraine without sleep disorders.

Materials & methods: In this clinical trial study, fifty-five children aged five to 15 years with migraines who had no sleep disorder were enrolled. The control group (twenty-seven patients) was treated with propranolol tablets, and the intervention group (thirty patients) was treated with propranolol tablets plus melatonin tablets for three months. Patients were visited before, one month, and three months after the start of treatment, and their data was collected and recorded.

Results: The number of headache attacks decreased significantly in the intervention group compared to the control group three months after the treatment (P=0.006). The number of patients with a good response to treatment in the intervention group was significantly more than the control group (p=0.023). Parents' satisfaction with the treatment in the intervention group was significantly higher than the control group (P=0.026). There was no significant difference in the intensity of disability caused by headaches after treatment in the two groups. No significant drug side effects were seen in any of the two groups.

Conclusion: Adding melatonin to the treatment of children with migraine without sleep disorders significantly reduces the frequency of headache attacks and increases satisfaction with the treatment.

Objectives: Hypoxic-ischemic encephalopathy (HIE) is still a relevant cause of neonatal mortality and morbidity. HIE severity can predict long-term outcomes. Sarnat staging is one of the most common methods used to evaluate HIE severity. However, an ongoing urge exists to find other accurate and affordable ways to accompany this clinical staging for HIE. This study aimed to evaluate the relationship between cerebral arteries' resistive indices and other hypoxic-ischemic encephalopathy indicators using Sarnat scoring of newborns subjected to perinatal asphyxia.

Materials & methods: In this retrospective study, 76 neonates with gestational age ≥34 weeks affected with HIE were investigated. The patients were categorized into three groups according to Sarnat staging: I, II, and III. Initially, perinatal data were analyzed to assess the correlation between HIE severity and various factors such as gestational age, type of delivery, Apgar scores, necessity for resuscitation, and requirement for respiratory assistance. Notably, these relationships were significant.

Results: Examining various symptoms in different HIE stages showed that the incidence of coagulopathy was significantly higher in severe HIE neonates than in mild neonates. Eventually, proposedly, cranial arterial Doppler indices, i.e., the anterior cerebral artery's resistive index (RI), significantly differed between HIE stage groups.

Conclusion: This study represented a combination of available and affordable data to achieve early HIE staging, including perinatal data, clinical symptoms, and a bedside Doppler ultrasonography of cerebral perfusion. Higher cranial artery RI was associated with severe HIE and could be considered for therapeutic hypothermia, which may reduce HIE mortality and morbidity.

Objectives: This study evaluated the efficacy of Polyethylene glycol 4000 for fecal disimpaction in children with cerebral palsy.

Materials & methods: A randomized control trial study was conducted on children with cerebral palsy between February - March 2017 in the pediatric neurology outpatient clinic Dr. Soetomo Hospital. Children aged 2-16 years with fecal impaction randomly assigned into polyethylene glycol 4000 (PEG 4000) and saline enema group. Polyethylene glycol 4000 was given at a dosage of 0.7 g/kg and enema using normal saline 15ml/kg twelve hourly. Constipation was diagnosed using ROME IV criteria, and abdominal palpation identified fecal impaction. Efficacy was evaluated by clinical observation and adverse symptom monitoring. Data were analyzed by statistical software using an independent t-test (p<0,05).

Results: Thirty-two children were randomized into the study. Muscle relaxant was discovered in 17/32 patients. Sex, age, and body weight were not statistically different between groups. The resolution of fecal impaction was significantly different between PEG 4000 and saline enema (21.69 hours and 39 hours respectively; p=0.001). Application of muscle relaxant and severity of the disease did not involve treatment efficacy. There was no adverse symptom reported during treatment.

Conclusion: Polyethylene glycol 4000 results in fecal disimpaction faster than enema in constipated children with cerebral palsy.

Objectives: Therapeutic plasma exchange (TPE) is a plasmapheresis procedure whose Safety data for pediatric neuro-immunological disorders (PNID) is confined. The present research documents TPE's safety and feasibility data in these conditions.

Materials & methods: The current study involved six distinct groups of patients with PNID undergoing TPE: neuromyelitis optic spectrum disorder (NMOSD), autoimmune encephalitis (AIE), acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), Guillain-Barre syndrome (GBS), and optic neuritis (ON). This study documented complications related to each TPE process. In addition, TPE's efficacy was studied in these patients.

Results: The present study recorded adverse effects in 18 patients with PNID that received 121 TPE cycles: five cycles (4.13%) in MS, three (2.48%) in AIE subgroup, one (0.82%) in ADEM, and two (1.65%) in GBS. No severe complications were observed among the patients.

Conclusion: Patients with PNID tolerated therapeutic plasma exchange, which was a safe process.

Spinal muscular atrophy (SMA) with progressive myoclonic epilepsy (PME) affects the nervous system. Symptoms appear in early childhood and include muscle weakness, difficulty walking, seizures, and cognitive decline. Despite introducing various therapies to restore acid ceramidase function or reduce ceramide accumulation and gene therapy to correct genetic mutations, there are still unknown underlying molecular mechanisms related to this disorder. This article reports a novel variant c.118G>C in the ASAH1 gene. The patient presented with clinical manifestations such as progressive muscle weakness and myoclonic convulsions. Clinical features and electrophysiological investigations revealed a motor neuron disease and generalized epileptic discharge. A significant temporal interval was observed between the initial diagnosis of SMA and the subsequent manifestation of myoclonic seizures. The proband was genetically assessed through whole exome sequencing (WES) followed by variant confirmation and bioinformatics analysis. According to this article's findings and previous research, further diagnostic testing and management are needed to determine the severity and progression of the patient's condition.

Myasthenia gravis (MG) is the most frequent transmission disease in the neuromuscular junction. Juvenile myasthenia gravis (JMG) is an autoimmune antibody-mediated disease of postsynaptic endplate defined as MG presentation in patients before the age of 18 years old. While many clinical features of JMG are identical to the adults, there are some significant differences between them regarding presentation, clinical course, antibody level, and thymus histopathology. In JMG, ocular symptoms are more frequent, the clinical course is comparably benign, and the outcome is better than adult MG. Antibodies attack the muscle endplate proteins in the postsynaptic membrane and interfere with transmission. These antibodies in most patients are against the acetylcholine receptors, but they may also be directed toward muscle-specific kinase, lipoprotein-related protein 4, and agrin. Findings show racial influences and genetic effects on the occurrence of JMG. The essential clinical symptom is fatigable weakness of muscles that can be in the form of isolated ocular type or more disseminated weakness. The diagnosis of JMG is essentially clinical, with fluctuating patterns of weakness and easy fatigability, but a series of diagnostic evaluations can confirm the diagnosis. Precise diagnostic evaluation and distinction from congenital myasthenic syndromes is critical. The treatment plan is conducted according to the clinical course (ocular or generalized), antibody type, and disease severity. The mainstay of treatment includes symptomatic therapy, long-lasting immunosuppressive treatment and treatment of myasthenic crisis. Novel medications are introduced and conducted to the specific pathophysiologic mechanisms of the disease, and they are used primarily in the refractory MG.

Objectives: Hyperinsulinism refers to improper insulin secretion in the presence of low plasma glucose, causing severe and persistent hypoglycemia in infants and children. The brain's occipital lobe, which includes the visual and plays an essential role in visual perception is specifically sensitive to hypoglycemia-induced damage. The present study aims to investigate the visual perception in children suffering from hyperinsulinism and to compare it with the control group.

Materials & methods: This cross-sectional control study, conducted in 2020 in Isfahan, Iran, involved 20 children aged 4-13 years with hyperinsulinism and 20 healthy children of the same age and gender for comparison. In both groups, the measuring instrument was the Test of Visual Perceptual Skills (non-motor) Third Edition.

Results: The mean visual perceptual quotient in the case and control groups was 80.50±26.74 and 116.50±7.56 (p-value<0.001), respectively. The results overall indicated that children suffering from hyperinsulinism were weaker than healthy children in all areas of visual perception.

Conclusion: Based on the obtained results, it is recommended that children suffering from hyperinsulinism be screened regarding visual perceptual disorders since this screening may be helpful in initiating different rehabilitation programs among these patients.

New daily data on the COVID-19 pandemic are circulating globally. This disease usually appears with respiratory symptoms such as cough, shortness of breath, and fever. The neurological complications of the disease are somewhat known in adults but rarely reported in children. Acute necrotizing encephalopathy of childhood (ANEC) is one of the brain complications associated‌ with Coronavirus disease that usually has a poor prognosis in children. In this case, we report a rare case of a 7-year-old boy who was referred to the hospital with symptoms of convulsions after contracting COVID-19 and developed cerebral necrotizing encephalopathy caused by COVID-19 infection. Although ANEC is a rare disease, clinical examination and MRI and CT scan findings play an essentialrole in diagnosing and treating the disease.‌.

Objectives: The progress of cardiac surgery in children and the increase in the survival of children with Congenital Heart Disease (CHD) has led to consider another issue called a neurodevelopmental disorder. In this study, 53 children with CHD were evaluated in terms of development with the Essence Q questionnaire, Otoacoustic Emission (OAE), and Auditory Brainstem Response (ABR) regarding these patients' hearing and risk factors. The Essence Q scores were also examined.

Materials & methods: In this prospective, cross-sectional study, the researchers included 53 children diagnosed with CHD. Initially, each child underwent ABR and OAE tests. Subsequently, data on potential risk factors associated with neurodevelopmental delay were collected. A trained project associate administered the Essence Q questionnaire, using parents' information as a guide. Following data collection, this study proceeded with an in-depth analysis of the information.

Results: Thirty-six boys (67.92%) and 17 girls (32.08%) with CHD were included in the study. The mean age of children was 26.98$\pm$10.64 months. The mean Essence Q score for boys was 7.48$\pm$2.57. Moreover, the average score for girls was 2.23 $\pm$ 8.11. According to this questionnaire, 39 patients (73.58%) had hyperactivity disorder, 46 patients (86.79%) had behavioral disorders, and ten patients (16.98%) had a motor delay. Unlike previous studies, all patients had normal OAE and ABR hearing.

Conclusion: This study demonstrated that factors such as developmental delay in the first year, a known genetic disease, and a history of seizures significantly impacted the Essence Q score. However, elements like prematurity, the use of ventilation, abnormalities on the dorsum, and the number of days post-surgery did not significantly affect the Essence Q score. Essence Q can be a reliable tool in screening for neurodevelopment in children with CHD.

Objectives: Seizures are changes in the electrical activity of the brain. These changes can cause significant or otherwise asymptomatic symptoms. Phenobarbital and phenytoin are known drugs for treating neonatal seizures, but little clinical experience exists using other drugs. The present study aims to evaluate the efficacy of other drugs, such as Levetiracetam and Topiramate, compared to Phenobarbital in treating neonatal seizures.

Materials & methods: In a double-blind clinical trial, all neonates admitted to a referral hospital for two years (2020-2022) due to seizures were included. All of the neonates were treated with a dosage of 10-40mg/kg/state IV Phenobarbital to control the acute seizure. After that, they were divided into three groups with specific treatment programs. Groups were ordered with oral Phenobarbital 5mg/kg/day maintenance (first group), oral Topiramate 3-8mg/kg/day (second group), and 10-40mg/kg/day Levetiracetam (third group). Seizures and potential side effects were investigated through interviews and medical EEG tests. The data was analyzed using the Chi-square test.

Results: Sixty infants (20 neonates in each group) were studied. Phenobarbital, Italept, and Topiramate did not significantly differ in controlling convulsions and changes related to brain paroxysmal discharges.

Conclusion: Due to the long treatment duration and side effects, it is essential to choose the appropriate drug for treating treatment-resistant seizures of neonates. The present study found that Phenobarbital, Levetiracetam, and Topiramate are equally effective in controlling seizures. These medications can also help eliminate abnormalities in children's brain paroxysmal.