[Diagnosis, pathogenesis and treatment of attention deficiency syndrome]. / Diagnostika, patogenez i lechenie sindroma defitsita vnimaniya.

Attention deficit hyperactivity disorder is the dominant form of cognitive and behavioral dysfunctions in childhood, the disease has a chronic course and manifests in adolescents and adults in 50-80% of cases. An adequate diagnosis is the testing of parents and teachers using the Conners questionnaire in two stages, the second is mandatory after 6 months to confirm the persistence of symptoms over time. The pathogenesis is based on violations of mediation of dopamine and norepinephrine in the fronto-striate-thalamic system, responsible for the function of constant attention, caused by molecular genetic mechanisms. Atomoxetine (Cognitera) in combination with pedagogical and psychological methods of correction seems to be an adequate drug for many months of use, taking into account international and Russian experience of use.

[Epileptic syndromes associated with focal clonic seizures]. / Epilepticheskie sindromy, assotsiirovannye s fokal'nymi klonicheskimi pristupami.

OBJECTIVE: To study clinical, electroencephalographic and neuroimaging features in children with epileptic syndromes associated with focal clonic seizures (FCS). MATERIAL AND METHODS: We examined 1258 patients with various forms of epilepsy with the onset of seizures from the first day of life to 18 years. RESULTS: FCS was identified in 263 patients (20.9%). FCS were included in the structure of 13 different epileptic syndromes: Rolandic epilepsy (28.1%), structural focal epilepsy (27.5%), structural focal epilepsy associated with benign epileptiform discharges of childhood (SFE-BEDC) (20.6%), focal epilepsy of unknown etiology (7.5%), epilepsia partialis continua (4.6%), pseudo-Lennox syndrome (3.4%), ESES syndrome (2.7%), Landau-Kleffner syndrome (1.5%), Dravet syndrome (1.1%), benign occipital epilepsy (1.1%), benign focal epilepsy in infancy (0.8%), MISF syndrome (0.8%), cognitive epileptiform disintegration (0.8%). In 50% of cases, epilepsy associated with FCS debuts before the age of 5 years (from 1 month to 18 years, average age 4.26±3.9). CONCLUSION: The groups of syndromes associated with FCS have different prognosis for remission of seizures. Prognostic predictors of seizure remission are: epileptic syndromes associated with BEDC, the presence of periventricular leukomalacia. A severe prognosis for the course of epilepsy is associated with local structural changes in the neocortex. Despite a favorable prognosis for seizures, continued diffuse interictal epileptiform activity with BEDC on the electroencephalogram is a predictor of the onset of cognitive impairment in children.

[Development of speech disorders in children]. / Narusheniya razvitiya rechi u detei.

The function of the formation of speech skills is decisive in the development of a child in communication and assimilation of information. Early pediatric diagnosis should become a routine practice of monitoring children from infancy to school age for the timely correction of speech disorders and associated dysgraphia and dyslexia. According to the ICD-10 classification, speech developmental disorders are divided into the following options: articulation impairment, delay in expressive and impressive speech. Articulation disorders, stuttering, impaired expressive speech are the most common and most favorable prognosis for speech therapy interventions. The data on the effectiveness of disorders of the receptive (impressive) language are ambiguous, since there is a high likelihood of a combination with neurogenetic diseases and autism. The development of speech therapy programs will make it possible to broader coverage of those in need of treatment, including complex course therapy with neuropeptides (cortexin).

[Alternating hemiplegia with epilepsy]. / Al'terniruyushchaya gemiplegiya, assotsiirovannaya s epilepsiei.

A clinical case of a genetically confirmed diagnosis of alternating hemiplegia associated with epilepsy is presented. The combination of two types of seizures in a child made it difficult to make a diagnosis. The result of video-EEG monitoring made it possible to understand that a child showed both epileptic seizures and non-epileptic seizures simultaneously with different periodicities. The mutation in the ATP1A3 gene was verified with genome-wide sequencing and targeted therapy was prescribed in a timely manner. As a result, both types of seizures stopped after treatment.

[Electromyography in childhood patients with tic hyperkinesis and Tourette's syndrome]. / Elektromiografiya u bol'nykh detskogo vozrasta s tikoznymi giperkinezami i sindromom Turetta.

OBJECTIVE: To determine the electromyographic patterns of motor tics in children. MATERIAL AND METHODS: 130 patients were examined, which were divided into 3 groups: the 1st group included 21 patients with motor tics; the 2nd group included 87 patients with motor-vocal tics; the 3rd group included 22 patients with Tourette's syndrome (ST). The control group consisted of 35 healthy children. Clinical methods for assessing the severity and semiotics of tics were used, including the YGTSS scale, hyperkinesis counting in 20 minutes, cutaneous electromyography (EMG) in rest and exercise modes with turn-amplitude analysis. RESULTS: The following EMG patterns of tic hyperkinesis in the resting mode were found: 1) the amplitude of the background activity exceeds the values in the control group (p<0.05); 2) in case of clonic tics, a high-amplitude volley activity (VA) arises simultaneously in the form of spindles with a duration of 10-30 ms with an amplitude of 500-2500 µV; 3) with dystonic tics, volleys in the form of rhombuses with a duration of 100-1000 ms and an amplitude of 1000-3000 µV; 4) volley-slow wave had a duration of 307.2±109.6 ms and an amplitude of 540.3±238.6 µV. In the mode of physical activity, VA increases, which is maximally expressed in amplitude on the circular muscle of the eye and the superficial flexor of the fingers in patients with CT (p<0.05). Analysis of correlations established an inverse relationship between the amplitude of VA and the number of hyperkinesis in 20 minutes. CONCLUSION: Qualitative and amplitude-time analysis of EMG makes it possible to objectify the disease, to isolate patients with CT from chronic motor-vocal tics and to model the prognosis. Probably, the tic can act as a compensatory manifestation of excessive impulsation of cortical-subcortical genesis.

[Tics and Tourette's syndrome in children]. / Tiki i sindrom Turetta u detei.

The article represents a review of modern literature on tics and Tourette's syndrome in paediatric population and describes recent advances in neurogenetics, neuroanatomy and neurotransmitter heterogeneity of the disease pathogenesis. The analysis of the literature supports the plausibility of transformation of tics and Tourette's syndrome from psychiatric disorders into neurological disorders (according to ICD-11) based on recent data on neurophysiology and functional neuroanatomy. Neuroanatomy of motor behaviour is described as the most complex self-regulating neural network of thalamo-cortical loop, basal ganglia in combination with the limbic system, insular and cingulate cortex with involvement of hippocampus, subthalamic region and cerebellum. The evaluation of pharmaceutical treatment options has been carried out, including a review of successful local experience in treating tics in paediatric patients with hopantenic acid, topiramate and neuropeptides of cortexin.

[The effectiveness of antiepileptic therapy in patients with epileptic syndromes associated with myoclonic seizures]. / Effektivnost' protivoepilepticheskoi terapii u patsientov s epilepticheskimi sindromami, assotsiirovannymi s mioklonicheskimi pristupami.

OBJECTIVE: To evaluate the effectiveness of antiepileptic therapy of epileptic syndromes associated with myoclonic seizures. MATERIAL AND METHODS: One hundred and three patients with epilepsy, in whom myoclonic seizures were identified during the observation, were examined. The observed myoclonic seizures entered the structure of 11 different epileptic syndromes. RESULTS AND CONCLUSION: Drug remission is achieved in 67% of patients, a decrease in the frequency of seizures by 50% or more was noted in 23,3%, the lack of effect in 9,7%. The effectiveness of antiepileptic therapy was significantly different in different groups of epileptic syndromes. The high effectiveness of antiepileptic therapy in the general group of patients in this study is mainly due to the prevalence of patients with juvenile myoclonic epilepsy, in which the highest percentage of remission was observed.

[Comorbidity of epilepsy and chronic disorders in children and adolescents with the assessment of the effectiveness of therapy]. / Komorbidnost' épilepsii i khronicheskikh tikoznykh rasstroistv u detei i podrostkov s otsenkoi éffektivnosti terapii.

AIM: To determine the comorbidity of epilepsy and chronic tic disorders (HTR) in children and adolescents based on the results of video-EEG monitoring and treatment efficacy. MATERIAL AND METHODS: One hundred and sixteen patients diagnosed with HTR, 83 boys and 33 girls, aged from 3 to 15 years, were studied. Clinical psychoneurological examination, psychological testing and video-EEG monitoring were performed at the first stage of the study. An effect of treatment was assessed at the second stage. RESULTS AND CONCLUSION: The high prevalence of epileptiform activity was observed in 46.6% of patients with HTR, comorbidity of epilepsy and chronic tic disorders in 16.4%. Antiepileptic drugs, in particular extended release valproate (depakine chronosphere), are most effective for both epilepsy and tic disorders.

[Results of a multicenter study on the efficacy of cortexin in treatment of cognitive dysfunction in children]. / Rezul'taty mul'titsentrovogo issledovaniia éffektivnosti primeneniia korteksina pri kognitivnykh disfunktsiiakh u detei.

AIM: To study the efficacy and tolerability of cortexin in the treatment of cognitive dysfunction in children. MATERIAL AND METHODS: The study included 635 patients, aged 3-7 years. Patients were divided into 4 clinical groups: group 1 (269 children with attention deficit hyperactivity disorder (ADHD)), group 2 (215 children with speech delay), group 3 (82 patients with the consequences of a perinatal lesion of the central nervous system), group 4 (69 patients with asthenic/neurotic syndrome). Attention, visual memory and thinking were assessed before and after treatment. Standard treatment of cortexin, including 10 intramuscular injections, was used. RESULTS AND CONCLUSION: The reliable effect of cortexin on cognitive impairment was shown. The best response to treatment was observed in patients with ADHD, aged 3-4 years, in particular on the tests measuring thinking. The tolerability of cortexin was good. It has been concluded that cortexin is an effective drug for treatment of children with ADHD, speech delay and consequences of a perinatal lesion of the central nervous system.

[Clinical and neuroimaging signs of cardioembolic stroke laboratory in children]. / Klinicheskie i neirovizualizatsionnye priznaki kardioémbolicheskogo insul'ta u detei.

AIM: To study clinical and neuroimaging signs of cardioembolic stroke (CES) in children. MATERIAL AND METHODS: Ten children (mean age 3.5±3.5 years) were examined. Eight children met the diagnostic criteria of CES CASCADE, two children were diagnosed with CES based on the complex of symptoms. A comparison group consisted of 90 children with other subtypes of ischemic stroke (IS). The diagnosis was confirmed by MRI and magnetic-resonance angiography; symptom severity was assessed with PedNIHSS and PSOM. RESULTS AND CONCLUSION: The PedNIHSS score in the acute stage of CES was higher (18.4±9.34) compared to patients with other subtypes of IS (12.6±6.2; p=0.0065). Six patients had large cerebral infarctions with the involvement of basal ganglia, internal capsule, white matter and cerebral cortex. In two patients, the infarction affected the cerebral cortex and underlying white matter, in one patient it affected the cortex from one side and the basal ganglia from the other. A damage of the cortex was noted in one patient. The frequency of recurrent CES was 30%, of fatal cases 20%. Recurrent IS and severe residual neurological deficit were more frequent in CES. CONCLUSION: Early disease onset, acute development and progression of neurological deficit up to the maximal level in the first 5 min, marked severity of the acute stage (PedNIHSS score >15), early onset of neurological function recovery, marked residual neurological deficit (PSOM score >2) are the additional signs of CES.

[Clinical and neuroimaging signs of cardioembolic stroke in children]. / Klinicheskie i neirovizualizatsionnye priznaki kardioémbolicheskogo insul'ta u detei.

AIM: To study clinical and neuroimaging signs of cardioembolic stroke (CES) in children. MATERIAL AND METHODS: A group of 100 patients with arterial ischemic stroke (AIS), aged from 1 month to 15 years, was stratified into the main group (10 patients with CES) and the comparison group (90 patients with other subtypes of AIS). CT and MRI, MR-angiography, ultrasound study as well as assessment of clinical symptoms on PedNIHSS were performed. The duration of follow-up was from 2 month to 14 years. The follow-up allowed the assessment of occurrence of secondary strokes, severity of residual neurological symptoms on the PSOM and fatal outcomes. Special attention was drawn to the use of CASSADE criteria for CES. RESULTS AND CONCLUSION: Clinical features that help to diagnose CES, even in the absence of adequate neuroimaging, in patients with heart disease, which is a potential high risk factor for cardioembolia, were established. These factors include early disease onset, acute development and progression of neurological deficit to the maximal level in the first 5 min., marked severity of the acute period (PedNIHSS > 15), early recovery of neurological functions, significant residual neurological symptoms (PSOM > 2). These symptoms should be used in addition to the CASCADE criteria in the diagnosis of CES of AIS type in children.

[The First Russian Consensus on the Multilevel Abobotulinumtoxin A Injections in Spastic Forms of Cerebral Palsy]. / Pervyi Rossiiskii konsensus po primeneniyu mnogourovnevykh in''ektsii abobotulinumtoxin A pri lechenii spasticheskikh form detskogo tserebral'nogo paralicha.

Spasticity treatment is one of the key aspects of the contemporary cerebral palsy (CP) rehabilitation that influences on the effectiveness of other methods. The paper presents the first Russian document that unites the recommendations for the BTA treatment of CP and could be used as the guideline for the multilevel injections. The Russian consensus on the multilevel botulinum toxin A (BTA) treatment of spastic CP is based on the international data and the results of national studies. The authors describe typical CP spasticity patterns in the upper and lower extremities, give recommended intervals for the BTA (Abobotulinum toxin A) dosages for the whole injection procedure and for the separate muscles. The method of dosage calculation for functional segments is also described. Attention is paid to the frequency, optimal intervals between the repeated injections and the whole duration of BTA treatment. The authors discuss effectiveness and safety of BTA, factors that potentially influence the results of the injections, including ultrasound and electromyography control, and indications for the continuation and termination of treatment.

[Arterial ischemic stroke in children with mild head trauma]. / Arterial'nyĭ ishemicheskiĭ insul'ta u deteĭ s legkoĭ travmoĭ golovnogo mozga.

AIM: To study the relationship between mild head trauma (MHT) and arterial ischemic stroke (AIS) in children. MATERIAL AND METHODS: We examined 81 patients with AIS, aged from 1 month to 15 years. The sample was stratified into two groups: patients with AIS caused by MHT (main group) and patients without MHT (comparison group). RESULTS AND CONCLUSION: Based on the results of clinical and instrumental studies, we identified the relationship between AIS and MHT. The main group was characterized by the following features: moderate severity in the acute period, stabilization within 7 days, isolated subcortical localization of ischemic lesion, no MRI-signs of steno-occlusive cerebral arteriopathy and a trend towards the risk of recurrent strokes.

[Classification system CASCADE for childhood arterial ischemic stroke].

In this review, a new consensus-based classification system for childhood arterial ischemic stroke CASCADE (2012) is described. The CASCADE is an anatomically based classification system which offers the specific diagnostic interventions and suggests the unified terminology. So, it can be used both in clinical practice and in any research in childhood arterial ischemic stroke.

[Secondary prevention of ischemic stroke in children].

Pediatric arterial ischemic stroke (IS) is an important cause of lifelong disability. Arteriopathies due to trauma and infection are an important underlying cause of childhood arterial ischemic stroke. The secondary prevention of IS should be conducted taking into account the main pathogenetic mechanisms and vascular risk factors. For secondary stroke prevention, the majority of children are treated with either anticoagulation or antiplatelet therapies. This review focuses on the recent international clinical recommendations in secondary stroke prevention based on the results of randomized multicenter clinical studies published by the USA. cardiology association. Experience of anticoagulation or antiplatelet therapies for secondary stroke prevention is insufficient in Russia. Taking into account the available international recommendations is expedient for creation and practical application of the Russian standards for secondary arterial ischemic stroke prevention.

Neuroimmune aspects of the pathogenesis of Tourette's syndrome and experience in the use of immunoglobulins in children.

A total of 60 patients aged 6-16 years with tics and Tourette's syndrome were studied. Antibodies to caudate nucleus proteins were assayed by western blot hybridization. Ten patients with Tourette's syndrome were found to have antibodies to caudate nucleus protein. Seven patients with neuroleptic-resistant types of Tourette's syndrome received single transfusions of immunoglobulin preparations, which produced regression of vocal and motor hyperkinesias and improvement in behavior (remission for more than six months). The observation of antibodies to caudate nucleus proteins and the positive effects seen on administration of immunoglobulins to patients with Tourette's syndrome support previous data on the possibility of using immunoglobulin therapy in the treatment of tic-type hyperkinesias and provide evidence of the involvement of autoimmune mechanisms inducing damage to the dopaminergic system of the striatum.

[Prothrombotic disturbances in children after ischemic stroke].

Thirty-six patients with stroke, aged from 8 months to 15 years, and 28 control children, aged 1-15 years, were studied. In both groups, the evaluation of anamnesis, neurologic status and genotyping for 11 most common prothrombotic polymorphisms were carried out. Coagulogram tests and measurement of homocysteine were performed before the anticoagulant therapy in the main group. The total frequency of prothrombin gene mutations (G20210A, factor V Leiden, MTHFR C677T and MTRR A66G) was 2.8 times higher in the main group compared to the control one. The most frequent genotypes were 677TT (8.3% of cases) and 66GG (30.6%). In patients with stroke, the homocysteine level exceeded the upper limit of normal age and also was significantly elevated in carriers of abovementioned risk genotypes: 10.29 +/- 1.55 mcmol/l vs 7.33 +/- 0.6 mcmol/l (p = 0.018). The coagulogram revealed disorders of anticoagulant system, including the decrease of protein C activity (22.7% of cases), protein S activity (13.6%) and antithrombin III (12.5%) and the increase of D-dimer level (21% of cases). It has been suggested to screen for common prothrombotic states and measure homocysteine levels in children with ischemic stroke and to lower homocysteine levels by using vitamins B6 and B12' and diet.

[Neuroimmunological aspects of the pathogenesis of Tourette syndrome and use of immunoglobulins in the treatment of children].

Sixty children, aged from 6 to 16 years, with tics and Tourette syndrome have been studied. Western immunoblotting techniques has been used to evaluate the serum antibodies against the caudate nucleus. These antibodies have been detected in 10 cases. Seven patients with Tourette syndrome who were treated with neuroleptics for a long time without any therapeutical effect received the immunomodulatory therapy with intravenous immunoglobulin. The regression of motor and vocal tics as well as improvement of behavioral symptoms were observed (duration of remission was more than 6 months). These findings and successful immunomodulatory therapy of patients with Tourette syndrome confirm previous reports in the literature and support the idea of immunologically triggered disturbance of the striatal dopaminergic system.