Iranian Journal of Child Neurology最新文献

Objectives: Pediatric arterial ischemic stroke (AIS) is a rare but serious condition leading to significant neurological disability. AIS is classified into anterior circulation AIS (ACAIS) and posterior circulation AIS (PCAIS). Understanding the differences between these subtypes is essential for early diagnosis and effective management. This study aimed to evaluate the demographic, clinical, and laboratory characteristics of pediatric ACAIS and PCAIS, focusing on lipid profiles, prothrombotic factors, and SARS-CoV-2 prevalence.

Materials & methods: A descriptive-analytical cross-sectional study was conducted on 34 children diagnosed with AIS at the Tabriz Children's Hospital from March 2020 to October 2021. Patients were categorized into ACAIS, PCAIS, and mixed, involving both anterior and posterior circulations based on neuroimaging findings. Demographic data, medical history, and laboratory parameters, including coagulation factors, lipid profiles, and SARS-CoV-2 antibodies, were analyzed. Statistical comparisons were performed using SPSS version 26, with a p-value of <0.05 considered statistically significant.

Results: Among the 34 patients, 73.5% were diagnosed with ACAIS, 20.6% with PCAIS, and 5.9% with both. PCAIS patients were older (median age: 8.0 vs. 2.0 years), and sex distribution varied, although not significantly. No significant differences were found in lipid profiles, coagulation factors, or SARS-CoV-2 prevalence between ACAIS and PCAIS groups.

Conclusion: No significant differences in demographic features, lipid profiles, or prothrombotic conditions were observed between ACAIS and PCAIS, suggesting that pediatric stroke mechanisms may differ from those in adults. Further large-scale studies are warranted to validate these findings and improve pediatric stroke management.

Objectives: Epileptic Spasm (ES) is a special type of convulsive disorder that primarily occurs in infants, typically within the first year of life. This disorder is usually resistant to routine anticonvulsant drugs. This study aims to evaluate the epidemiology and factors affecting one-year prognosis in patients with ES in Tabriz Children's Hospital.

Materials & methods: This descriptive-analytical cross-sectional study focused on patients diagnosed with ES who were referred to the neurology department of Tabriz Children's Hospital between 2015 and 2019. Data was collected using a census sampling method and a checklist that included demographic information and clinical and treatment histories. The results were then reported statistically.

Results: Thirty-seven patients were studied, with 15 (40.5%) exhibiting flexor seizures, eight (21.7%) extensor seizures, and 14 (37.8%) mixed seizures. The most common causes were prenatal insult (10 cases, 27.0%), cryptogenic (9 cases, 24.3%), and Central Nervous System (CNS) malformations (6 cases, 16.2%). Electroencephalography findings included modified hypsarrhythmia in 12 cases (30.8%), hypsarrhythmia in 16 (41.0%), and frequent epileptiform discharge in nine (23.1%). MRI and CT scans showed abnormalities in three cases (8.1%) and 16 cases (43.2%), respectively. Treatment involved Phenobarbital in 35 patients (94.6%), Vigabatrin in 29 (78.4%), and ACTH in 11 (29.7%). The one-year prognosis indicated 25 patients (67.6%) experienced disease recurrence, seven (18.9%) recovered with complications, and two (5.4%) died.

Conclusion: The most common causes of ES are prenatal insults, cryptogenic factors, and CNS malformations. The key one-year prognoses include disease recurrence and recovery with complications.

Acute necrotizing encephalopathy of childhood (ANEC) is a devastating childhood disease characterized by rapid neurologic deterioration after a viral febrile illness. Seizures, encephalopathy, and fatal acute necrotizing encephalopathy are well-defined neurologic complications of H1N1 virus infections. Symmetrical, multifocal lesions on cranial magnetic resonance imaging (MRI) are the best-known features of ANEC. Various treatment options include glucocorticoids, intravenous immunoglobulin (IVIG), and plasma exchange (PEX). Herein, we present a 45-month-old girl diagnosed with ANEC and treated with a novel immunomodulation technique, the "zipper method." It is a combined treatment method in which PEX and IVIG treatments are used together. In the first session of plasma exchange, one and a half volumes of patients' plasma were removed using 5% albumin as a replacement solution. At the end of the PEX session, 0.4 g/kg IVIG infusion was started. The second PEX session was applied with one volume 24 hours after the end of the IVIG infusion. This plasma exchange-intravenous immunoglobulin cycle was repeated five times. Furthermore, this case report presents her outcome 3-years after discharge: full recovery. This case is a unique example of ANEC treated successfully with the zipper method.

Objectives: Cochlear synaptopathy, caused by the destruction of synaptic connections due to aging, noise exposure, and ototoxic agents, is defined as auditory dysfunction despite the normal hearing threshold, specifically in challenging situations. One of the main obstacles in synaptopathy studies and the integration and generalization of research findings is the need for a valid diagnostic test. Although the issue of identifying synaptopathy has received considerable critical attention, little agreement is available on a valid and efficient diagnostic method for cochlear synaptopathy.

Material & methods: A critical review was conducted on previous animal and human studies addressing cochlear synaptopathy, with particular emphasis on the paired-click paradigm and I' wave electrophysiological assessments. Subsequently, pertinent physiological and biophysical models elucidating excitatory postsynaptic potentials at the inner hair cell ribbon synapse were analyzed. Finally, the feasibility and limitations of I' wave recording were theoretically evaluated, with recommendations for future validation studies.

Results: A review of the existing evidence and analysis of biophysical modeling data indicate that the I' wave in the auditory brainstem response, particularly when using the paired-click paradigm, represents the excitatory postsynaptic potential (EPSP) generated at the inner hair cell ribbon synapse.

Conclusion: The present hypothesis attempts to bring forward a non-invasive tool that can investigate synaptic function. It sheds new light on future studies in cochlear synaptopathy by suggesting the I' wave as its biomarker.

Objectives: The symptoms of Attention-Deficit/Hyperactivity Disorder (ADHD) consist of persistent challenges with lack of attention and/or hyperactivity and impulsiveness. ADHD can have far-reaching and long-lasting effects on every part of an individual's life, influencing various areas such as daily activities, work, social participation, and family life. "Engagement in a life situation" defines participation and encompasses various aspects, including being present and actively engaging. There is increasing evidence that children diagnosed with ADHD have restricted participation compared to their typically developing peers. Little research has focused on the involvement of children with ADHD in their everyday routines and leisure activities. Hence, this study aims to investigate the participation patterns in extracurricular activities among children with and without ADHD.

Materials & methods: The participants included 100 children aged 7-11 years with ADHD and 100 children without ADHD of the same age. They were recruited from multiple clinics and one hospital across Tehran city as a convenient sample. All the participants completed the child symptom inventory (CSI-4) and the Children's Assessment of Participation and Enjoyment (CAPE). The study examined various aspects of participation, including diversity, intensity, enjoyment, place, and partners across 55 activities. The study was conducted online and completed by parents.

Results: The two groups showed no statistically significant difference in demographic characteristics (P> 0.05). The results show that children with ADHD had notably lower participation rates in all types and domains of activities, indicating lower intensity (P value<0.001).

Conclusion: Based on this study's findings, children between the ages of 7 and 11 with ADHD experience significant limitations in engaging in daily activities compared to their typical peers. According to the results, including extracurricular activities in treatment plans seems particularly significant. Therapists and parents should focus on the child's daily life at home and in therapy sessions and emphasize the inclusion of extracurricular activities in the treatment of children with ADHD.

Objectives: This study aimed to translate and develop the Persian version of The Visual Function Classification System (P-VFCS) while assessing its validity and reliability.

Materials & methods: The VFCS was created to classify visual function among individuals with Cerebral Palsy (CP). Until this point, no such classification system was available in Iran. Therefore, the VFCS underwent translation into Persian, with the original developer approving the back-translation. A panel of ten occupational therapists and ten parents evaluated the face validity. The Persian version of VFCS (P-VFCS) was administered to 156 participants with CP, aged 1 to 19 years (age range: 2 to 18 years; mean age: 9.1 years; standard deviation: 6.32 years). Inter-rater reliability was measured among 156 parents and four occupational therapists. Test-retest reliability was evaluated throughout two to four weeks with 77 parents and two occupational therapists.

Results: The inter-rater reliability of the weighted kappa between OTs and parents for the P-VFCS was 0.88 (95% confidence interval [CI], 0.62 to 0.89). The weighted kappa test-retest reliability for the P-VFCS was 0.91 (95% CI: 0.93 to 1.00) for occupational therapists and 0.90 (95% CI: 0.89 to 1.00) for parents. The face validity assessment revealed that 80% of parents of individuals with CP (8 out of 10) and 100% of occupational therapists (10 out of 10) found the P-VFCS suitable in translation and face validity quality.

Conclusion: The findings indicated that the P-VFCS is a reliable and valid classification system for classifying visual functions in individuals with CP within clinical settings.

Objectives: Hypoglycemia is a widespread pediatric emergency that can manifest in various ways. One of the most critical symptoms is the occurrence of seizures. Recognizing these episodes promptly is essential in managing the condition effectively. Physicians' apprehension, specifically pediatricians', can lead to early diagnosis and improve the prognosis by decreasing the neurologic aftermath. By presenting data and analysis on the prevalence of hypoglycemic seizures, triggers, and neurologic side effects, we plan to raise awareness of the issue. This study intends to demonstrate the leading causes of hypoglycemia and the major neurological sequels by assessing the prevalence of hypoglycemia in a children's hospital center to raise awareness of the condition.

Materials & methods: This cross-sectional study was conducted with two parts: demographic data (age, gender), clinical presentations, and lab data (blood sugar) on seizure onset, cause of hypoglycemia, and comorbidities before admission. The second part was based on neurologic evaluation and sequela follow-up.

Results: Collectively, 79 pediatric patients were enrolled in the study, of which 51 were male (64.6%).44.3% had experienced a first episode of hypoglycemic seizures in neonacy, 24.05% in infancy, and 31.65% in childhood. In the neonatal period, poor intake, birth asphyxia, and IUGR are, respectively, the leading causes of hypoglycemic seizures. As for the infant-child group, the most common etiology was the following: diabetic patients treated with insulin, ketotic hypoglycemia, hyperinsulinism, and Glycogen Storage Disease (GSD), respectively. The most common sequelae were global psychomotor delay, isolated speech delay, and isolated motor delay, respectively.

Conclusion: This study reveals that we can help prevent hypoglycemia by maternal education on breast-feeding and avoiding early patient discharge. Regarding the post-neonatal group, parental education on the proper application of insulin can prevent probable seizure and other consequences.

Angelman syndrome (AS) is a genetic neurodevelopmental disorder characterized by mental retardation, ataxia, and happy social behavior. Aripiprazole is an atypical antipsychotic drug used for treating irritability in patients with autism spectrum disorder in > 6-year-old children and adolescents. Notably, prolonged penile erection is an unknown complication of Aripiprazole. Therefore, the present case report aimed to present a unique prolonged penile erection in an adolescent with Angelman syndrome under Aripiprazole. In this case report, priapism occurred several hours after consuming 5 mg of Aripiprazole. Since he was neither a cigarette smoker nor an alcohol abuser, had no medical issues, and did not report any history of penile or perineal trauma, the urologist suggested that the priapism he experienced might be linked to the use of Aripiprazole. Fortunately, he recovered after stopping the medication. Generally, we mentioned controversial results from previous case reports on the effect of Aripiprazole. It is unknown why some case reports noted priapism post Aripiprazole use, and some mentioned priapism palliation by switching to this drug from other antipsychotics. While it seems that the tendency to priapism may be an idiosyncratic reaction or the α1-adrenergic receptors change or disorders in the predisposed patient, there is a need for further comprehensive studies on this issue.

Objectives: Identifying neonates at risk for hypoglycemia and establishing treatment protocols to prevent potential neurological complications are essential. This study aims to investigate the possible risk factors for epilepsy in children with a history of neonatal hypoglycemia in North Khorasan Province.

Materials & methods: This case-control study analyzed 64 children with a previous history of neonatal hypoglycemia between 2017 and 2021 in North Khorasan Province. Nineteen children with epilepsy were selected as the case group, and their MRI data were extracted from medical records. In the control group (45 individuals who did not develop epilepsy), children were randomly selected, and their data were also collected. The researchers completed the ages and stages questionnaire (ASQ) for both case and control groups at follow-up. Pregnancy, delivery, and neonatal health information was obtained from hospital records using a checklist. Statistical analysis was performed using SPSS v20, with data entry and coding accuracy ensured before analysis.

Results: This case-control study was conducted on 64 children (19 with epilepsy and 45 without epilepsy) with neonatal hypoglycemia. The mean age for the case and control groups were 4.1 and 4.6 years, respectively. An association was observed between epilepsy prevalence (58%) and familial history (p<0.05). Children who developed epilepsy had more extended periods of hypoglycemia and NICU stays (p<0.05). The age when hypoglycemia starts has a significant impact on the development of epilepsy, with an eight times higher risk for every extra day of life at the time of hypoglycemia (p<0.05). The ASQ findings revealed significant deficiencies between case and control groups (p<0.05). MRI results demonstrated that ~82% of children with epilepsy displayed irregularities, predominantly gliosis, and encephalomalacia in the occipital area (abnormal pathologic findings).

Conclusion: Neonatal hypoglycemia significantly raises the likelihood of developing epilepsy in later childhood. This risk is particularly high when the newborn requires an extended stay in the NICU, experiences delayed onset of hypoglycemia, or has a family history of epilepsy. Prompt recognition and focused intervention for newborns with these risk factors are essential to minimize the chances of developing epilepsy and related neurodevelopmental issues.

Neuroblastoma is one of the most common pediatric cancers, predominantly affecting young children. Despite progress in initial treatments, high-risk cases remain challenging due to frequent relapse or resistance, with long-term survival for relapsed or refractory neuroblastoma below 20%. This highlights an urgent need for novel therapies. Emerging approaches such as GD2-targeted immunotherapy with monoclonal antibodies like dinutuximab, CAR-T cell therapy, 131I-MIBG and Lutetium-177-Dotatate radionuclide treatments, metronomic chemotherapy, oncolytic virotherapy, and tailored chemotherapy are showing promise, with autologous stem cell transplantation (ASCT) becoming integral to multimodal regimens. However, challenges persist, including treatment-related toxicity, tumor resistance, and the logistical limitations of personalized medicine. The future of neuroblastoma treatment lies in exploiting genomic profiling, biomarkers, and combinatorial strategies like immunotherapy paired with radionuclide therapy. Rigorous clinical trials will be key to refining these innovations and establishing protocols for widespread use. In summary, advancements in therapy offer hope, yet achieving durable remissions and improved survival still demands intensive research innovation to address current gaps and resistance mechanisms in this complex pediatric malignancy.