[Behcet's disease with neurologic manifestations]. / Bolezn' Bekhcheta s nevrologicheskimi proiavleniiami.

Behcet's disease (BD) is a type of systematic chronic inflammatory disease of unknown etiology that is characterized by the combination of recurrent oral aphthous, genital ulcers and uveitis. A damage of the nervous system is observed in 3-9% of patients, in which two variants of the course of the disease are possible: parenchymal in the form of immune-mediated meningoencephalitis, and non-parenchymal, which can be represented by venous sinus thrombosis, intracranial arterial aneurysms and intracranial hypertension. The article presents a clinical case of BD with a dominant nervous system damage in the clinical presentations of the disease.

[Mutation del 1,02kb in the CLN3 gene and extrapyramidal syndrome]. / Mutatsiya del 1,02 kb v gene CLN3 i sindrom ekstrapiramidnykh rasstroistv.

Mutations in the GBA and SMPD1 genes, which lead to the development of lysosomal storage diseases, are high risk factors for Parkinson's disease and dementia with Lewy bodies. We screened the mutations in the GALC and CLN3 genes in patients with Parkinson's disease and control subjects. A heterozygous CLN3 mutation (del 1.02 kb) carrier with clinical features of the unusual extrapyramidal syndrome was identified. A role of CLN3 mutations in the development of neurodegenerative disorders is discussed.

[The formation of social professional skills of students with limited possibilities].

The education of children and adolescents with limited possibilities is a system of economic, public, legal and individual personal measures supporting the optimal conditions to overcome this defect up to a degree of participation in public life on par with other citizen. In the Nijny Novgorod academic boarding school for the disabled the organization of activities forming the social professional skills are differentiated on terms and targets of education. The students of first course need the optimization of communication, enhancement of self-consciousness, formation of educational information perception, reasoning, involuntary memory voluntary logical memory attention, will, educational general intellectual expertise. The students of second course need more attention to making and optimization of various types of activity, activation of labor activity, forming of labor attitudes, communicative skills and enhancement of emotional and motivational sphere. The students of third course need to develop the skills of self-reliant work, motoric activity, dexterity labor skills, working discipline skills, rational attitude to group work, professional knowledge.

[The issues of the medical social rehabilitation of the handicapped persons of able-bodied age].

Among the medical social and economic problems having important universal public significance, a particular place belongs to the issues of the disability and the organization of care of persons with limited physical and mental possibilities. The Nijhny Novgorod oblast experience of work with the handicapped persons demonstrates that in the large-scale industrial region the level of disability is rather high with the neatly marked tendency to increase. The need for the professional education and further employment assistance is quite meaningful among the handicapped persons of able-bodied age. To meet this demand is possible only on the way of the development of the system of professional rehabilitation on the oblast level. The most appropriate conditions for the professional rehabilitation of the young handicapped persons are established in the special-purpose professional specialized schools where the rehabilitation divisions are deployed. This approach allows to work effectively on the implementation of the individual programs of handicapped persons rehabilitation and in that way to provide a higher level of social security of the students (i.e. higher scholarship, free-of-charge meals and medical services). In the city of Nijhny Novgorod specialized boarding school for the handicapped persons a system of activities is developed to enhance the social adaptation including the blocks of labor and everyday life adaptation and practical work training.

[The social problems of the families with disabled children of adolescent age].

Presently, the number of families bringing up children with limited capabilities in the domestic conditions is increasing. The upbringing of child suffering of development deviation purposes to achieve the harmonious functioning of atypical child, the highest possible development of the potential capabilities of child and every family member to be successfully integrated into society everyday life. The parents need timely support in the upbringing of this category of children, including social psychological care to overcome their alienation in society and to increase the self-care among them. In the specialized boarding school for disabled the division of rehabilitation is established to provide the overall rehabilitation care to the studying disabled adolescents.