[Disease-modifying drugs in pediatric patients with multiple sclerosis]. / Preparaty, izmenyayushchie techenie rasseyannogo skleroza, u detei i podrostkov.

The vast majority of therapies are being evaluated and introduced for the treatment of adult multiple sclerosis (MS). A role of these drugs in the management of pediatric MS has yet to be defined both in Russia and in the whole world. Despite the fact that today the study of new drugs in the pediatric population have included in routine practices of the big pharmaceutical agencies, such as FDA and EMA, recommendations for the treatment of pediatric patients with MS are based not so much on a long period of systematic clinical research, but on professional consensus of international expert associations, in particular, the International pediatric multiple sclerosis study group (IPMSSG). The clinical trials include the small number of patients which is not comparable to those conducted in adults. Therefore, there is a need for study designs for assessment of efficacy and safety of the drugs for MS treatment in children and adolescents. The authors present the IPMSSG concept on the treatment of pediatric MS taking into account peculiarities of the Russian legislation and experience of national experts.

[Differential diagnosis of multiple sclerosis with pediatric onset: the experience of the Moscow Division for treatment of children and adolescents with multiple sclerosis].

The diagnosis of multiple sclerosis in children and adolescence should be differentiated from a group of rare white matter diseases, with fuzzy diagnostic criteria. Some of these conditions require modern diagnostic techniques and wide knowledge of the doctor. The Moscow Division for treatment of children and adolescents with multiple sclerosis is a specialized advisory structure, which has specialists with experience in the differential diagnosis of multiple sclerosis with pediatric onset. The emphasis on the clinical aspects in differential diagnosis of early onset multiple sclerosis can reduce the time for the diagnosis and help to avoid diagnostic mistakes.

[The comparative study of efficacy and tolerability of intramuscular introduction of beta-interferon-1a in adults and adolescents with remitting multiple sclerosis].

The experience of the treatment of patients with remitting multiple sclerosis (MS) with intramuscular introduction of beta-interferon-1a (avonex) is presented. Seventeen children and adolescents, aged from 11 to 18 years, and 55 adults, aged over 55 years, were treated for at least one-year period. Results revealed a significant reduction of exacerbations in both groups (from 1.35 to 0.06 in average in adolescents and from 0.86 to 0.17 in adults). The changes were accompanied by the stabilization of MS severity index: EDSS scores have decreased in 23.6% of adults and in 17.6% of adolescents. In both groups, good tolerability of the treatment was noted. There was a low probability of side-effects with the exception of increased frequency of a flu-like syndrome (47% cases) in patients younger than 18 years that demands special attention from children neurologists. The high efficacy and good tolerability and safety profile of beta-interferon-1a give grounds for administering this drug to children and adolescents with MS.

[Development of cognitive functions in children with multiple sclerosis: criteria for assessment of current state and prognosis of further development].

The results of neuropsychological and clinical examination of 29 children and adolescents with multiple sclerosis were analyzed. Specifics of cognitive development of patients at the early onset of disease and association between neuropsychological and MRI data as well as peculiarities of disease course were found. The authors emphasized the possibility of using neuropsychological diagnostics in the early stage of disease for prognostic purposes and assessment of treatment efficacy.

[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.

[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.