Iranian Journal of Child Neurology

Objective: Developmental Coordination Disorder is a neurodevelopmental condition affecting approximately 5-6% of school-aged children, characterized by significant impairment in motor control and coordination, which interferes with a person's ability to perform physical movements efficiently. This systematic review seeks to consolidate known research on the effectiveness of coordination exercises in improving hand function in children with Developmental coordination disorder.

Methods: A comprehensive literature search was performed using four electronic databases: Scopus, PubMed, PEDro and The Cochrane Library databases for interventional trials published from 2010 to April 2024. The PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were used for the systematic review. The methodological quality of the studies was assessed using the PEDro scale, the GRADE system, Risk of Bias, and the Level of Evidence.

Result: The search identified a total of 383 studies through database searching. Six studies met the inclusion criteria: five were randomized controlled trials, and one was a quasi-experimental study. All interventions resulted in improvements in fine motor skills across various aspects of hand function. Specifically, haptic perception training programs and modified Taekwondo-training demonstrated significant improvement with large effect sizes (Cohen's d > 0.8) and p-values less than 0.05.

Conclusion: Coordination exercises seem to hold promise as a viable strategy for enhancing hand function in children with developmental disorders. The results indicate that these exercises could be integrated into rehabilitation regimens to augment motor skills. Nevertheless, given the varied interventions and children’s demographics under investigation, additional research is advised to develop uniform protocols and assess the enduring effectiveness of coordination exercises for this objective.

Objectives:

As permanent hearing loss is irreversible, individuals often rely on hearing aids or cochlear implants as effective rehabilitation solutions. Stem cell therapy has been used this disability at the level of preclinical studies and effectiveness has not been determined to improve of auditory brainstem response (ABR) and restore damaged hair cells of cochlea. This systematic review shows the role of stem cell injection on ABR threshold changes in noise induced hearing loss (NIHL) models.

Materials & Methods:

Search strategies for Embase, PubMed, and Web of Science were performed based on keywords stem cell, hearing loss, and noise from 2000 to 2024. Nine studies were evaluated for study variables and risk of bias, and only six studies were analyzed to estimate effect size of auditory threshold.

Results:

The broadband noise was the most common noise to create NIHL model. The improvement of ABR threshold was estimated -2.46, 95% (CI: -3.26, -1.66) after stem cell injection. Significant mean differences were observed in ABR-threshold for local injection (n = 148) and mesenchymal stem cell (MSC) injection (n = 129) with high heterogeneity between studies.

Conclusion:

According to high heterogeneity between studies, it may be concluded that MSC, systemic injection, and human-derived cells are effective in recovery of auditory brainstem function.

Objectives:

Autoimmune Encephalitis (AE) is an immune-mediated inflammatory disorder of the central nervous system with heterogeneous clinical manifestations and variable outcomes. The prognostic significance of antibody status (seropositive vs. seronegative) remains incompletely understood, particularly in Iranian populations. This study aimed to compare clinical characteristics, therapeutic interventions, and prognostic outcomes between seropositive and seronegative patients with AE (≤ 18 years) admitted to two tertiary referral hospitals in Tehran, Iran.

Material & Methods:

In this study, medical records of 58 patients diagnosed with AE between 2019 and 2024 at Ali Asghar and Hazrat Rasool Hospitals were reviewed. Patients were classified as seropositive or seronegative based on antibody testing.

Results:

Of the 58 patients, 31 (53.4%) were seropositive and 27 (46.6%) were seronegative. The mean age was 41.94 ± 24.87 months, and 72.4% were male. Seizures were the most common clinical manifestation. No significant differences were observed between seropositive and seronegative groups in initial disease severity, functional outcomes, neurological complications, treatment allocation (all p > 0.05). In multivariable analysis, initial disease severity (OR = 13.3, p = 0.032) and the presence of underlying diseases (OR = 31.4, p = 0.007) were independently associated with poor prognosis, whereas serologic status was not.

Conclusion:

Serologic status does not significantly influence disease severity, treatment response, or prognosis in patients with AE. Initial clinical severity and comorbidities are the primary determinants of poor outcome. Management strategies should prioritize early recognition, assessment of disease severity, and timely immunotherapy regardless of antibody status.

Objectives:

Autism Spectrum Disorder (ASD) is a neurodevelopmental condition characterized by impairments in communication, social interaction, and executive functions. Individuals with High-Functioning Autism (HFA) often demonstrate average or above-average intelligence and typical language development. However, difficulties in social communication persist. Previous studies have indicated that children with HFA exhibit challenges in language, particularly in grammar and vocabulary, which may be associated with executive dysfunction, including deficits in Working Memory (WM). Therefore, the present study aimed to investigate the relationship between verbal WM and grammatical abilities in Persian-speaking children with HFA.

Materials & Methods: Twenty monolingual Persian-speaking children diagnosed with HFA, who met the inclusion criteria, were recruited for the study. Morpho-syntactic abilities were assessed through the collection of speech samples analyzed using Persian Developmental Sentence Scoring (PDSS). Additionally, Phonological Working Memory (PWM) tasks, including direct and reverse recall of numbers and repetition of non-words, were administered. Statistical analyses were performed using SPSS software to examine correlations between PWM and grammatical skills.

Results: The highest significant correlation was observed between the PDSS and the repetition of three-syllable non-words, while the lowest correlation was found with the immediate repetition of one-syllable non-words. (r = 0.594, p < 0.05). Furthermore, most PDSS subtests demonstrated statistically significant correlations with PWM tasks, with correlation coefficients varying from weak to strong.

Conclusion: This study concluded that HFA children experience difficulties in their grammatical skills, and a significant relationship is observed between their PWM and grammatical abilities, underscoring the importance of considering PWM in language intervention strategies, particularly targeting grammatical development in these children.

Objectives: Childhood migraine is a prevalent neurovascular disease in children with a considerable reduction in quality of life and high social cost. Anticonvulsants, such as Valproate and calcium channel blockers, including Cinnarizine are used frequently as preventive medications. This research evaluates the cost-effectiveness and cost-utility of Cinnarizine versus Valproate in managing childhood migraine, based on an Iranian societal perspective.

Materials & Methods:

A randomized cohort trial was conducted, involving 150 children aged 5 to 17 years at the Imam Reza Clinic in Shiraz, Iran. Patients were administered Cinnarizine (1–2 mg/kg/day) or Valproate (10–20 mg/kg/day) for three months, evaluated by decision-tree models for cost-utility and cost-effectiveness. Effectiveness was measured by reduction in migraine attack frequency and Quality-Adjusted Life Years (QALYs). Costs were obtained from patient medical records, questionnaires, and national datasets, including direct and indirect costs. Sensitivity analysis, such as Monte Carlo simulations, was performed to validate the results.

Results:

Patients receiving Cinnarizine experienced a mean QALYs of 0.213 versus 0.209 for the Valproate group and a higher rate of response at 26% (Cinnarizine) versus 14% (Valproate). Total costs during the study period favored Cinnarizine ($183 vs. $191) with an Incremental Cost Effectiveness Ratio of $-156 per additional effect and an Incremental Cost Utility Ratio of $-2069 per QALYs. Adverse effects noted were drowsiness (17.8%) for the Cinnarizine and dizziness (13.5%) for the Valproate arm. Probabilistic sensitivity analysis contained an 88.2% probability that Cinnarizine was cost-effective with a willingness-to-pay of $17,922 per QALYs.

Conclusion:

Cinnarizine was a cheaper option compared to Valproate as a prophylactic agent in pediatric migraine in Iran with higher response rates, lower expenses, and similar effectiveness. Further research with long-term follow-ups and varied populations is needed to validate the results

Objective:

Spinal muscular atrophy (SMA) involves the survival motor neuron (SMN) 1 gene, leading to motor neuron degeneration. SMN2 is a homologous gene to SMN1, which can produce SMN protein at lower levels. The new gene-based drugs modify SMN2 pre-messenger RNA splicing, leading to production of functional SMN protein.

Materials & Methods:

This study aimed to evaluate the effectiveness and safety of Risdiplam and Nusinersen in patients with SMA types I–III. Hammersmith Functional Motor Scale-Expanded (HFMSE) was used for motor evaluation.

Results:

Results revealed that changes were significant after six months (p< 0.001). Improvements were compared between the two drugs, age groups, and SMA disease types, and no significant differences were found. No severe side effects were experienced and only a few patients reported headaches, and backaches following Nusinersen.

Conclusion:

both Risdiplam and Nusinersen led to significant improvements in motor function; however, based on cost-effectiveness considerations, we recommend Risdiplam.

Objectives:

Evaluating risk factors is essential in the context of hyperbilirubinemia and its clinical outcomes, particularly when attributed to its primary causes: maternal-fetal ABO and Rh incompatibility This study aims to determine the prevalence rate of predisposing factors and complications of hyperbilirubinemia in newborns with ABO blood group incompatibility, compared to those with Rh incompatibility.

Materials & Methods:

This is a descriptive-analytical study. The newborns with jaundice who were referred to Ghaem Teaching Hospital affiliated to the Mashhad University of Medical Sciences, Mashhad, Iran, from 2017 to 2019, divided into two groups of ABO incompatibility (n = 83) and Rh incompatibility (n = 81). The neurodevelopmental assessment was performed by Denver Developmental Screening Test II (DDST-II). Following that, a comparison was made between these two groups.

Results:

The mean age of the ABO incompatibility group was 5.84 days and the Rh incompatibility group was 4.91 days at admission time. The mean values of bilirubin level obtained at 28.26 ± 5.9 and 30.17 ± 8.19 in newborns with ABO and Rh incompatibility (P = 0.089). No significant difference was observed between the two groups regarding age, Apgar score, bilirubin level (P > 0.05), delivery type (χ2 = 1.56; P = 0.21), and gender (χ2 = 0.403; P = 0.52). Among the study population, 45.8% and 89.1% of the infants with Rh incompatibility and with ABO incompatibility had a near-normal neurological development in the first three years of life. A significant difference was found between the two groups regarding developmental status (χ2 = 25.13; P < 0.05).

Conclusion:

Despite similar bilirubin levels and treatment approaches, neonates with Rh incompatibility experienced significantly higher rates of developmental delay compared to those with ABO incompatibility. By age three, moderate to severe delays were more frequent in the Rh group, underscoring the greater neurodevelopmental risk associated with Rh-mediated hemolysis.

Objectives:

This study aimed to develop a structured, culturally responsive neurodevelopmental care framework for preterm infants during the first three months following the neonatal intensive care unit (NICU) discharge, based on research evidence and multidisciplinary expert consensus in Iran .

Materials & Methods:

A two-phase study was conducted. First, a scoping review (2000–2024) identified key domains and items for post-discharge care. Second, a panel of 24 multidisciplinary experts completed a two-round Delphi survey. Consensus was defined as 75% agreement or higher and a mean Likert score of 3.75 or above on a five-point scale. Quantitative and qualitative analyses were performed in accordance with Delphi study guidelines .

Results:

The finalized framework comprises 57 items categorized across eight primary domains. Expert agreement ranged from 87% to 95%, with mean Likert scores ranging from 4.42 to 4.65. Positioning and postural care showed the highest agreement (95% t, mean = 4.57). Experts emphasized continuity of care, parent-mediated interventions, and integration of developmental, emotional, and environmental components.

Conclusion:

This culturally adapted, family-centered framework provides structured post-discharge neurodevelopmental care for preterm infants. It promotes infants’ motor, sensory, cognitive, and emotional development while supporting parental empowerment and psychological well-being, potentially optimizing early neurodevelopmental outcomes in home-based care settings .

Neurocutaneous melanosis (NCM) and Dandy-Walker malformation (DWM) are both uncommon congenital condition, involving the central nervous system (CNS). Combined NCM with DWM have been reported in a few studies. Potential consequences, including hydrocephalus and CNS malignancies make these illnesses extremely challenging to diagnose and treat. Occurrences of NCM and DWM together, can result in progressive neurological consequences and patients with this condition seems to have really poor prognosis. This case report describes a 5-day-old girl who was born with several large pigmented cutaneous nevi all over her body. She was born at term to non-consanguineous parents who had no family history of similar conditions. Imaging studies, such as brain CT scan, showed cystic dilatation of the fourth ventricle, hypoplasia of the cerebellar vermis, enlargement of the posterior fossa, and possibly secondary hydrocephalus—all of which are indicative of DWM. A biopsy of the nevi confirmed NCM. The patient had no neurological symptoms or systemic complications at the time of presentation. The combination of NCM and DWM is extremely rare, with fewer than 40 reported cases so far. While the exact connection between these two conditions is not fully understood, their coexistence of these conditions might be linked to their shared embryonic origin. Both melanocytes and parts of the CNS come from the neural crest during early development. Since these conditions can have serious neurological and systemic effects, managing them requires multidisciplinary care because of potential consequences of these disorders.

Landau-Kleffner syndrome and related epilepsy-aphasia spectrum disorders are characterized by childhood-onset language regression, sleep-activated epileptiform activity, and frequently refractory seizures. This case report describe a boy with normal early development who developed progressive aphasia and non-motor seizures around age three, with electroencephalographic findings consistent with spike-wave activation during slow sleep, while neuroimaging and metabolic evaluations were normal. Standard antiseizure medications and repeated immunotherapy provided no sustained benefit. Genetic testing at age 12 identified a pathogenic heterozygous GRIN2A gain-of-function missense variant (p.T531M), guiding initiation of targeted therapy with memantine, and an NMDA receptor antagonist. Following memantine treatment, the patient showed marked improvement in speech and social interaction together with reduced sleep-related epileptiform discharges, although some deficits persisted. This case underscores the value of early genetic evaluation in refractory epilepsy-aphasia syndromes and supports the potential role of precision NMDA-modulating therapy in GRIN2A-associated epileptic encephalopathy.