COGNITIVE EPILEPTIFORM DISINTEGRATION: DEFINITION, DIAGNOSIS, THERAPY

Cognitive epileptiform disintegration — symptom complex of acquired disorders of cognitive functions in children, associated with marked epileptiform activity on EEG in the absence of epileptic seizures (isolated epileptic seizures in the medical history are possible). On background of review and discussion of current scientific literature and own data the author represents the fundamental article, devoted to cognitive epileptiform disintegration. The author describes in detail the aspects of definition, aetiology and pathogenesis, diagnostic criteria and differential diagnostics of this disorder, and approaches to the treatment. As the main factors of aetiology and pathogenesis the author describes the role of hereditary impairment of brain maturation and pathologic effects of continuous prolonged epileptiform activity on cognitive functions, as well as morphologic alterations (lesion) of the brain, caused by pathology of prenatal period. The clinical features of cognitive epileptiform disintegration depending on predominance localization of epileptiform activity in different areas of brain cortex are discussed. The especial attention is given to approaches to the treatment of cognitive epileptiform disintegration (approaches to the treatment of seizures and cognitive disorders are discussed separately). Efficacy of valproates, corticosteroid hormones and antiepileptic drugs of other groups is considered and assessed

RUFINAMIDE (INOVELON) IN THE TREATMENT OF LENNOX–GASTAUT SYNDROME (A REVIEW OF LITERATURE AND CLINICAL CASE)

Lennox–Gastaut syndrome (LGS) is childhood-onset epileptic encephalopathy manifested by frequent polymorphic seizures, including tonic axial, obvious cognitive impairments, characteristic ECG changes, and therapeutic resistance. Due to the concurrence of several types of seizures in the clinical presentation of the disease and their resistance to drug therapy, great hopes are pinned on the design of novel antiepileptic drugs with fundamentally other mechanisms of action and aimed specially at treating this severe type of epilepsy. The authors review the foreign literature on the new antiepileptic drug rufinamide (Inovelon) registered in Russia in January 2015 to directly treat LGS as adjunctive treatment in children 4 years and older and adults. Multiple trials have demonstrated that rufinamide has efficacy and good tolerability in treating LGS.The authors describe an observed case of the efficacy of rufinamide in a 17-year-old male patient with LGS. Despite the drug resistance of epilepsy and no response to multiple antiepileptic drugs used alone and in different combinations, the incorporation of rufinamide into a treatment regimen had a pronounced therapeutic effect: the frequency of convulsive seizures decreased by 70 %. At the time of writing this paper, the patient has been receiving rufinamide for more 5 months. He has been tolerating the therapy well

Epileptic spasms: Nosological characteristics and approaches to therapy

Objective: to study the incidence, anamnestic, clinical, electroencephalographic, and neuroimaging features of epileptic syndromes associated with epileptic spasms (ES) and the efficiency of antiepileptic therapy in patients with these conditions. Patients and methods. The study covered 1261 patients with epileptic seizures in the history with their onset from the first day of life to 18 years.Results. A history of ES was recorded in 112 patients, which accounted for 8.9% of cases among all the forms of epilepsy with its onset less than 18 years of age. ES was detected in 47.1% of the patients with seizure onset in the first year of life. There was a slight male preponderance: 59 (52.7%) versus 53 (47.3%). Epilepsy with ES onset occurred in the first 6 months of life in 57.9% of cases. The West syndrome was found in 58.9% of the patients with ES-associated epilepsy, which amounted to 5.2% of all forms of epilepsy developing at the age of less than 18 years; Ohtahara syndrome in 32.1% (2.9% of all forms of epilepsy), early myoclonic encephalopathy in 0.9% (0.08%) and symptomatic focal epilepsy with periodic spasms in 8.1% (0.7% of all forms of epilepsy). Antiepileptic therapy led to complete remission in 45.5% of ES-associated epilepsy cases. There was a 50% or more reduction in the rate of seizures in 35.7% of the patients treated with antiepileptic drugs. No effect was seen in 18.8% of cases

EFFICACY AND TOLERABILITY OF LAMITOR (LAMOTRIGINE) IN THE TREATMENT OF IDIOPATHIC FOCAL AND GENERALIZED EPILEPSY IN MONOTHERAPY AND IN POLYTHERAPY IN CHILDREN AND ADOLESCENTS

Results of research of efficacy and tolerability of lamitor (lamotrigine) in the treatment of idiopathic focal and generalized forms of epilepsy as monotherapy or in polytherapy in children and adolescents are presented. 25 patients were included in our research, aged from 2 till 18 years, with idiopathic focal (n=10) and generalized (n=15) epilepsy, which received lamitor in monotherapy (5 patients) or in polytherapy (20 patients). As a whole, remission was reached at 18 of 25 patients (72%), significant improvement – at 5 of 25 (20%), the effect was absent – 1 patient (4%), deterioration was noted only in 1 case (4%). Efficiency of lamitor in dependence on epilepsy form was considered. Good tolerability of lamitor is noted. Thus, our research demonstrated high efficiency and good tolerability of lamitor as an additional antiepileptic drug and in monotherapy in the treatment of idiopathic epilepsies in children and adolescents

Scaling of THz-conductivity at metal-insulator transition in doped manganites

Magnetic field and temperature dependence of the Terahertz conductivity and permittivity of the colossal magnetoresistance manganite Pr_{0.65}Ca_{0.28}Sr_{0.07}MnO_3 (PCSMO) is investigated approaching the metal-to-insulator transition (MIT) from the insulating side. In the charge-ordered state of PCSMO both conductivity and dielectric permittivity increase as function of magnetic field and temperature. Universal scaling relationships between the changes in permittivity and conductivity are observed in a broad range of temperatures and magnetic fields. Similar scaling is also seen in La_{1-x}Sr_xMnO_3 for different doping levels. The observed proportionality points towards the importance of pure ac-conductivity and phononic energy scale at MIT in manganites.Comment: 5 pages, 4 figure

The multiferroic phases of (Eu:Y)MnO3

We report on structural, magnetic, dielectric, and thermodynamic properties of (Eu:Y)MnO3 for Y doping levels 0 <= x < 1. This system resembles the multiferroic perovskite manganites RMnO3 (with R= Gd, Dy, Tb) but without the interference of magnetic contributions of the 4f-ions. In addition, it offers the possibility to continuously tune the influence of the A-site ionic radii. For small concentrations x <= 0.1 we find a canted antiferromagnetic and paraelectric groundstate. For higher concentrations x <= 0.3 ferroelectric polarization coexists with the features of a long wavelength incommensurate spiral magnetic phase analogous to the observations in TbMnO3. In the intermediate concentration range around x = 0.2 a multiferroic scenario is realized combining weak ferroelectricity and weak ferromagnetism, presumably due to a canted spiral magnetic structure.Comment: 8 pages, 8 figure

Terahertz spectroscopy of electromagnons in Eu_{1-x}Y_xMnO_3

Dielectric permittivity spectra of yttrium-doped EuMnO$_3$ in the composition range 0 =< x =< 0.5 have been investigated in the terahertz frequency range. Magnetoelectric contributions to the permittivity were observed in all compositions for ac electric fields parallel to the crystallographic a-axis. Well defined electromagnons exist for x >= 0.2 close to \nu ~ 20 cm^{-1} and with dielectric strength strongly increasing on doping. In addition to electromagnons, a broad contribution of magnetoelectric origin is observed for all compositions. For Eu_{0.8}Y_{0.2}MnO_3 the electromagnons can be suppressed by external magnetic fields which induce a canted antiferromagnetic phase. Magnetoelectric effects in the different doping regimes are discussed in detail.Comment: 7 pages, 9 figures include

Nested Bethe ansatz for Y(gl(n)) open spin chains with diagonal boundary conditions

In this proceeding we present the nested Bethe ansatz for open spin chains of XXX-type, with arbitrary representations (i.e. `spins') on each site of the chain and diagonal boundary matrices $(K^+(u),K^-(u))$. The nested Bethe anstaz applies for a general $K^-(u)$, but a particular form of the $K^+(u)$ matrix. We give the eigenvalues, Bethe equations and the form of the Bethe vectors for the corresponding models. The Bethe vectors are expressed using a trace formula.Comment: 15 pages, proceeding for Dubna International SQS 09 Worksho

A CLINICAL CASE OF SYNGAP1 GENE MUTATION IN A GIRL WITH EPILEPSY, MENTAL RETARDATION, AUTISM, AND MOTOR DISORDERS

The introduction of the latest genetic techniques into practice could discover a basis for the comorbidity of genetic epilepsies and behavioral disturbances with cognitive impairments. Some chromosomal syndromes are characterized by a specific electroencephalogram (EEG) pattern, the type of seizures, and the variant of the course of epilepsy. This paper describes a case of synaptic RAS GTP-ase-activating protein 1 (SYNGAP1) gene mutation in a 9-year-old female patient with eyelid myoclonic epilepsy, atypical absences, and atypical autism with mental retardation. The patient’s parents visited a physician for epilepsy (myoclonic absences), markedly delayed psycho-speech development, and specific communication problems in the child. The characteristics of autistic behavior were manifested from birth; routine EEG recorded epileptiform activity at the age of 2 years; epileptic seizures appeared at 5 years. Valproic acid and levetiracetam in this patient exerted a good effect on seizures; however, a clinical and encephalographic remission was achieved by a combination of levetiracetam and ethosuximide. The clinical case including the neurological and psychic statuses, logopedic characteristics, the result of psychological testing, and video-EEG monitoring findings are analyzed in detail.The SYNGAP1 gene is located on chromosome 6p21.3. About 50 cases of SYNGAP1 syndrome are now known worldwide. After normal maternal pregnancy and delivery, the patients show delayed psychomotor development with pronounced regression at 1 to 3 years of age. At this age, there are diffuse polyspike discharges on the EEG or an onset of generalized epileptic seizures (atonic, myoclonic, eyelid myoclonic, and absence seizures), commonly photosensitivity and autoinduction, mental development stops, speech regresses, behavioral disorders that are typical of autism develop. Drug-resistant epilepsy is noted in approximately half of the described cases. There is a correlation between the severity of epilepsy and cognitive deficit

Study of Δ(1232)\Delta(1232) isobar electroproduction at VEPP-2M e+e−e^+e^- collider

Results from the Spherical Nonmagnetic Detector (SND) on $\Delta (1232)$ isobar electroproduction in the collisions of beam electrons (positrons) and residual gas nuclei in the VEPP-2M $e^+e^-$ collider are presented. On the basis of the obtained data the expected counting rate of this process in future high luminosity $e^+e^-$ colliders (~$\phi$-, $c$-$\tau$- and $b$-factories) was estimated.Comment: 7 pages LATEX and 3 figure