[A use of high dosages of piracetam in the treatment of Kozhevnikov epilepsy syndrome].

An efficacy of high dosages of piracetam in the treatment of children with Kozhevnikov epilepsy syndrome (epilepsia partialis continua) has been studied. The study included 6 patients, aged from 9 to 16 years, with diagnosis of Kozhevnikov epilepsy (KE) who received piracetam (nootropil, UCB) in mean dose of 1g/kg/day (up to 35 g/day) intravenous in drops. The treatment course was 30 days. During therapy, there was decrease of myoclonus frequency by 75% in 3 patients and its full stopping in 3 patients. Focal clonic seizures were stopped in 2 cases, their frequency decreased in 4 cases. Secondary generalized seizures did not relapse in 2 cases and there was a decrease of seizures by 50% and over in 4 remained cases. The use of nootropil led to the improvement of neurological status in 5 out of 6 patients with KE. The intensity of hemiparesis was reduced in 3 patients treated with nootropil. The improvement of cognitive functioning was observed in 5 out of 6 patients. The duration of clinical improvement was 1-2 months after infusions cessation. The study revealed the efficacy of high dosages of nootropil in combination with basic antiepileptic drugs in the treatment of Kozhevnikov epilepsy.

[Clinical and psychological features of children with agenesis of the vermis cerebelli].

The article considers behavioral disturbances in children with anomalies of the cerebellum found by MRI studies. Presented are literature data on the relations between pathology of the cerebellum and early autism in children. The cerebellum is involved not only in movement coordination but also in social adaptation and speech communication. Cerebellum-specific genes expressed in early age are similar to those of hippocampus. Our own study of children with agenesis of the vermis cerebelli detected by MRI and behavioral disturbances included 20 children aged 3-15 years (mean age 7,05 years, 12 male, 8 female). Some autistic features have been found.

[Epilepsy with electrical status epilepticus during slow sleep: diagnostic criteria and approaches to therapy].

Fourteen patients, aged from 5 to 14 years, with syndrome of electrical status epilepticus during slow sleep (ESESS) have been studied. The absence of epileptic attacks was observed in 21.5% of patients and diagnosis was established by a combination of continuous diffuse epileptiform activity with marked cognitive disturbances. In 78.5% patients, epileptic attacks presented as follows: pharyngeal and oral, hemicephalgia (100% patients with attacks), hemiclonic (18%), atypical absences (27%), negative myoclonus (18%), automotor (18%), focal adverse with vomiting (18%), secondary generalized (36%). Drugs of choice were valproate (depakine) and topamax in patients with attacks; suxilep and frisium in the absence of attacks and in case of continuous diffuse epileptiform activity on the sleep EEG. Two variants have been singled out by the character of ESESS syndrome. The first one, a "symptom variant", was featured by mostly hemiclonic, secondary generalized and automotor epileptic attacks, presence of continuous regional or lateral, less frequent diffuse epileptiform activity, detected by the local structural lesions in MRI, marked cognitive disturbances persisting after stopping of the attacks. The second ("idiopathic") variant is characterized by normal development of children before attack manifestation, appearance of mostly "rolandic" attacks, atypical absences and negative myoclonus, presence of exclusively continuous diffuse epileptiform activity in the phase of slow sleep, standard MRI results (moderate cortical subatrophy in single cases), cessation or substantial decrease of cognitive disturbances after attack stopping. It is emphasized that prognosis of ESESS syndrome should be considered separately with regard to attacks and cognitive disturbances. A prognosis for attacks is always favorable. Cognitive disturbances despite the therapy can persist for a long time.

[Cognitive disturbances in multiple sclerosis].

Results of the study of cognitive functions in children with multiple sclerosis (MS), including literature data on cognitive disturbances in this disease, are presented. Fourteen patients, aged from 8 to 17 years, with definite MS have been studied. A neuropsychological study revealed impulsivity in the performance of tasks without the sufficient orientation on the test instruction; difficulties in the visual perception in conditions of different complexity and difficulties of the simultaneous synthesis; elements of the left-side ignoring, difficulties in understanding, the usage of logical grammatical construction, activity programming and the rhythm perception. These peculiarities are interpreted in the context of A.R. Luria conceptions on the marked impairment of the functional activity of the right hemisphere, reduction of an operational constituent of mental processes related to the activity of the second functional block in the brain (parietal, occipital and temporal areas); decrease of regulatory aspects of the activity related to the activity of the third structural-functional block, which included the convexital frontal cortex with all the cortical and subcortical connections; reduction of the activating provision of mental processes related to the work of the first structural-functional block (deep, subcortical brain structures and their connections). It is emphasized that cognitive disturbances in children may appear on the earlier stages of MS.

[Diagnostic differentiation between two syndromes of progressive myoclonus epilepsy]. / Differentsial'nyi diagnoz dvukh sindromov progressiruiushchei mioklonus-épilepsii.

The study aimed to differentiate, according to clinical and electroencephalographical criteria, between the most frequent types of myoclonus epilepsy--Unverricht-Lundborg disease (ULD) and Lafora disease (LD). Two patients with ULD and two with LD, aged 13-16 years, have been examined. In all cases, the diagnosis of myoclonus epilepsy has been verified by using molecular genetic analysis. The differential diagnostic criteria have been ascertained for ULD and LD: the earlier age-of-onset in ULD comparing to LD (8.8 and 12.5 years, respectively); tonic clonic paroxysms in ULD and partial ones with visual aura in LD; a presence of nonepileptical subcortical myoclonus, according to EEG data (visual monitoring); negative myoclonus emerging in manifested stage of LD; appearing of regional epileptiform activity for posterior regions on EEG in LD; more rapid progressiveness of extra pyramidal disturbances with organic dementia outcome in myoclonus LD.

[3D FSPGR (fast spoiled gradient echo) magnetic resonance imaging in the diagnosis of focal cortical dysplasia in children]. / Ispol'zovanie rezhima 3D FSPGR v magnitno-rezonansnoi diagnostike kortikal'nykh displazii u detei.

Small dysplastic lesions of the cerebral cortex are often missed by conventional MRI methods. The identification of subtle structural abnormalities by traditional multiplanar rectilinear slices is often limited by the complex convolutional pattern of the brain. We used a method of FSPGR (fast spoiled gradient-echo) of three-dimensional MRI data that improves the anatomical display of the sulcal structure of the hemispheric convexities. It also reduces the asymmetric sampling of gray-white matter that may lead to false-positive results. We present 5 from 12 patients with dysplastic cortical lesions in whom conventional two-dimensional and three-dimensional MRI with multiplanar reformatting was initially considered normal. Subsequent studies using 3D FSPGR identified various types of focal cortical dysplasia in all. These results indicate that an increase in the detection of subtle focal dysplastic lesions may be accomplished when one improves the anatomical display of the brain sulcal structure by performing 3D FSPGR.

[Psychosomatic motor disorders in the structure of depression in children and adolescents]. / Psikhosomaticheskie dvigatel'nye narusheniia v strukture depressii u detei i podrostkov.

106 patients (40 boys and 66 girls) at the age from 3 to 15 years with depression of neurotic level with motor disorders (hyperkinetic, hypokinetic, pseudoepileptic) were examined. The motor disorders were estimated as pseudoneurological ones. The treatment of such patients must be performed with estimation of the typological variations of depression (asthenic type was observed in 31 cases, anxious type--in 37, melancholic type--in 6, combined type--in 32), of the degree of depressions manifestation, of their etiology as well as the pathological "basis" and personal peculiarities of the patients. 81 patients were treated by antidepressants (AD) in combination with tranquilizers and nootropes. AD weren't applied in 25 cases. Of all the patients in which AD were used complete correction of pseudoneurological and depressive disorders was observed in 46 cases (56.8%), considerable improvement--in 26 patients, the improvement--in 9 cases. Meanwhile improvement was observed only in 15 cases and slight improvement in 10 persons in patients who had not received any AD (group differences were significant).