Russian Journal of Child Neurology
Russian Journal of Child Neurology is a quarterly peer-reviewed journal that publishes articles addressing basic research and comprehensive management of patients with neurological disorders.
This is the official journal of Svt. Luka’s Institute of Child Neurology and Epilepsy.
Editor-in-Chief — Andrey Petrukhin Sergeyevich, MD, Professor of the Department of Neurology and Neurosurgery of the Medical Faculty of the Pirogov Russian National Research Medical University, full member of the International Association of Pediatric Neurologists, the European Academy of Epilepsy (EUREPA), the Royal Medical Society of Great Britain.
Russian Journal of Child Neurology is included in the List of leading peer-reviewed journals which provide publications on the results of masters’ and doctoral theses.
IF RusSCI = 1.15. H-Index: 11.
Main focuses of interest include: modern diagnostic methods (video- electroencephalographic monitoring, neuroimaging) and treatment of a wide range of neurological disorders in children (including innovative therapies for epilepsy), publications on the methods of classification, nosologic specificity of multiple types of epilepsy and effective treatment approaches (antiepileptic therapy, literature reviews and experience with the new generation of antiepileptic drugs, as well as preoperative examination in patients with epilepsy and surgical treatment).
In comparison with the traditional approach of highlighting common neurological disorders, Russian Journal of Child Neurology primarily covers rare and atypical neurological diseases. This is mainly due to the assessment of neurological signs in rare syndromes that allows to view ordinary neurological disorders from a different perspective, be critical in the questions of their diagnostics and treatment, move forward into in-depth understanding of neurology.
Both Russian neurologists, as well as international scientists are included in a collective work in this journal. It enables to provide a full view on current problems and achievements in pediatric neurology.
Target Audience: neurologists, epileptologists, neurophysiologists, neurosurgeons, functional diagnosticians (e.g. specialised in electroencephalography, video-electroencephalographic monitoring, polysomnography), experts in neuroimaging (e.g. magnetic resonance imaging, computed tomography), preoperative evaluation and surgical treatment of epilepsy, psychiatrists, pediatricians, general practitioners, specialists in the history of medicine.
Frequency: 4 issues per year.
Format: А4.
Volume: 60–80 pages.
Circulation: 2000 copies.
Disrtibution: addressed on the territory of the Russian Federation and CIS countries.
Index of subscription: in the “Press of Russia” catalogue — 88083.
Anyone can subscribe to the Journal in the site of the «ABV-press» Publishing house.
Information about types of advertising in the printed publications can be found in «Cooperate» section.
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Vol 21, No 2 (2026)
ORIGINAL REPORTS
Cognitive and emotional impairments in childhood absence epilepsy
Abstract
Background. Cognitive impairments in epilepsy develop under the influence of a complex interplay of interrelated factors, including age at disease onset, seizure frequency and duration, as well as the use of antiseizure medications. Their objective assessment is complicated by the heterogeneity of methodological approaches and the diversity of neuropsychological instruments employed an issue that is particularly relevant in pediatric populations.
Aim. To determine the nature and severity of cognitive and emotional-behavioral impairments in children and adolescents with childhood absence epilepsy (CAE) using age-appropriate psychodiagnostic instruments.
Materials and methods. A retrospective analysis of follow-up data from 70 patients with CAE was conducted. The cohort included children with active absence seizures, patients undergoing routine clinical follow-up, and individuals in sustained medication-induced remission. The study also included 34 healthy volunteers. The following psychodiagnostic methods were used: the Digit Span test, the Stroop test, and M.A. Panfilova’s “Cactus” graphic method in the 4–12 age subgroup; the Trail Making Test, the Achenbach System of Empirically Based Assessment, and the “Well-being, Activity, Mood” questionnaire in the 12–18 age subgroup.
Results. The identified cognitive impairments in children and adolescents with CAE across both age subgroups predominantly involved memory, attention, and executive thinking processes. In the emotional and personality domains, reduced levels of subjective well-being and activity were observed, along with difficulties in social adaptation and the presence of somatic complaints.
Conclusion. The presence of neuropsychological impairments in patients both during the active phase of the disease and during clinical remission indicates that CAE may be accompanied by disturbances in cognitive and emotional-volitional functioning, which may significantly reduce patients’ quality of life.
8-16
Lamotrigine (Sazar) in the treatment of epilepsy: seven years of experience in Svt. Luka’s Association of Medical Institutions for the Diagnosis, Treatment, and Rehabilitation of Nervous System Diseases and Epilepsy
Abstract
Aim. To assess the efficacy and tolerability of lamotrigine (Sazar; LLC “Alkaloid-Rus”, Republic of Macedonia) for various forms of epilepsy, based on long-term experience of Svt. Luka Association of Medical Institutions for the Diagnosis, Treatment, and Rehabilitation of Nervous System Diseases and Epilepsy (Svt. Luka’s Institute of Child Neurology and Epilepsy/Svt. Luka’s Institute of Child and Adult Neurology and Epilepsy).
Materials and methods. A retrospective cohort study was conducted based on the analysis of clinical data from 628 patients (aged 3 to 46 years; 595 children and 33 adults (20 women and 13 men); mean age 10.5 years; 319 males and 309 females) monitored at Svt. Luka Association of Medical Institutions for the Diagnosis, Treatment, and Rehabilitation of Nervous System Diseases and Epilepsy who received Sazar over a 7-year period (from June 2018 to September 2025).
Distribution by epilepsy types: structural and presumably structural focal epilepsy/focal epilepsy of unknown etiology (n = 496); genetic and presumably genetic epilepsies, as well as developmental and epileptic encephalopathies (n = 97); idiopathic generalized epilepsies (n = 35).
Sazar was administered as adjunctive therapy or monotherapy at a dose of 3–5 mg/kg/day, with gradual titration over 4–8 weeks to a target dose of 37.5–500.0 mg/day. Subsequently, the dose of Seizar was adjusted depending on the clinical effect, electroencephalography findings, and blood drug concentrations. Three adult female patients received Sazar as a single daily dose of 200 mg/day (at bedtime in 2 cases, and in the morning in 1 case). In all other cases, patients received Sazar twice daily.
Sazar was administered as monotherapy in 176 patients or as adjunctive treatment to other antiepileptic drugs in 452 patients (combination of Sazar with one antiepileptic drug occurred in 379 patients, and with two antiepileptic drugs in 73 patients). In five patients, valproate was added to Sazar, achieving a therapeutic effect without subsequent discontinuation of Sazar. In two patients with high treatment efficacy, a subsequent successful switch from polytherapy to Sazar monotherapy became possible. Twelve patients previously treated with another lamotrigine generic were switched to Sazar without any worsening of epilepsy course.
The follow-up period ranged from 6 months to more than 7 years. Patients followed up for at least 6 months were included in the analysis.
Results. Therapeutic remission was achieved in 306 out of 628 patients (48.72 %) receiving Sazar. Therapeutic remission with Sazar was achieved in 306 out of 628 patients (48.72 %). An additional 157 out of 628 patients (25 %) showed an at least 50 % reduction in seizure frequency; 165 patients had no therapeutic effect (26.3 %).
In 113 cases (17.9 %), Sazar was discontinued due to insufficient efficacy or loss of the initial robust response (after 6–12 months of treatment). Overall, a therapeutic effect (remission or an at least 50 % reduction in seizure frequency) was achieved in 463 out of 628 patients (73.7 %). Given the predominance of patients with drug-resistant forms of epilepsy in this study, this rate can be considered exceptionally high. Considering that the study population predominantly consisted of patients with drug-resistant forms of epilepsy, this rate can be considered exceptionally high.
Sazar demonstrated the highest efficacy in focal epilepsies (structural, presumably structural, structural-genetic, and that of unidentified etiology), as well as in idiopathic generalized epilepsy syndromes. Efficacy was lower in genetic generalized epilepsies and epileptic encephalopathies.
The majority of the patients (n = 573 (91.3 %)) demonstrated good tolerability of Sazar. Sazar-associated side effects were reported in 55 patients (8.76 %). In most cases, transient disturbances such as dizziness, headache, tremor, and nausea were observed, none of which required discontinuation of the medication. Allergic reactions predominantly manifested as a skin rash, which was reported in 37 out of 628 patients (5.8 %), within the first two months of therapy. Angioedema (Quincke’s edema) was reported in one patient. Discontinuation of Sazar due to poor tolerability was required in only 15 of 628 patients (2.4 %), with allergic reactions being the sole reason for treatment cessation. In the remaining cases, the skin rash was transient, did not require drug discontinuation, and its association with lamotrigine treatment remained unproven.
Two female patients of reproductive age started Sazar to reduce the valproate dose that caused severe menstrual disorders, weight gain, alopecia, and edema. Halving the dose of valproate (up to 750 mg/day) in combination with Sazar significantly improved treatment tolerance. One patient gave birth to a healthy baby when she was receiving monotherapy with Sazar at a dose of 350 mg/day.
Improvements in mood (in adult patients) as well as behavior, development, and learning (in children) during Sazar therapy were observed in 380 of 628 patients (60.5 %). In five cases, improvements in mental status during Sazar therapy enabled the discontinuation of antidepressants in adult patients with previously diagnosed depression. No negative effects of Sazar on memory, attention, mood, or behavior were observed in any of the cases, as reported by patients and parents, and in some instances, by a neuropsychologist.
The high efficacy and favorable tolerability of Sazar are confirmed by treatment adherence data, with 500 of 628 patients (79.6 %) continuing the drug throughout the follow-up period (range: 6 months to 7 years).
These data indicate that, with correct titration and close clinical monitoring, lamotrigine can be considered a well-tolerated drug. This is particularly significant for children with comorbid developmental and behavioral disorders, for whom sedative effects and cognitive slowing are of critical concern.
Conclusion. Our analysis demonstrated the high efficacy and favorable tolerability of Sazar in a large cohort, predominantly consisting of pediatric patients, with various forms of epilepsy.
17-45
Epilepsy in cerebral palsy: problems of antiepileptic therapy
Abstract
Epilepsy and cerebral palsy (CP) are among the most frequently co-occurring neurological disorders and are characterized by high comorbidity. This article provides a brief overview of the issue and presents clinical cases of patients with CP combined with focal epilepsy of childhood with structural brain changes and benign epileptiform discharges of childhood on electroencephalogram (FECSBC-BEDC) and infantile epileptic spasms syndrome. Vigabatrin is a first-line drug for the treatment of infantile epileptic spasms syndrome and can also be successfully used for focal epilepsy in patients with CP. Due to its GABA-mediated mechanism of action, vigabatrin has demonstrated the ability to reduce symptoms of spasticity in dystonia. Although the FECSBC-BEDC syndrome has been described in patients with CP, it has not yet been assigned a taxonomic position in the International Classification of Epilepsies and Epileptic Syndromes. Sulthiame has demonstrated efficacy and good tolerability in patients with FECSBC-BEDC.
46-58
Zonisamide in children with focal epileptic seizures: results of a prospective multicenter observational study (October 2025–June 2026)
Abstract
Background. Management of pediatric epilepsy remains a critical challenge due to the insufficient efficacy of first-line therapies, adverse effects, and constraints within current clinical guidelines.
Aim. To evaluate the efficacy of zonisamide and its impact on the frequency, severity, and types of seizures after 3 and 6 months of therapy. Additionally, to assess the safety and tolerability of zonisamide treatment, the incidence and nature of adverse events (AE), and its impact on body weight and cognitive functions using the pediatric EpiTrack test after 3 and 6 months of therapy.
Materials and methods. A multicenter study was conducted involving 35 physicians from various regions of the Russian Federation. The study enrolled 115 patients aged 6 to 17 years inclusive (mean age: 9.95 years), including 52 % boys, with a confirmed diagnosis of focal epilepsy. Zonisamide (Zonegran, JSC “Firma EUROSERVICE”, Russia) was prescribed according to the manufacturer’s instructions as adjunctive therapy for focal epilepsy with or without secondary generalization. Epileptic syndromes for which zonisamide was initiated included: self-limited focal epilepsies of childhood, structural focal epilepsies, structural variant of Lennox–Gastaut syndrome, and genetic epilepsies. Individual patients could exhibit a combination of multiple focal seizure types. Zonisamide was administered as add-on therapy at a mean daily dose of 4.6 mg / kg. After 3 months, 16 patients dropped out of the study due to non-compliance with the study protocol. During these first 3 months, no patients discontinued the study due to zonisamide withdrawal. A total of 99 patients continued the study. After the subsequent 3 months (i.e., 6 months from the baseline), an additional 5 patients dropped out; thus, 94 patients completed the study. Among these 5 remaining dropouts, only 2 patients discontinued due to zonisamide withdrawal, while the others dropped out due to protocol violations. Consequently, the baseline cohort consisted of 115 patients, with a total of 21 patients dropping out (19 due to protocol violations and 2 due to zonisamide discontinuation).
Results. Efficacy of zonisamide treatment was observed in 83 out of 94 patients. The highest rate of complete seizure control was recorded in the early adjunctive therapy group, where zonisamide was prescribed as the second add-on medication. Five patients developed AE that required treatment discontinuation. In the remaining patients, AE occurred during the titration period and were deemed acceptable (decreased body weight and appetite in overweight patients). Concurrently, a reduction in migraine frequency was reported in one patient, a decrease in tic severity in one patient, and an improvement in hyperactive behavior.
Conclusion. Zonisamide demonstrates high efficacy and favorable tolerability. The incidence of AE is comparable to that of other modern antiepileptic drugs.
59-71
REVIEWS AND LECTURES
Cognitive impairments in children with dyslexia
Abstract
This article presents a review of scientific publications dedicated to the role of genetic, neurobiological, psychological, and environmental factors in the etiology and pathogenesis of dyslexia. The paper outlines the results of neuropsychological and neurophysiological studies demonstrating impairment of certain higher mental functions in dyslexia, as well as the results of neuroimaging studies that have identified structural features of the brain characteristic of this disorder. Current understanding of the main neurocognitive deficits observed in dyslexia is described: impaired working memory, decreased information processing speed, and insufficient automation of new skills. The principles of dyslexia treatment and the potential use of Cereton in the combination therapy of this pathology are also discussed.
73-79
Epilepsy in patients with Down syndrome (literature review and clinical cases)
Abstract
An overview of the literature and clinical cases of epilepsy in Down syndrome (DS) is presented. Epilepsy in DS has a bimodal distribution with two peaks: before 5 years of age and after 40 years. In childhood, the most common epileptic syndrome is infantile epileptic spasms syndrome. First-line treatment includes vigabatrin and corticosteroids.
The second peak of epilepsy occurs after the age of 40 and is associated with the coexistence of epilepsy and Alzheimer’s disease, which is known as late-onset myoclonic epilepsy in DS, the seizure semiology of which is similar to that of juvenile myoclonic epilepsy.
Clinical cases of patients with infantile epileptic spasms syndrome and DS successfully treated with vigabatrin are presented. Comorbidities in DS include a high prevalence of obstructive sleep apnea syndrome, increased carbonic anhydrase type 2 activity, and the presence of myoclonic seizures similar to those in juvenile myoclonic epilepsy. These features support the use of sultiame as a pathogenetically justified drug for the treatment of epilepsy in patients with AD.
A clinical case of a patient with DS is presented, in whom sultiame demonstrated high efficacy and good tolerability, as well as a positive impact on development and behavior
80-96
Autoimmune encephalitis and demyelinating diseases: intersections
Abstract
This article examines the growing conceptual and clinical convergence between autoimmune encephalitis (AE) and demyelinating diseases (DD) of the central nervous system. The author emphasizes that the traditional boundaries between these disease groups are becoming increasingly blurred due to shared immunopathological mechanisms leading to autoimmune damage to the central nervous system. The article begins with clear definitions: AE are characterized by an immune attack on neuronal antigens (synaptic receptors, intracellular proteins), which clinically manifests as acute neuropsychiatric symptoms such as psychosis, seizures, cognitive impairment, and dyskinesias. Meanwhile, DD, a classic example of which is multiple sclerosis, primarily affect the myelin sheath, leading to focal neurological deficits (paresis, sensory disturbances, optic neuritis).
The focus is on the intersection points and overlap syndromes, where patients exhibit features of both disease groups simultaneously. Key examples include:
- myelin oligodendrocyte glycoprotein antibody-associated disease, which can manifest as both a demyelinating disease (e.g., optic neuritis) and an encephalitis with cortical lesions;
- anti-NMDAR encephalitis, which in rare cases can coexist with or precede multiple sclerosis;
- AQP4-positive neuromyelitis optica, severe forms of which, involving the brainstem or hypothalamus, can phenotypically mimic encephalitis.
The author provides a detailed comparative table, highlighting the differences and similarities between AE and DD in terms of parameters such as the primary immune target, leading symptoms, magnetic resonance imaging findings, laboratory markers, prognosis, and triggers.
The section on treatment notes that in the acute phase, therapy for both groups is similar and includes pulse corticosteroid therapy, plasmapheresis, and intravenous immunoglobulin aimed at suppressing the autoimmune response. However, maintenance therapy differs fundamentally: in AE, the focus is on identifying and eliminating the trigger (e.g., tumor) and using second-line drugs (rituximab), whereas in DD, the primary goal is long-term relapse prevention with disease-modifying therapies specific to each disease (e.g., ocrelizumab in multiple sclerosis, eculizumab in AQP4-positive neuromyelitis optica).
The paper concludes that AE and DD represent a continuum of immune-mediated central nervous system lesions. The importance of comprehensive diagnostics using expanded antibody panels and neuroimaging methods to identify overlap syndromes is emphasized, as early and adequate treatment is critical to improving prognosis and minimizing irreversible damage. Future research should be aimed at developing personalized therapeutic approaches.
97-102
Practical aspects of intracerebroventricular infusions of cerliponase alfa in patients with neuronal ceroid lipofuscinosis type 2
Abstract
Neuronal ceroid lipofuscinosis type 2 (CLN2 disease) is a rare, autosomal recessive neurodegenerative disorder caused by mutations in the TPP1 gene, which lead to a decrease or complete loss of lysosomal tripeptidyl peptidase 1 enzyme activity. The classic form of CLN2 disease typically manifests between the ages of 2 and 4 years. Over time, there is a rapid and predictably severe progression of intellectual and motor deficits, loss of visual acuity, and an increased frequency of epileptic seizures refractory to antiepileptic therapy. In its natural course, the classic form of CLN2 disease leads to severe disability by 6–7 years of age and death on average during the 10th year of life. Currently, pathogenetic enzyme replacement therapy has been developed only for CLN2 disease, utilizing the drug cerliponase alfa. In Russia, children with CLN2 disease are provided with cerliponase alfa through funding from the “Circle of Kindness”, a state fund supporting children with severe life-threatening and chronic diseases, including rare (orphan) conditions.
Cerliponase alfa cannot cross the blood-brain barrier and must be administered directly into the cerebrospinal fluid via intracerebroventricular (ICV) infusion through a surgically implanted reservoir and ventricular catheter. Infectious complications can result in missed ICV infusions, which, in turn, may adversely affect the efficacy of enzyme replacement therapy. To reduce the risk of infectious complications, strict adherence to aseptic rules is essential during patient preparation, as well as during and after the ICV infusion.
These practical guidelines for performing ICV infusions of enzyme replacement therapy in patients with CLN2 disease have been developed to implement measures in clinical practice aimed at minimizing the risk of infectious complications and damage to ICV infusion devices.
103-114
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