Iranian Journal of Child Neurology

Objectives:

To describe the historical development of child neurology in Iran, from early Persian–Islamic medical concepts of neurological disease to contemporary subspecialty practice, education, research, and service delivery.

Materials & Methods:

This narrative historical review synthesized accessible peer-reviewed publications, institutional histories, university and hospital documents, professional-society resources, and publicly available records concerning Persian medical scholarship, medical education, pediatric hospitals, subspecialty training, neonatal care, epilepsy services, newborn screening, and neurogenetics in Iran. When historical dates or attributions were inconsistent, they were interpreted cautiously and reported as approximate or unspecified.

Results:

Early Persian and Islamic medical traditions, including the works associated with Rhazes, Avicenna, and Jorjani, documented observations relevant to seizures, paralysis, developmental disorders, and cranial nerve function, although child neurology was not yet a distinct specialty. Modern development was facilitated by nineteenth-century educational reform, especially Dar al-Funun, followed by the establishment of pediatric teaching and referral hospitals, including Bahrami Children’s Hospital, Children’s Medical Center, and Mofid Children’s Hospital. Integration of medical education and health services after 1985 strengthened specialty training and referral systems. Contemporary child neurology includes structured two-year subspecialty training, pediatric clinical neurophysiology and epilepsy fellowship programs, long-term video-electroencephalography monitoring, epilepsy monitoring units, neonatal intensive care expansion, newborn screening for congenital hypothyroidism and phenylketonuria, neurogenetic resources such as Iranome, dedicated research centers, and an indexed specialty journal. Persistent challenges include uneven regional access, economic constraints, sanctions-related procurement barriers, and workforce migration.

Conclusion:

Child neurology in Iran has evolved from descriptive neurological knowledge embedded in Persian–Islamic medicine into a hospital-based, research-oriented pediatric subspecialty. Future progress depends on retaining the specialist workforce, strengthening provincial referral pathways, expanding registries and genomic capacity, and improving resilience of clinical and research infrastructure

Objectives:

The varying results of comparing different doses of tenecteplase (TNK) with alteplase (ALT) make it challenging to establish clear guidelines for managing acute ischemic stroke (AIS). This meta-analysis aims to compare the benefits and risks of tenecteplase 0.25 mg/kg versus ALT.

Materials & Methods:

This study is a comprehensive search of multiple databases to identify randomized controlled trials (RCTs) comparing the effects of TNK 0.25mg/kg and ALT in patients with AIS, until June 10, 2024. We followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Pooled analyses were performed using Stata V.17.

Results:

The present study analyzed eight trials involving 3647 patients with AIS who were treated with TNK and ALT. Compared to alteplase, tenecteplase (TNK 0.25 mg/kg) demonstrated a significant improvement in excellent recovery at three months (RR: 1.079, 95% CI 1.00–1.15). TNK exhibited non-inferiority to ALT regarding complete recanalization (RR: 1.47, 95% CI 0.87–2.50), combined complete/partial recanalization (RR: 1.20, 95% CI 0.86–1.68), early neurological improvement (RR: 1.04, 95% CI 0.83–1.29), and good recovery (RR: 1.02, 95% CI 0.90–1.15). While these findings trended in favor of TNK 0.25 mg/kg, the differences did not reach statistical significance. Furthermore, no significant differences were observed between TNK and ALT regarding poor recovery (RR: 0.87, 95% CI 0.67–1.13), parenchymal hematoma (RR: 0.78, 95% CI 0.21–2.81), intracerebral hemorrhage (ICH) (RR: 0.91, 95% CI 0.77–1.08), ICH symptoms (RR: 1.05, 95% CI 0.71–1.56), or 90-day mortality (RR: 1.00, 95% CI 0.83–1.19)

Conclusion:

Intravenous thrombolysis with TNK 0.25mg/kg in patients with AIS was shown to be as effective and safe as ALT, demonstrating non-inferiority. TNK showed significant superiority in terms of excellent recovery when compared to ALT. However, given the limitations of the present study, further RCTs with larger populations and more specific objectives are needed.

Objectives:

We aimed to assess if the claustrum is involved in seizure networks in humans with epilepsy.

Materials & Methods:

We identified eight patients submitted for presurgical evaluation with stereoelectroencephalography (SEEG) recordings from the claustrum from 2016-2022. Demographic, paraclinical, and outcome data (Engel Classification) was collected from retrospective chart review. We analyzed the ictal and interictal pattern as well as high frequency oscillations (HFOs) in the claustrum.

Results:

The ictal onset zone was in the temporal lobe in 87% (n=7) of patients and the cingulate cortex in 12% (n=1). A total of 25 seizures were detected and 96% (n=24) involved the claustrum cortex. Of those seizures involving the claustrum, 88% (n=21) were involved before the rest of the electrodes. Two ictal onset patterns were identified: slow potential shift with HFOs were found in 46% (n=11) of seizures and rhythmic spikes <13 hz were found in 54% of seizures (n=13). The ictal pattern in the claustrum was synchronous with the ictal onset zone in 41% (n=10) seizures, however, in four patients with status epilepticus, the claustrum was noted to stop discharging just before ictal onset. The interictal pattern included high-amplitude slow waves with or without HFOs. HFOs were identified in the claustrum in 83% (n=20) of post-ictal periods. All patients had poor outcomes following surgical resection (Engel Class III-IV).

Conclusions:

Our study suggests that the claustrum is often involved during seizures arising from the temporal lobe and cingulate cortexes and could play a role in modulation of the epileptogenic network

Objectives:

The classification of status epilepticus (SE) has undergone several revisions, with the most recent update published by the International League Against Epilepsy (ILAE) in 2015. However, in seizures lasting longer than 10 minutes, there is still no consensus on optimal drug selection.

Materials & Methods:

This retrospective study reviewed the medical records of patients diagnosed with SE and followed in the Pediatric Intensive Care Unit, Pediatric Neurology, and Pediatric Emergency Unit at Erciyes University Faculty of Medicine.

Results:

A total of 64 patients were included. Sequential therapy with levetiracetam following phenytoin proved to be an effective treatment strategy prior to escalation to third-line agents. In our clinic, rapid MRI protocols, including axial T1, axial T2, sagittal T2, and diffusion-weighted sequences, were preferred for emergency neuroimaging. These rapid protocols were both practical and effective in the acute management of SE.

Conclusions:

Rapid brain MRI techniques are easy to apply and provide valuable diagnostic insights into the etiology of SE. Sequential administration of second-line drugs may reduce the side-effect profile and optimize seizure control in children with SE.

Objectives:

Children with Cerebral Palsy (CP) frequently encounter motor limitations that restrict access to in-person rehabilitation services. This study examined the feasibility and preliminary consequences of Telehealth-Delivered Occupational Performance Coaching (Tele-OPC) in improving occupational performance, functional mobility, and parental anxiety. Materials & Methods:

A single-case experimental design was employed, involving five children (aged 3–6 years; GMFCS levels II–III) and their parents in an outpatient rehabilitation clinic in Tehran, Iran. Participants underwent a 12-week intervention consisting of weekly Tele-OPC sessions conducted via Google Meet. Outcomes were assessed using the Canadian Occupational Performance Measure (COPM), the Gross Motor Function Measure-88 (GMFM-88), and the Beck Anxiety Inventory (BAI) at baseline, intervention, and follow-up. Fidelity was monitored using the Occupational Performance Coaching-Fidelity Measure (OPC-FM). Data were analyzed using visual trend analysis and Cohen’s d effect sizes.

Results:

OPC-FM scores increased from 46.59 at session one to 69.23 at session twelve. All five participants achieved large improvements in occupational performance and satisfaction on the COPM, as well as in functional mobility on the GMFM-88, with effect sizes exceeding 2.67. Four parents demonstrated a significant effect size in their anxiety levels, and one had a moderate improvement, with Cohen’s d ranging from 0.87 up to 2.67. When you look at the graphs, the baseline levels stayed steady, and improvements stuck around even later. The PND analysis had mostly perfect non-overlap between different phases—almost 100%—except for one area (COPM satisfaction), where it was 67%.

Conclusion:

Tele-OPC appears feasible and may improve occupational performance, mobility, and parental anxiety. More extensive studies with extended follow-up are recommended

Objectives:

In resource-limited settings where the measurement of biomarkers of febrile seizures (FS) is difficult to carry out, it is essential to know the feasible factors associated with FS.

Materials & Methods:

This hospital-based case-control study was conducted based on 340 medical records of children aged six to 60 months who were hospitalized at the children’s ward of two hospitals in Indonesia, from 2019 to 2020. Data were analyzed using Mann-Whitney U, Chi-square tests, and logistic regression.

Results:

The case group had a higher proportion of males (59.4%) and a significantly younger median age of 24.3 months than the control group of 30.3 months (p = 0,003). Moreover, the case group had a significantly lower median hemoglobin level of 11.2 g/dL than the control group of 11.5 g/dL (p = 0.022). Each month, increasing age was associated with a reduced likelihood of exhibiting FS of 0.980-fold with a 95% CI (0.966, 0.995). Moreover, children with anemia were 2.350 times more likely to exhibit FS than those without anemia, 95% CI (1.399, 3.947).

Conclusion:

In low-resource settings. Younger age and reduced hemoglobin levels are useful indicators of febrile seizure risk in children under five, highlighting the importance of early detection and parental education

Abstract

Objectives:

Given the significance of sleep and physical activity (PA) as modifiable factors influencing overall health and development in childhood, this study aims to examine the association between sleep problems and PA levels in children with cerebral palsy (CP) and to compare these findings with typically developing (TD) children.

Materials & Methods:

This cross-sectional observational study included 40 children aged 6–12 years. The participant group comprised 20 children diagnosed with CP, exhibiting Gross Motor Function Classification System (GMFCS) levels I–II, and 20 age- and sex-matched TD peers. PA was measured using MOX accelerometers worn continuously for 24 hours on weekdays. Sleep habits were assessed using the Children’s Sleep Habits Questionnaire (CSHQ) and the Epworth Sleepiness Scale (ESS), both completed by parents.

Results:

Children with CP demonstrated significantly lower levels of light, moderate, and vigorous PA compared to children with TD (p < 0.05). No significant difference was observed in total sleep habit scores between the groups (p = 0.423). A significant positive correlation was found between moderate PA and sleep habit in the CP group (r = 0.49, p = 0.03). Among children with TD, those with sleep problems engaged in significantly less vigorous PA than those without (p = 0.044).

Conclusion:

Children with CP exhibit lower PA levels than their TD peers. In this population, moderate-intensity PA appears to have a positive effect on sleep problems. Promoting moderate PA may contribute to improved sleep and overall health in children with CP. These findings highlight the importance of integrated PA and sleep health strategies in pediatric rehabilitation

Abstract

Objectives:

Different drugs have been proposed to control migraine headaches in children, which have different effectiveness. This study aims to compare the effectiveness of Pregabalin and Cinnarizine in the preventive treatment of children with migraine headaches.

Materials & Methods:

In this randomized, double-blind clinical trial, 74 children aged 6 to 17 years with migraine headaches were included. Patients were randomly assigned to Pregabalin (n = 37) and Cinnarizine (n = 37) groups. In the Pregabalin group, one oral capsule (50 or 75 mg) was prescribed at bedtime, and in the Cinnarizine group, patients received oral tablets (25 or 50 mg) at bedtime. The frequency of migraine headaches, the severity of migraine headaches, and the severity of migraine disability were measured and recorded at the beginning of the study, 4, 8, and 12 weeks after initiation of intervention, through the patient’s notes, pain scale (VAS), and PEDMIDAS, respectively. The data were analyzed using SPSS version 25, and the significance level for all statistical tests was set at < 0.05.

Results:

Three out of 74 patients did not continue follow-up for unknown reasons, and later it was found that all three were in the pregabalin group. Of 71 subjects, 38 (53.5%) were boys: 17 (50%) in the Pregabalin group and 21 (56.8%) in the Cinnarizine group. No significant difference was observed between the two groups regarding gender (P-value = 0.0569). The mean and standard deviation of the patients’ ages were 10.24 and 2.51, respectively, and their age range was 6 to 17 years. Headache frequency decreased by 72% in the Cinnarizine group and 58% in the Pregabalin group. Headache intensity decreased by 63% and 35% in the Cinnarizine and Pregabalin groups, respectively. Furthermore, the reduction rate of disability caused by migraine attacks was 89% in the Cinnarizine group and 73% in the Pregabalin group.

Conclusion:

The findings revealed that both drugs reduced migraine headaches, but Cinnarizine was more effective than Pregabalin in controlling children’s migraine headaches, with this improvement sustained through the third month.

Tuberculosis (TB) remains a major health challenge in high-burden countries, with children particularly vulnerable to severe extrapulmonary disease. Intramedullary spinal TB is exceptionally rare and may mimic neoplasms, often resulting in delayed diagnosis and poor outcomes. This study aims to report a complex pediatric case of multisystem TB with intramedullary spinal involvement initially misdiagnosed as a neoplasm and to highlight the devastating consequences of delayed recognition. A 16-year-old boy from a low socioeconomic background presented with progressive bilateral lower-limb weakness, visual impairment, and systemic features. MRI demonstrated an intramedullary mass at T5–T6 with syringomyelia, para-aortic lymphadenopathy, and hepatosplenomegaly, initially suspected to be a spinal tumor. During hospitalization, he developed hydrocephalus, seizures, and respiratory failure. He underwent ventriculoperitoneal shunting and excision of the spinal lesion. Histopathology confirmed intramedullary spinal TB. Despite maximal supportive management, he deteriorated and ultimately progressed to brainstem death due to multisystem complications. This case emphasizes the need to consider TB in the differential diagnosis of spinal cord masses in children from endemic regions, particularly when ocular or systemic involvement is present. Early recognition and prompt initiation of anti-tuberculosis therapy are essential to prevent irreversible morbidity and mortality.

Cerebral malaria (CM) is a severe encephalopathy typically caused by Plasmodium falciparum (P. falciparum), presenting with altered consciousness, confusion, psychomotor agitation, and/or seizures. Diagnostic criteria require the laboratory confirmation of P. falciparum infection, alongside the systematic exclusion of alternative etiologies for encephalopathy. However, rare cases implicating Plasmodium vivax (P. vivax) as the causative agent have been reported. Pakistan is endemic for P. vivax, generally considered a benign cause of malaria, and encephalopathy is almost never encountered in clinical practice. This study presents a rare case of CM in a 16-year-old boy caused solely by P. vivax, confirmed on thick and thin peripheral smears showing rings and trophozoites. The patient was started on intravenous artesunate, to which he responded well, and was discharged on primaquine for radical cure. This case highlights the need for clinical vigilance, timely intervention, and recognition of P. vivax as a potential cause of CM, particularly in endemic regions.

Pompe disease (glycogen storage disease type II) is a rare autosomal recessive lysosomal storage disorder caused by GAA gene mutations, leading to acid α-glucosidase deficiency and glycogen accumulation in multiple tissues. Late-Onset Pompe Disease (LOPD) typically manifests after infancy without significant cardiac involvement. The present study reports a 16-year-old Iranian girl of Baloch ethnicity, born to consanguineous parents, who presented at age 11 with progressive proximal muscle weakness, waddling gait, scoliosis, dysphagia, dyspnea, and mild conductive hearing loss. Developmental history revealed delayed walking, recurrent respiratory infections, and echocardiographic evidence of intraventricular septal hypertrophy. Dried Blood Spot (DBS) assay showed markedly reduced GAA activity at pH 3.8 (0.42 nmol/spot/21 h; reference: 1.5–10). Whole-exome sequencing identified a homozygous missense variant c.2015G > A (p.Arg672Gln) in exon 14 of GAA, classified as likely pathogenic by ACMG criteria. Sanger sequencing confirmed segregation. Enzyme replacement therapy with alglucosidase alpha (20 mg/kg biweekly) led to stabilization of disease progression and improvement in motor function over five years. While LOPD usually lacks early cardiac manifestations, our patient exhibited septal hypertrophy and probable cardiomegaly in infancy, suggesting a broader phenotypic range for the p.Arg672Gln mutation. The studied case underlines the importance of considering Pompe disease in patients with unexplained muscle weakness, even when cardiac involvement is present early. Early diagnosis through biochemical and molecular testing, particularly in high-consanguinity regions, allows timely initiation of ERT, potentially improving long-term outcomes in LOPD.