[Differential diagnosis of epileptogenic substrates of unknown etiology using a modification of the routine MRI protocol]. / Differentsial'naya diagnostika epileptogennykh substratov neyasnoi etiologii s pomoshch'yu modifikatsii protokola standartnoi MRT.

OBJECTIVE: To evaluate the diagnostic capabilities of modifying the standard MRI protocol as part of an interdisciplinary presurgical examination of patients with epileptogenic substrates of unknown etiology. MATERIAL AND METHODS: The results of dynamic MRI of 8 patients with a referral diagnosis of focal cortical dysplasia (FCD) were analyzed. In 7 patients, epilepsy was the reason for a standard MRI of the brain; in another patient with myasthenia, MRI was performed as part of a comprehensive examination. All patients, in addition to standard MRI, underwent a modification of the real-time scanning protocol to include contrast, tractography (DTI), and perfusion techniques (ASL/DSC). In 1 case, with questionable results, the results of a modification of the standard MRI protocol, high-resolution MRI (HR MRI) and hybrid positron emission CT with 11C-methionine (PET/CT with 11C-MET) were combined. RESULTS: Seven patients underwent epileptic surgery and 1 patient was operated on for a tumor. In 4 out of 8 patients, based on the results of a modification of the standard MRI protocol, radiological signs of a neoplastic process were identified, which suggested a low-grade tumor. One of them needed PET/CT to confirm the assumption. The results of pathomorphological examination correlated with the direct diagnosis for surgical treatment. One of the 4 patients was suspected to have dysembryoplastic neuroepithelial tumor (DNET) based on the results of the protocol modification, which was also confirmed by pathological examination. In another 4 patients in whom it was possible to narrow the differential between FCD type II and DNET based on the results of the modification, FCD IIb was pathomorphologically verified. CONCLUSION: The proposed modification of the standard MRI protocol can significantly facilitate the differential diagnosis between the neoplastic and dysplastic origin of an epileptogenic substrate of unknown etiology, which in turn affects the patient's management tactics.

[The presurgical evaluation of patients with drug-resistant epilepsy]. / Prekhirurgicheskoe obsledovanie patsientov s farmakorezistentnoi epilepsiei.

Comprehensive multimodal examination of patients with focal refractory epilepsies is currently a prerequisite for high-quality pre-surgical diagnostics, which aims not only to expand the indications for radical treatment of epilepsy, but also to avoid the appearance of severe postoperative deficits. High-resolution MRI plays a key role in preoperative assessment. In this review, we illustrate the modern aspects of a pre-surgical diagnostical complex in examinations of patients with focal drug-resistant epilepsy, including the possibilities of neuroimaging.

[The structural basis of epileptogenesis in an adult patient: the comprehensive diagnostics experience]. / Strukturnaya osnova epileptogeneza u vzroslogo patsienta: opyt kompleksnoi diagnostiki.

On the example of the diagnosis of the structural basis of focal epilepsy in an adult patient, the possibilities of a multimodal and interdisciplinary approach to diagnosis, combining the latest methods of neuroimaging with the results of neurophysiological examinations, are considered. The interaction and high qualification of specialists in epileptology, neuroradiology and pathomorphology provide a high probability to determine the cause of the focal forms of epilepsy. Along with the introduction of super-inductive MR systems, it is important to use their capabilities correctly and optimize the scanning protocol for the individual characteristics of the patient. With a long-term pharmacoresistant course of focal epilepsy, accompanied by low quality of life, the progression of neurological deficits and the aggravation of cognitive and personality problems in patients, it becomes obvious that these patients, even with negative results of standard MRI, are candidates for a more in-depth comprehensive examination to identify the structural basis of epileptogenesis and subsequent epileptic surgery. However, the problem of epilepsy pseudoresistance should be considered, when a patient with uncontrolled seizures takes antiepileptic drugs for a long time in inadequate dosages. Modern comprehensive diagnostics offers new rational approaches to antiepileptic therapy indication on the part of the doctor, as well as to improve the patient's compliance to the treatment.

[Features of the neuroradiological picture of ganglioglioma on the example of 20 clinical cases]. / Osobennosti neiroradiologicheskoi kartiny gangliogliomy na primere 20 klinicheskikh sluchaev.

OBJECTIVE: To analyze the images obtained during pre-surgical neuroimaging in patients with gangliomas for the presence of specific signs and verification of the neoplastic process. MATERIAL AND METHODS: The results of presurgical MRI (3.0, 1.5 Tesla) of 20 patients with gangliomas were analyzed to identify specific signs of a neuronal-glial tumor and verify the neoplastic process based on the results obtained and a review of the literature. In addition to high-resolution MRI (HR MRI), various protocol modifications were applied to patients with epileptogenic pathological substrates of unclear etiology, including tractography (DTI) and contrast-free MR perfusion (ASL). In 5 cases, a multi-modal study was performed that combined the results of CT, routine MRI, HR MRI, functional MRI (fMRI) in various combinations and PET CT. RESULTS AND CONCLUSION: In 17 cases, patients underwent epileptic surgery. Three patients without epilepsy were operated on for a tumor diagnosed by radiological examination. In all 20 cases, gangliogliomas were verified, including 1 anaplastic, 1 infantile desmoplastic, and another patient had histological samples showing signs of a composite tumor. Combination with FCD IIIb was observed in 3 cases. Two patients had a double pathology (cases of tumors combination with lissencephaly and neuronal heterotopia) and another had a composite neuronal-glial tumor. In 15 cases, gangliogliomas showed neuroradiological features typical for dysembryoplastic neuroepithelial tumor (DNT) such as multicystic, nodular, and diffuse (dysplastic) described in the literature. In addition, in 9 cases, they had significant signs of neoplastic process such as contrast enhancement, continued growth and remodeling of the underlying bone. Verification of the neoplastic process based on the results of neuroradiological studies was difficult in 6 cases. In 2 cases, it was not possible to confirm the presence of neoplasm by radiological methods, and in 1 patient, the verification of the tumor during differential diagnosis took more than 8 years. The most common differential diagnosis was performed with DNT and FCD type IIb, which have a number of similar neuroradiological features.

[Features of neurological manifestations of the COVID-19 in children and adults]. / Osobennosti nevrologicheskikh proyavlenii COVID-19 u detei i vzroslykh.

The number of COVID-19 patients is increasing worldwide and the number of patients with neurological manifestations of a new coronavirus infection is increasing as well. Pathognomonic for COVID-19 is the presence of cephalgic syndrome, infectious-toxic encephalopathy, hypo- and anosmia and ageusia. Inducing of pathological autoimmune response contributes to the development of Miller Fischer and Guillain-Barré syndrome. Hyperergic reaction with the generation of the so-called «cytokine storm¼ provokes multisystem hemorrhagic complications such as Kawasaki disease and acute necrotizing hemorrhagic encephalopathy. There is also a special form of COVID-19-associated stroke. Almost all post-COVID-19 patients complain of severe fatigue, emotional lability, and sometimes have features of asthenic-neurotic, anxiety-phobic disorders and apato-abulic syndromes, which require rehabilitation measures, as well as courses of restorative neurotrophic and nootropic therapy.

[Early infantile epileptic encephalopathy type 14: three cases of epilepsy in infancy with migrating focal seizures due to KCNT1 mutations]. / Rannyaya mladencheskaya epilepticheskaya entsefalopatiya 14-go tipa: tri sluchaya epilepsii mladenchestva s migriruyushchimi fokal'nymi pristupami, obuslovlennymi mutatsiyami gena KCNT1.

OBJECTIVE: To study clinical and neurophysiological data of early infantile epileptic encephalopathy type 14 caused by KCNT1 mutations. MATERIAL AND METHODS: For the period 2017 to 2019, 3 non-relative girls with clinical characteristics of epilepsy of infancy with migrating focal seizures (EIMFS) and mutations in the KCNT1 gene are identified and studied. DNA sequencing was performed using the Hereditary epilepsy panel (Next Generation Sequencing on platform IlluminaNextSeq 500, USA). Dynamical video-EEG monitoring was done with "Encephalan-Video" RM-19/26 ("Medicom MTD", Russia). RESULTS AND CONCLUSION: De novo KCNT1 mutations are identified and studied in three unrelated Russian girls: M.V., 3 years and 3 month old, T.V., 9 month old and M.A., 5 month old. M.V. has the previously unknown mutation in exon 12 (chr9:138656907C>T) with amino acid substitution Arg356Trp. T.V. has the previously described mutation in chromosome 9: 138651532G>A with amino acid substitution Gly288Ser (OMIM: 608167.0010). M.U. has the previously unknown mutation in exon 15 (chr9:138660712A>G) with amino acid substitution Asp480Gly. M.V. has seizure onset at the age of 4 month with behavioral arrest seizures and tonic versive seizures. T.V. developed seizures at 4,5 month in the manner of behavior arrest and ophthalmo-clonic seizures with hyperemia of face. M.U. has neonatal seizures with bilateral tonic-clonic seizures, cyanosis and further development of status epilepticus of alternating hemiconvulsive seizures. Further all the girls develop polymorphic seizures of multiregional genesis up to migrating status epilepticus with typical electro-clinical pattern of EIMFS. Therefore, KCNT1 is likely to be a major gene causing this rare and severe epileptic syndrome.

[Cognitive and paroxysmal disorders in the long-term period of traumatic brain injury in children and adolescents]. / Kognitivnye i paroksizmal'nye rasstroistva v otdalennom periode cherepno-mozgovoi travmy u detei i podrostkov.

Traumatic brain injury (TBI) clinical course and outcomes in children have peculiarities as the damage impacts brain, which growth and maturation are continuing. Thus, TBI interferes into normal processes of neuroontogenesis leading to negative consequences on the cognitive functions development, school education, social skills acquisition. Cognitive and behavioral disorders in children and adolescents in the long-term period of TBI become more prominent in co-occurrence with paroxysmal disorders, including posttraumatic headaches, posttraumatic epilepsy and subclinical epileptiform activity on the EEG. Therapeutic and rehabilitation procedures in in the long-term period of TBI in children and adolescents should be conducted not only during the first 12 months after injury, when they are expected to be the most efficient, but also later on taking into consideration continuing processes of the CNS morphological and functional maturation along with the high neuroplasticity of the developing brain.

[Speech and language neurodevelopmental disorders in epilepsy: pathophysiologic mechanisms and therapeutic approaches]. / Narusheniia razvitiia rechi pri épilepsii: patofiziologicheskie mekhanizmy i terapevticheskie podkhody.

Speech and language development may be impaired in all forms of epilepsy involving specialized functional areas in the dominant cerebral hemisphere and their connections. The concept of epilepsy-aphasia clinical spectrum was recently proposed, but the notion of aphasia is quite conditional here as many of these patients demonstrate disorders of speech and language development from their infancy. Those forms of epilepsy are considered as continuum from the most severe Landau-Kleffner syndrome (LKS) and epilepsy with continuous spike-and-wave during sleep (CSWS) (also indicating as electrical status epilepticus during sleep - ESES) to intermediate epilepsy-aphasia disorders (with incomplete correspondence to diagnostic criteria of LKS and epilepsy with CSWS). The mild end of the spectrum is represented by benign childhood epilepsy with centrotemporal spikes (rolandic), which is often associated with speech and language disorders. The importance of genetic factors is discussed, including mutations in SRPX2, GRIN2A and other genes. The perspectives of individualized pharmacotherapy in epilepsy, co-morbid with neurodevelopmental disorders or impairments of speech and language development, are depending on the progress in genetic studies. In the new generation of antiepileptic drugs the positive influence on neuroplasticity mechanisms and higher cerebral functions are supposed for levetiracetam.

[Human prion diseases: current issues]. / Prionnye zabolevaniia cheloveka: sovremennye aspekty.

Prion diseases, or transmissible spongiform encephalopathies, are a group of neurodegenerative diseases with progressive dementia and movement disorders. There are three variants of prion diseases pathogenesis: direct contamination, genetic and sporadic forms. The following clinical forms are known: Creutzfeldt-Jakob disease (common type), variant Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker disease, variably protease-sensitive prionopathy, fatal insomnia and fatal familial insomnia, kuru, prion disease associated with diarrhea and autonomic neuropathy. Clinical characteristic of prion diseases, molecular-genetic aspects of their pathogenesis and current diagnostic approaches are discussed. Because of the lack of effective treatment, prevention of both alimentary prion infections (consumption of contaminated meat products) and transmissible iatrogenic infections (the use of biopreparations from animal tissues) is important. The safety of such biopreparations should be ensured by modern manufacturing technologies and specially developed procedures that meet international requirements and standards.

[The course and development of epilepsy in patients with typical variant of Rett syndrome and mutations]. / Osobennosti épilepsii u detei s tipichnym variantom sindroma Retta, vyzvannym mutatsiei.

AIM: To study the anamnesis, clinical state, electro-encephalographic and brain MRI characteristics in patients with Rett syndrome (МЕСР2) and epilepsy. MATERIAL AND METHODS: Eleven female patients, aged from 3 to 23 years, with Rett syndrome and MeCP2 mutations were studied. The study continued for 10 years (2006-2015). Assessment of neurological and mental status, night sleep video-EEG monitoring, MRI were performed. RESULTS AND CONCLUSION: Epilepsy was diagnosed in six cases (54.5%). Mean age at onset of epileptic seizures was 3 years 9 month. The following types of seizures were described: generalized, myoclonic, myotonic, tonic, versive, focal motor, atypical absences. Status epilepticus developed in one patient. Generalized seizures were identified in 56.25%, focal seizures in 43.75%. EEG changes were found in 9 patients (81.8%): slowing of the activity, episodes of periodic regional slowing, regional epileptiform activity and diffuse epileptiform activity, benign focal epileptiform discharges (BFED) of childhood, multiregional epileptiform activity. Five patients were treated with antiepileptic drugs. All of them had improved during treatment: a reduction of frequency of seizures was up to 50% in 4 cases (80%). One patient with resistant epilepsy was treated with the combination of drugs (levetiracetam, topiramate, zonisamide, benzodiazepine) that led to stopping of seizures during night sleep and decrease in the frequency of daytime seizures by 50%. Further research of epilepsy and efficacy of antiepileptic drugs in Rett syndrome is required.

[The use of low-field MRI for visualization of epileptogenic brain lesions in children]. / Primenenie nizkoinduktivnoi magnitno-rezonansnoi tomografii dlia vizualizatsii strukturnykh épileptogennykh porazhenii golovnogo mozga u detei.

AIM: To analyze MR-images of patients with focal epilepsy using a method close to the protocol of epileptic scanning on a MRI-device with the magnetic field tension of 0.4 Tesla. MATERIAL AND METHODS: MRI data of 50 children who underwent examination due to difficult-to-treat or drug-resistant forms of focal epilepsy were analyzed. MRI study was conducted using open-ended device of static magnetic field HITACHI 'APERTO' with the magnetic field tension of 0.4 Tesla. The thickness of the slices and the scan step was performed at 3.0 and 3.5 mm with the use of special positioning of slices in the coronal and axial projections, T2, T1, STIR, FLAIR weighted images (WI) perpendicular and parallel to the long axis of the hippocampus. RESULTS: Potentially epileptogenic structural changes were identified in 37 patients. Abnormalities of brain development of different severity were identified in 16 patients. In 21 cases, the changes were due to the consequences of cerebral vascular catastrophes, neuroinfections, brain traumas affecting the cortical plate. A method close to the epileptic scanning protocol allowed the identification of signs of acute cerebral catastrophes in two patients. In 8 patients, potentially epileptogenic changes after false-negative descriptions of the results of previous MRI studies were newly identified. The most difficult for the diagnosis was the visualization of small structural changes in mediobasal regions of the temporal lobes. Confirmation of this supposition according to the recommended thorough investigation was obtained only in 5 out of 12 patients with suspected small-bore pathological changes in mediobasal temporal regions. CONCLUSION: Low-tension technique approximated to the epileptic scanning protocol does not allow the reliable diagnosis of small and similar in signal characteristics changes in the visualization of mediobasal temporal lobes regions. It was not possible to reliably differentiate some cortical-subcortical neoplastic formations from various forms of focal cortical dysplasia.

[Peptidergic nootropic therapy in cerebral palsy associated with epilepsy]. / Peptidergicheskaia nootropnaia terapiia pri detskom tserebral'nom paraliche v sochetanii s épilepsiei.

AIM: To assess the efficacy and safety of сortexin in the treatment of children with cerebral palsy (CP) combined with epilepsy. MATERIAL AND METHODS: Eighty-four patients (55 boys and 29 girls), aged from 1 to 11 years, with CP combined with epilepsy received cortexin together with antiepileptic drugs (AEDs). Cortexin was administered in doses of 5-10 mg depending on the patient's age and body weight intramuscularly during hospitalization. RESULTS AND CONCLUSION: Cortexin as add-on to AEDs reduced for more than two times the number of seizures, along with improvement of motor function, in 31 (36.9%) patients. The improvement of motor function, but without a significant decrease in epileptic seizures, was achieved in 15 (17.8%) of the patients. Reduction of epileptic seizures frequency (>2 times), but without a significant effect on motor function, was observed in 14 cases (16.7%). Twenty-three patients (27.4%) did not respond the therapy. The aggravation of epileptic seizures during cortexin therapy was observed in only 1 girl with West syndrome (1.2%), and this was significantly lower than the probability of seizures aggravation on AED. Polypeptide nootropic medication cortexin demonstrated efficacy and safety as adjunctive therapy in children with CP combined with epilepsy.

[New classifications of epilepsies and seizure types created by the International League against Epilepsy (2017)]. / Novye mezhdunarodnye klassifikatsii épilepsii i épilepticheskikh pristupov Mezhdunarodnoi ligi po bor'be s épilepsiei (2017).

This review presents the recently published revised classifications of epilepsies and seizure types developed by the International League Against Epilepsy (ILAE). The Classification of Epilepsies includes several diagnostic levels (steps): 1) from seizure type to epilepsy type (generalized/focal/combined generalized and focal/unknown), 2) diagnosis of epilepsy syndrome 3) etiology (genetic/ structural/ infectious/ metabolic/ immune/unknown). A clinician can use any level of the classification. Operational classification of seizure types is replaced by the previous classification that was grounded on the anatomical basis. Seizures are classified by the onset (focal, generalized or unknown). All types of seizures can be motor or non-motor. Focal seizure may evolve to bilateral tonic-clonic (previously called secondary-generalized). Atonic, clonic, tonic, myoclonic seizures and epileptic spasms can be either of focal or generalized onset. Unclassified type of seizure was introduced. New types of seizures (absence with eyelid myoclonia, myoclonic absence, myoclonic atonic and clonic-tonic-clonic seizures) were added. New terminology, definitions and some concepts developed by ILAE are presented.

[The course and the development of epilepsy in patients with typical variant of Rett syndrome and mutations]. / Osobennosti techeniia i razvitiia épilepsii u detei s tipichnym variantom sindroma Retta, vyzvannogo mutatsiei.

AIM: Studying data of anamnesis, clinical state, electro-encephalographic, brain MRI in patients with Rett syndrome (МЕСР2). MATERIAL AND METHODS: We studied 11 patients (female) from three to 23 years old with Rett syndrome and MeCP2 mutations. Observation continued 10 years (2006-2015). We analyzed the results of the neurological status, night sleep video-EEG monitoring, MRI. RESULTS AND CONCLUSION: Epilepsy diagnosed in six cases (54, 5%). The overage age of debut of epileptic seizures was 3 years 9 months. There are some types of seizures: generalized, myoclonic, myotonic, tonic, versive, focal motor, atypical absences. Status epilepticus evolved in one patient. Generalized seizures were 56, 25%, focal seizures - 43, 75%. EEG changing marked in nine patients (81, 8%): slowdown back activity, episodes of periodic regional slowdown, regional epileptiform activity, and diffuse epileptiform activity like benign focal epileptiform discharges (BFED). five patients took antiepileptic drugs. All of them had improved during treatment. There were reducing of frequency of the seizures up 50% - 4 cases (80%). one patients with resistant epilepsy was taken combination of drugs (levetirecetam, topiromat, zonisamide, benzodiazepine) with stopping of seizures in the night sleep and decreasing of frequency of daytime seizures to 50%. We believe there is very important of study epilepsy in patients with Rett syndrome and improvement of its treatment.

[Treatment of epilepsy in children with brain tumors]. / Lechenie epilepsii u detei s opukholyami golovnogo mozga.

AIM: Symptomatic focal epilepsy is frequently caused by supratentorial brain tumors that may be surgically removed. The authors studied outcomes of surgical treatment depending on the use of electrocorticography c (ECoG). MATERIAL AND METHODS: Seventy-five children, aged 5-7 years, with supratentorial brain tumors were examined. Symptomatic epilepsy was found in 52 (69.3%) patients. Dysembryoplastic neuroepithelial tumors (DNET) and fibrillary astrocytomas were the most epileptogenic tumors. RESULTS AND CONCLUSION: The outcomes on the Engel scale were as follows: in 27 patients with surgical intervention without ECoG: class I - 9 patients, class II - 7 patients, class III - 5 patients, class IV - 6 patients and in 25 patients operated with ECoG: class I - 19 patients, class II - 4 patients and class III - 2 patients. The significant difference (p<0.01) between I+II Engel classes in comparison with III+IV Engel classes in operated patients demonstrated the necessity of ECoG during surgery in the resection of supratentorial brain tumors in patients with symptomatic epilepsy.

[MRI-characteristics of epileptogenic supratentorial brain tumors in children].

OBJECTIVE: To analyze MR-images in patients with symptomatic epilepsy associated with the brain tumor. MATERIAL AND METHODS: MRI results of 52 patients with symptomatic epilepsy operated for tumors of supratentorial localization were analyzed. The most epileptogenic tumors with atypical MRI signs and subtle clinical presentation were identified. All patients with tumors were operated using different methods of surgical intervention. RESULTS: Dysembryoplastic neuroepithelial tumors (DNET), diffuse astrocytomas (DA) and gangliogliomas (GG) were the most frequent epileptogenic tumors. In all the cases of DNET and in 4 patients with GG, epileptic seizures were the first, and in 4 of 5 cases of DIO were the only clinical sign of tumor presence. In DNET, DA and GG, there was an iso- or hypointensive signal on T1 WI and a signal varying in intensity from moderate to hyperintense in T2 and FLAIR WI, while in cases with DNET and GG, no mass effect and perifocal edema was practically seen. The so-called «spume-like¼ (multicystic) structure was most clearly observed in FLAIR WI. No significant changes in the dimensions of the DNET and GG were identified. The combination of DNET with focal cortical dysplasia was noted in one case. In DA, it was difficult to distinguish the perifocal edema from tumorous tissue and normal brain tissues, and the growth potential of malformation was slow. CONCLUSION: Epileptogenic tumors can imitate the x-ray characteristics of each other, and mimicry to gangliogliomas, oligodendrogliomas and astrocytomas Gr I, II, and others. They are the most frequent causes of symptomatic focal epilepsy. The presence of these malformations is necessary to exclude first of all in all cases of pharmacoresistant epilepsy.

[Neurodevelopmental disorders in children with epilepsy]. / Narusheniia nervno-psikhicheskogo razvitiia u detei s épilepsiei.

Neurodevelopmental disorders, including intellectual disability, autistic-spectrum disorders, speech disorders, attention deficit hyperactivity disorder (ADHD), learning disabilities, are more prevalent in children with epilepsy compared with the general population. Marked developmental delay and regression of acquired skills are characteristic of epileptic encephalopathies. Conditions, in which neurodevelopmental disorders are associated with the marked epileptiform EEG activity, while clinical epileptic seizures are absent, represent a serious problem. The authors consider the features of epilepsy with electrical status epilepticus during slow-wave sleep, pseudo-Lennox syndrome, Landau-Kleffner syndrome, children autistic epileptiform regression, autosomal-dominant rolandic epilepsy with verbal dispraxy and a combination of epilepsy and subclinical epileptiform EEG activity with developmental dysphasia and ADHD. In addition to the optimization of basic treatment with antiepileptic drugs (AEDs), nootropic drugs which do not increase epileptiform activity (hopantenic acid), are recommended.

[Developmental dysphasia and epileptiform EEG activity in children]. / Narusheniia razvitiia rechi u detei i épileptiformnaia aktivnost' na ÉÉG.

Objective. To study the electrical activity of the brain in children with developmental dysphasia (alalia). Material and methods. We analyzed the EEGs of 65 children with developmental dysphasia, including 48 boys and 17 girls, aged from 3 to 4 years 11 months. General speech underdevelopment (GSU) of the 1st level (with active vocabulary less than 15-20 words) was found in 31 children and GSU of the 2nd level (with active vocabulary of 20-50 words) - in 34 children. To specify the changes in the brain electrical activity, we conducted video-EEG-monitoring during sleep and waking states in 27 patients. Results. Focal epileptiform EEG changes with no concomitant paroxysmal symptoms were recorded in 12,3% of children with dysphasia. The epileptiform activity was more frequent in GSU of the 1st level (5 (16.1%) patients) than in GSU of the 2nd level (3 (8.8%) patients). Benign epileptiform discharges of childhood with low index were identified in 2 (6,5%) children with GSU of the 1st level and in1 (2,9%) child with GSU of the 2nd level; low index spike-waves were recorded in 3 (9,7%) children with GSU of the 1st level and in 2 (5,9%) with GSU of the 2nd level. Conclusion. The data allow to clarify the frequency of epileptiform EEG activity in those children with developmental dysphasia, who do not have autism or history of seizures. The differential diagnosis with rare epileptic encephalopathies is needed, such as epilepsy with electrical status epilepticus during slow sleep (ESES) and Landau-Kleffner syndrome.

[Efficacy and safety of topiramate depending on patient's age and forms of epilepsy].

Seven hundreds and twenty-two epileptic patients receiving topiramate (374 males, 348 females), aged from 3 month to 57 years, were followed with video-EEG control during the period of 2002-2012. Topiramate was effective in 465 (64.4%) patients, and among them the efficacy of monotherapy (72.2%) was higher compared to combined therapy (61.9%). The low efficacy was seen in 198 (27.4%) patients. The aggravation effect was noted in 59 (8.2%) of patients. Drug compliance (for >1 year) was 60.7%. In the group <1 year, the high efficacy was observed in 55.2%, low efficacy - in 34.5%, aggravation - in 10.3%. In the group 1-3 years, these indicators were 54.8%, 31.8% and 13.4%, respectively. In the pediatric population (>3 years), they were 67.3%, 26.2% and 6.5% as well as in the adult population (>18 years) - 82.1%, 16.6% and 1.3%, respectively. Thus, topiramate is a highly effective medication in the therapy of idiopathic generalized epilepsies without absences and in symptomatic/cryptogenic focal forms of epilepsy. The efficacy of topiramate raised with increasing of age while the aggravation risk decreased significantly.

[The syndrome of malignant migrating partial seizures in infancy or Coppola-Dulac syndrome (19 cases)].

Malignant migrating partial seizures in infancy (MMPSI) or Coppola-Dulac syndrome is a rare epilepsy syndrome with the onset in the first 6 months of life, characterized by multiple continuous electroencephalographic and electroclinical focal ictal patterns due to the involvement of different independent areas of both hemispheres with the arrest of psychomotor development. This article is based on the personal observations of 19 cases. Four subtypes of the syndrome were determined in our population of infants with MMPSI (n=19): 1) a "classic" form with pharmacoresistant migrated status epilepticus (SE) of migrating multifocal seizures, and with absolutely poor prognosis (n=7); 2) a severe pharmacoresistant mixed form (MMPSI + EME) with the combination of electroclinical characteristics of MMPSI with migrating multifocal SE and early myoclonic encephalopathy (n=5); 3) a "mild" variant (n=5); 4) a "subtle" form (n=2). In basic therapy, drugs of choice are antiepileptic drugs (AEDs) with a wide range of action (valproates, in the combination with barbiturates, benzodiazepines and levetiracetam) and also very old drugs as bromides. The preferred drugs are the valproate forms available for patients with disturbances of swallowing (depakine chronosphere or depakine syrup).