Introduction. Epileptic spasms (ES) are a special type of epileptic seizures, accompanied by hypsarrhythmia on the interictal electroencephalogram (EEG) and regression of psychomotor development. ES are the most common cause of epileptic encephalopathy in infancy.

Materials and methods. We examined two hundred three  children with the age of onset of epileptic spasms up to 2 years.  The patient were selected in retrospective group including  180 children (boys — 95/180 (52.7%), girls — 85/180 (47.3%)) (χ²=0.9, p=0.3428) and  prospective group consisted of  23 children (boys — 13/23 (56.5%), girls — 10/23 (43.5%) (χ² = 0.3478, p = 0.5553.

Results. The present study demonstrates the need for sleep electroencephalogram in children with developmental delay or regression. Our work confirms  in general, antiepileptic drugs, with the exception of vigabatrin in children with tuberous sclerosis-associated epilepsy, to show low efficacy in the group of children without tuberous sclerosis as seizures were stopped in 14.3% of cases. The use of methylprednisolone (the regimen includes pulse therapy followed by prolonged oral administration of the drug) makes it possible to achieve the cessation of seizures and the disappearance of hypsarrhythmia in 69.5% of patients. Methylprednisolone is a fairly safe and well tolerated hormone. The study did not record any life-threatening side effects among the prospective and retrospective groups.The treatment regimen using methylprednisolone shows equal efficacy with the regimen using tetracosactide. 

Conclusion. The results of the study demonstrate the need for early corticosteroid therapy in ES children. 

Contribution:
Okhapkina T.G. — concept, writing text;
Belousova E.D. — concept, writing text;
Kalmykova G.V. — text editing.
All co-authors are responsible for the integrity of all parts of the manuscript and approval of its final version.

Acknowledgements. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: November 5, 2022
Accepted:  December 5, 2022
Published: January 15, 2023

Botulinum therapy has been used to treat increased muscle tone and spasticity in cerebral palsy (CP) over almost 30 years. Despite this, every year a large number of published scientific articles present new clinical studies devoted to various aspects of the use of botulinum toxin type A (BTA) products in children. The article discusses in detail the results of three major international randomized clinical trials concerning determination the efficacy and safety of the product Xeomin (IncobotulinumtoxinA) in spastic forms of cerebral palsy. All these studies were performed in accordance with the criteria of evidence–based medicine — randomized, prospective, multicenter, comparative, and longitudinal with a large number of patients, clear inclusion and exclusion criteria. The TIM (Treatment with incobotulinumtoxinA in Movement) study demonstrated the effectiveness of the treatment of spasticity of the lower extremities muscles when using different doses of IncobotulinumtoxinA (8, 6 and 2 units/kg of body weight to correct one pathological pattern) in a double-blind study. The TIMO (Treatment with IncobotulinumtoxinA in Movement Open Label) study showed the efficacy and safety of high doses of IncobotulinumtoxinA (total doses of 16-20 units/kg of body weight) in the treatment of spasticity of the lower and upper extremities with prolonged use. The XARA study (IncobotulinumtoxinA in Arm Treatment in Cerebral Palsy) presented data on the high efficacy of IncobotulinumtoxinA when using a multi-level approach for the treatment of lower and upper limb spasticity in CP children and adolescents, which reflects the real clinical needs of a large number of patients. In each of these studies, the safety of the use of IncobotulinumtoxinA was also evaluated. Good tolerability of therapy was demonstrated (84.1% of patients completed all visits in total according to three studies — TIM, TIMO and XARA) and its safety (the frequency of adverse events associated with therapy was observed in less than 2% of cases). Treatment with IncobotulinumtoxinA was characterized by the lack of an immunologic response — Xeomin as a starting therapy provides a stable effect without fading associated with the development of neutralizing antibodies.

Contribution:
A.L. Kurenkov — concept and design of the study;
A.L. Kurenkov, B.I. Bursagova — writing the text;
A.L. Kurenkov, A.R. Artemenko — editing.
All authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgements. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: November 17, 2022
Accepted:  December 5, 2022
Published: January 15, 2023

Intraneral perineurioma (IP) is a rare and little-studied benign neoplasm of peripheral nerves in children and young adults.  The clinical picture is usually a slowly progressive mononeuropathy, but plexuses with a predominance of motor deficits may also be involved.  The sciatic nerve and its branches are most often affected.  It is difficult to diagnose and requires a large amount of research to identify and confirm the diagnosis. Today, non-invasive neuroimaging methods such as magnetic resonance imaging (MRI) of nerve trunks and ultrasound of peripheral nerves are widely used for diagnosis, which are and allow getting ahead of the size and location of the neoplasm. MRI and ultrasound of the peripheral nerves reveal a focal fusiform enlargement of the nerve within one segment of the limb with an increase in the intensity of the MR signal from the IP.  These neoplasms are considered rare, but recent advances in MRI and ultrasound diagnostics allow them to be detected at an early stage. MRI also helps distinguish IP from other peripheral nerve neoplasms.  Confirmation of the diagnosis is based on histological examination of the altered nerve trunk.  To date, there is no generally accepted strategy for the management of IP patients.  Both conservative and surgical treatments are used.  Neurolysis and nerve decompression improve neurological deficits in half of IP patients.  IP does not recur after surgical treatment and does not become malignant during long-term follow-up of the patient.

Contribution:
Druzhinina E.S. — review of reports on the topic а the article, writing the text;
Druzhinin D.S. — writing the text, checking critical intellectual content;
Zavadenko N.N. — writing the text.
All co-authors are responsible for the  integrity of all parts of the manuscript and approval of its final version.

Acknowledgements. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: November 02, 2022
Accepted:  December 2, 2022
Published: January 15, 2023

Nervous Developmental Disorder with Involuntary Movements (NEDIM) (OMIM 617493) is a rare movement disorder in children on the spectrum of GNAO1-associated movement disorders. With NEDIM, movement disorders appear in early childhood, progress and lead to disability. The disease is caused by pathogenic heterozygous variants in the GNAO1 gene and has an autosomal dominant mode of inheritance. The epidemiology of NEDIM has not yet been established. Clinical symptoms are extensive, ranging from severe motor and cognitive impairment with self-injurious behaviour and seizures to a mild phenotype of movement disorders without mental retardation and seizures. Some patients develop epilepsy. Hyperkinetic syndrome in most children is manifested with chorea, athetosis, dystonia, and ballism, affecting the muscles of the body, limbs and face. According to MRI, in some patients, gradually progressive atrophy of the brain substance is visualized. Currently, the disease has no developed pathogenetic methods of therapy. Treatment is symptomatic, including various drug regimens to reduce the severity of movement disorders and seizures. Management of nutrition of the patient and the prevention of secondary complications of movement disorders are also important. In foreign sources there is described the experience of using topiramate and teterabenazine, as well as deep brain stimulation (DBS), which demonstrate a good effect in the form of a significant reduction in the frequency of dystonic storms and the severity of motor disorders. The article presents a clinical case of diagnosis and treatment of a child with this disease, and also current trends in therapy.

Compliance with ethical standards. All photographs are published with the written consent of the child’s parents.

Contributions:
Kuzenkova L.M. — concept, writing, editing  the text;
Lyalina A.A. — writing, editing the text;
Zyryanova O.I. — writing, editing the text;
Yarosh M.A. — writing, editing the text;
Savostyanov K.V. — editing the text, molecular genetic examination;
Kanivets I.V. — the molecular genetic examination.
All co-authors are responsible for the integrity of all parts of the manuscript and approval of its final version.

Acknowledgements. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: November 11, 2022
Accepted: December 2, 2022
Published: January 15, 2023

Levamizole is an imidazole derivative used in the treatment of various types of cancer, dermatological diseases and parasitoses. The drug has immunomodulatory properties due to stimulating the activity of macrophages, neutrophils, monocytes and T-lymphocytes. Common side effects are gastrointestinal disorders, skin manifestations and hematological disorders. There is also a wide range of neurological side effects, such as headache, dizziness, vomiting, aphasia, blurred vision, diplopia, weakness, which can be observed in 1.3–5.0% of patients. Multifocal levamizole-induced leukoencephalopathy is one of the most striking neurological side effects of levamizole. MRI of the brain in this pathology reveals numerous foci of demyelination, located mainly periventrically, in the white matter of the cerebral hemispheres, cerebellum, brain stem, corpus callosum, basal ganglia. The treatment with corticosteroids showed the significant improvement in the condition, with almost complete regression of neurological symptoms [8]. Although the symptoms are most often manifested as subacute, over 2–8 weeks of drug use, there were reported cases  with clinical manifestations developed both 1 day and months after levamizole administration. It should be noted that the neurotoxic effects of levamizole can manifest independently on the dose, even after a single application of 50 mg, which indicates that its toxic effect is a manifestation of idiosyncrasy. 

The literature provides a sufficient number of descriptions of clinical manifestations, MRI features and therapy of drug complications of levamizole use in adults. However, observations in pediatric practice are few. We would like to present clinical, laboratory data, imaging results, information on the treatment and follow-up of a 17-year girl who developed levamizole-induced inflammatory leukoencephalopathy and autoimmune hepatitis after self-administration of levamizole for the prevention of helminthic invasion. Leukoencephalopathy caused by levamizole should be taken into account in the differential diagnosis of demyelinating diseases, including acute multiple encephalomyelitis and multiple sclerosis, and the neurotoxic effects of the drug should also be taken into account for the timely initiation of therapy.

Contribution:
Globa O.V. — concept, text writing, text editing
Kuzenkova L.M. — concept, text editing
Firumyants A.I. — concept, text editing
Abdullaeva L.M. — concept, text editing
All co-authors are responsible for the integrity of all parts of the manuscript and approval of its final version.

Acknowledgements. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: November 14, 2022
Accepted:  December 2, 2022
Published: January 15, 2023