[Cognitive disorders in adolescents with multiple sclerosis: problem and methods].

A possible approach to evaluation of mild cognitive deficits in patients with multiple sclerosis (MS) is provided in the article. The results of the approbation of authors' experimental technique for studying cross-modal interactions that was carried out in healthy adolescents and MS patients are presented. Perspectives of its using in further research and some preliminary data on deficits in cross-modal syntheses in adolescents with MS are discussed. The role of impairment of interactions between different cognitive functions in the development of cognitive deficit is emphasized.

[Successful treatment of massive thrombosis of the vena cava inferior with nephrotic syndrome and chronic bilateral pulmonary artery thromboembolism in a patient with genetic thrombophilia].

A case is reported of a 23-year-old male patient who developed, after severe blunt injury of the lumbar region, massive thrombosis of the vena cava inferior (VCI), both renal veins, bilateral pulmonary artery thromboembolism (PATE), nephrotic syndrome (NS). In spite of anticoagulant therapy, the condition of the patient progressively aggravated for 1.5 year: thrombosis involved the ileac and femoral arteries on the right, thrombus floated in the right atrium with PATE recurrent episodes, pulmonary hypertension reached 120 mm Hg with formation of decompensated cor pulmnonale, proteinuria and hypoalbuminemia deteriorated, anasarca edema developed Multigenic thrombophilia was diagnosed (1 homozygous and 5 heterozygous mutations). A radical one-stage operation was successful: thromboectomy from the VCI, right ileac and left renal veins, thrombendarterectomy from the pulmonary arteries, suture of the interatrial septum defect, installation of cava-filter After the operation pulmonary pressure lowered to 40-45 mm Hg, right heart volume normalized, immunosuppressive therapy with prednisolone and cyclosporine led to nephropathy remission. The discussion covers mechanisms and factors (including genetic) of thrombosis progression, correlations between intravascular thrombosis, NS and chronic glomerulonephritis (possible NS development due to bilateral thrombosis of the renal veins and nephropathy role in thrombosis progression), approaches to conservative and surgical treatment of such patients. Global experience in conduction of pulmonary thrombendarterectomy and thrombectomy from VCI is reviewed (one-stage operations were not described earlier).

[Megalencephalic leukoencephalopathy with subcortical cysts (van der Knaap disease)].

A history of the discovery, hypotheses of the pathogenesis, neuromorphologic data, clinical appearances, differential diagnostics and an own description of a single case of rare hereditary children leukoencephalopathy, with megalencephaly, changes of myelin structure and formation of subcortical cysts are presented. Taking into account non-specific clinical appearances of this disease and difficulties of genetic analysis, a priority role of MRI-diagnostics revealing typical signs of the disease is emphasized.

[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.

[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.