[Polymorphism of electroencephalographic pattern in benign epileptiform discharges in childhood]. / Polimorfizm élektroéntsefalograficheskogo patterna dobrokachestvennykh épileptiformnykh narushenii v detstve.

Incidence and significance of benign epileptiform discharges of childhood (BECD), or rolandic spikes, have been studied in 2723 children, aged 2-15 years, with (841 patients) and without (1882) epilepsy. All the patients underwent standard electroencephalographic (EEG) study with video-EEG monitoring made in cases of epileptiform abnormalities. In the non-epileptic group, BECD frequency was 1.33%. There was a significant predominance of boys in both groups. The age of maximal BECD expression was 4-5 years in the non-epileptic group and 9-10 years--in children with epilepsy. The majority of children without seizures demonstrated different neurological and neurocognitive abnormalities, such as chronic headaches (25%), attention deficit hyperactivity disorder (25%) and speech delay (21%). Epileptiform abnormalities were observed mostly in the right hemisphere in children with epilepsy and in the left hemisphere in non-epileptic patients. There was a morphological similarity of electroencephalographic patterns in patients with rolandic epilepsy, benign occipital epilepsy, pseudolennox syndrome, Landau-Kleffner syndrome and electrical status epilepticus during slow sleep. The authors conclude that BECD are nonspecific feature of rolandic epilepsy and can occur in the broad spectrum of disturbances forming "hereditary impairment of brain maturation" group. Hereditary mechanisms involved in realization of various electroclinical features of focal brain dysfunction are suggested.

[Age dynamics of temporal lobe epilepsy in children]. / Vozrastnaia dinamika visochnoi épilepsii u detei.

To study clinical features of temporal lobe epilepsy in children of different age, to trace disease development and to define risk factors for its occurrence and adverse course, 88 patients aged 3-14 years were examined. The study confirmed that temporal lobe epilepsy has its characteristic clinical features in children of different age. In children under 7 years of age, motor and autonomic paroxysms prevail accompanied in older children with psychomotor and psychosensory fits. With age the latter become more complicated. In children under 10 years of age psychomotor seizures prevail and in those older 10 years sensory and psychosensory ones predominate. Children over 10 years were characterized by polymorphic seizures or combinations of several kinds of seizures. The seizures can transform into either generalized or focal ones. In our study, the course of temporal lobe epilepsy was rather favorable. Seizures number reduced by more than a half in 82% of the cases. Adverse disease course was mainly detected in children with perinatal complications in their anamnesis and "spike-wave" complexes in EEG as well as in those suffering from psychomotor and psychosensory seizures.

[Botox in combined treatment of cerebral palsy in children]. / Botoks v kompleksnom lechenii detskogo tserebral'nogo paralicha.

Ten patients with cerebral palsy (CP) were treated with botox (botulinum toxin, type A). The next forms of the disease were observed in the patients: a spastic dysplegia, a spastic hemiplegia, a spastic-hyperkinetic form of CP. Botox was injected into the damaged muscles in a dose of 100-300 Units. Therapeutical effect was found in 90% of the patients, an average duration of the medical effect was 6-12 months. Because of the small cohort of the patients treated with botox there was made a preliminary conclusion about the high efficiency and safety of botox for CP treatment. The need of further clinical observations was emphasized.

[Reversible red cell aggregation in children with insulin-dependent diabetes mellitus]. / Obratimaia agregatsiia eritrotsitov pri insulinzavisimom sakharnom diabete u detei.

Red cell aggregation has been studied by photometry in 47 children with insulin-dependent diabetes mellitus. The disease was associated with boosting of reversible red cell aggregation, which was most of all expressed during metabolic decompensation with ketoacidosis. When metabolic subcompensation was attained, this parameter did not completely normalize and still had to be corrected.

[The neuroradiological aspects of infantile spasms]. / Neiroradiologicheskie aspekty infantil'nykh spazmov.

The observation of 38 children with early form of children epilepsy that is with syndrome of infantile spasms (IS) was performed by means of computed tomography of the brain. The structural alterations of the brain were revealed in 89.5% of cases. They varied from rough developmental anomalies and pronounced destructive phenomena to microdysgenesias and moderate cortical atrophies. These changes were systematized according to the time of the beginning of some alteration on the definite stage of neuro-ontogenesis. There were embryofetal disturbances (23.6%), peri- and postnatal alterations (50%) and combined ones (15.7%). The definite correlation was established between neuroradiological damages, clinical pattern of IS, differentiated policy of the treatment and the prognosis of neuropsychic development of a child.

[Epilepsy and dreams in children]. / Epilepsiia i snovideniia u detei.

24 patients at the age of 10-18 years with different forms of epilepsy and 10 healthy children and juveniles were examined. The analysis of dreams testified both intensification and quantitative peculiarities of dreams in epileptic patients. Their specific features were: frightening character, stereotypy, brightness and contrastness of colour images as well as psychosensory disorders and sensations of different modalities, peculiar and original topic. The dreams characteristic for epilepsy arise frequently long before the objective clinical manifestation and might serve as one of the important markers of cerebral epileptisation.

[The Outpatient Pediatric Neurology Service of Moscow today]. / Ambulatornaia detskaia nevrologicheskaia sluzhba Moskvy na sovremennom étape.

The organisation of children's neurological service was considered in terms of 25 years of medical-diagnostic work of children's consultative neurological out-patient clinic (CCNC) attached to one of Moscow children's clinical hospital (Morozov hospital). The work of appropriate departments of CCNC was presented together with the consideration of the main problems of child's neurology as well as of the peculiarities of nervous system's pathology in terms of age. The close connection was emphasized between CCNC and child's neurologists of general out-patient clinics as well as the role of CCNC both in coordination of their work and in the rise of their qualification.

[Features of surface architectonics and ultrastructure of peripheral blood in children with insulin-dependent diabetes mellitus]. / Osobennosti poverkhnostnoi arkhitektoniki i ul'trastruktury perifericheskoi krovi u detei, bol'nykh insulinzavisimym sakharnym diabetom.

Electron microscopic study of the surface architectonics and ultrastructure of the peripheral blood erythrocytes in 25 children with insulin-dependent diabetes mellitus (IDDM) revealed that the decrease of the count of disk-shaped cells, increased count of transitional and degenerative forms, and alteration of their ultrastructure. Traditional therapy failed to completely normalized these characteristics over the entire follow-up.

[Rasmussen's chronic progressive focal encephalitis]. / Khronicheskii progressiruiushchii ochagovyi éntsefalit Rasmussena.

Chronic progressive focal encephalitis or Rasmussen encephalitis (RE) is chronic brain disease of viral etiology. It is manifested by either partial motor or myoclonic seizures combined with hemiparesis. RE was described in detail in a girl of 8.5 years. The disease debut was observed at the age of 5.5 and manifested in the form of simple partial motor fits with serial episodes of secondary generalization. Post-attack hemiparesis was observed from the age of 7.5 and became permanent after 8. From this time myoclonic fits became constant, changed for the worse, memory and attention deteriorated. NMR tomography revealed widening and deepening of the Sylvian fissure as well as a focal decrease of left temporal area density. The patient was resistant to anticonvulsant, hormonal and immunosuppressive drugs. The criteria of RE diagnosis, its difference from Kozhevnikov's epilepsy are provided. Estimation of drug and surgical treatment effectivity is given. The necessity of early surgical treatment (functional hemisphere ectomy) is emphasized.

Heat shock proteins in Amoeba: I. Effect of high temperature on Amoeba proteus and Amoeba borokensis.

A sudden increase of temperature of surrounding medium, even in the sublethal range, induced in Amoeba proteus (strain War) and Amoeba borockensis the synthesis of a new class of proteins, which might be classified as the heat shock proteins (hsp). The new pattern of protein synthesis, determined by incorporation of (35)S-methionine, was detectable within 30 min after beginning of the exposure of the cells to the elevated temperature. Amoeba proteus (strain War) and Amoeba borokensis differ distinctly in thermosensitivity, however, no distinct difference in the rate of the heat shock proteins' synthesis between the studied species was found.

[Chromosome instability in diseases of the nervous system]. / Nestabil'nost' khromosom pri zabolevaniiakh nervnoi sistemy.

The authors review both their own findings and literature data about chromosomal instability in neurologic diseases. The genetic mechanisms responsible for the development of chromosomal instability and its role in the pathogenesis of different diseases of the nervous system are discussed.

[Features of the course of temporal-lobe epilepsy in children]. / Osobennosti techeniia visochnoi épilepsii u detei.

Forty-nine patients (aged 7-15 years) with temporal epilepsy were examined using clinical and electrophysiological methods; 27 children were also subjected to psychological examination and 14 of them to computerized tomography of the brain. Characteristics of temporal paroxysms and correlation between the course of epilepsy and its duration and progression are described in detail. It was shown that neuroinfections, craniocerebral traumas, perinatal pathology of the nervous system and genetic predisposition play a certain role in the genesis of temporal epilepsy. Psychological examination of patients with temporal epilepsy revealed various disorders of neuropsychic processes in 27 children. In 5 cases computer-aided tomography of the brain revealed various changes including petrifacates, cysts, foci of gliosis with partial brain atrophy and deformation of the lateral ventricles.

Response to light-shade difference in anucleate and polynucleate specimens of Amoeba proteus.

The enucleated specimens of Amoeba proteus, the anucleate fragments, and the polynucleate individuals which all are capable of cortical contraction but not of locomotion, may be reactivated by the light-shade difference established across their body. Individual cells or fragments migrate toward the shade. The motory polarity and coordinated movement disappear immediately after cessation of the stimulus. The results are interpreted according to the earlier hypothesis that the necessary to maintain the motory polarity of amoebae. It is suggested that the anucleate and polynucleate specimens are incapable of coordinated movements when non-stimulated, because of a deficiency or an excess, respectively, of the regulatory relaxing factor secreted by the nucleus of Amoeba proteus.

[Neurologic syndromes in children with phakomatosis]. / Nevrologicheskie sindromy pri fakomatozakh u detei

The author studied 189 patients with the main forms of phakomatosis (Louis-Barre syndrome, Sturge-Weber, Hippel-Lindau disease and Recklinghausens neurofibromatosis.) The use of pathogenetical treatment in the Louis-Barre syndrome (a transplantation of a neonatal thymus with the bone marrow) promoted an improvement in the clinico-immunological indices. A study of familial forms of the Louis-Barre syndrome and the Sturge-Weber disease demonstrated a similarity according to the main clinical indices among the sibs in 80% which may speak of the role of the genetical factors in the formation of the disease.

[Lipid metabolism disorder in ataxia-telangiectasia]. / Narushenie obmena lipidov pri ataksii-teleangiéktazii

A clinico-morphological study of ataxia-teleangiectasia permits to establish the depth and distribution of lesions. Morphological findings indicate to lesions mainly in the extrapyramidal system, cerebellum, subcortical structures and in the stem structures and spinal cord. Besides, there were changes in the hemispheres in the form of cell atrophy, demyelinization, degeneration and gliosis. Demyelinization was found in the posterior and lateral columns of the spinal cord; in the extrapyramidal conductive paths. A study of lipid metabolism in 14 patients with ataxia-teleangiectasia also demonstrated an increase of lipid content in myelin, free cholesterol, etherconnected cholesterol, triglycerides and diglycerides in the plasma. There was a correlation between the degree of the nervous system demyelinization and an increased level of the main fractions in the lipid spectrum. In some patients following a transplantation of a neonatal thymus-sternum complex there was a drop in the previously increased content of the lipid fractions. The authors discuss some problems of the pathogenesis of ataxiateleangiectasia.