Article is devoted to one of the orphan diseases — mucopolysaccharidosis (MPS), which is the result of any lysosomal enzyme deficiency (which determines the type of illness). The most common is the MPS type II (Hunter syndrome), developing as a result of deficiency of the enzyme alpha-L-iduronosulphatsulphataze. The authors are observing the largest group of children with this pathology in the Russian population — 40 patients. On the example of their own clinical cases the only existing on the date the pathogenetic treatment is provided — replacement therapy with idursulphase that significantly improves the disease prognosis. Key words: MPS, types, Hunter syndrome, clinical course, diagnosis, treatment, prognosis, children.

Medications for the immunomodulatory therapy of the multiocular sclerosis are widely spread and have been applied in adult neurologic practices for a long time, although the data about their use among the patients under 18 is scarce and not classified. We present the history of foreign and russian experience in application of the medication for the immunomodulatory therapy of the multiocular sclerosis — interferon beta 1b — among children and teenagers along with the brief analysis of the acquired data about effectiveness and safety of this type of treatment in pediatrics in the published research. Key words: multiocular sclerosis, immunomodulatory therapy, children, teenagers.

The lecture deals with the present-day data (author-obtained and literature-derived) concerning multiple sclerosis (MS) in children, including the incidence of the disease, problems related to the classification, aetiology, and pathogenesis. The authors present a detailed description of different variants of clinical manifestations in MS, principles of diagnosis and differential diagnosis of the disease, widely presenting various aspects of MS therapy: pathogenetic and symptomatic, in steady progression and during remission and stabilization of the disease. Special attention is paid to the prognosis, invalidization and social life in the paediatric-age patients with multiple sclerosis.