Neuroimmune aspects of the pathogenesis of Tourette's syndrome and experience in the use of immunoglobulins in children.

A total of 60 patients aged 6-16 years with tics and Tourette's syndrome were studied. Antibodies to caudate nucleus proteins were assayed by western blot hybridization. Ten patients with Tourette's syndrome were found to have antibodies to caudate nucleus protein. Seven patients with neuroleptic-resistant types of Tourette's syndrome received single transfusions of immunoglobulin preparations, which produced regression of vocal and motor hyperkinesias and improvement in behavior (remission for more than six months). The observation of antibodies to caudate nucleus proteins and the positive effects seen on administration of immunoglobulins to patients with Tourette's syndrome support previous data on the possibility of using immunoglobulin therapy in the treatment of tic-type hyperkinesias and provide evidence of the involvement of autoimmune mechanisms inducing damage to the dopaminergic system of the striatum.

[Neuroimmunological aspects of the pathogenesis of Tourette syndrome and use of immunoglobulins in the treatment of children].

Sixty children, aged from 6 to 16 years, with tics and Tourette syndrome have been studied. Western immunoblotting techniques has been used to evaluate the serum antibodies against the caudate nucleus. These antibodies have been detected in 10 cases. Seven patients with Tourette syndrome who were treated with neuroleptics for a long time without any therapeutical effect received the immunomodulatory therapy with intravenous immunoglobulin. The regression of motor and vocal tics as well as improvement of behavioral symptoms were observed (duration of remission was more than 6 months). These findings and successful immunomodulatory therapy of patients with Tourette syndrome confirm previous reports in the literature and support the idea of immunologically triggered disturbance of the striatal dopaminergic system.

[Myoclonus epilepsy in children and adolescents]. / Mioklonus-épilepsiia u detei i podrostkov.

Twelve cases of myoclonus epilepsy initiating in childhood and adolescence were studied clinically, electrophysiologically and biochemically. Myoclonic and epileptic syndromes being most pronounced, the clinical pattern presented certain polymorphism: asymmetry, hyperkinesis, myodystonia, epileptic episodes, mental disorders. The electroencephalographic picture of the disease was variable. Hyperexcretion of glycosaminoglycans and changes in their fraction composition with predominant release of heparan sulphate evidenced for deranged metabolism of mucopolysaccharides.

[Renal excretion of glycosaminoglycans under normal conditions and in several nervous system diseases in children]. / Pochechnaia ékskretsiia glikozaminoglikanov v norme i pri nekotorykh zabolevaniiakh nervoi sistemy u detei

The authors studied 101 children with diseases of the nervous system (with hereditary and acquired pathology) and 205 practically normal children. The urine excretion of specific components of the connective tissue (glycosaminoglycanes) was higher than in normals. Lesions of the connective tissue stroma in such forms of pathology is expressed not only in quantitative changes of glycosaminoglycane, but in their qualitative characteristics. Disturbances of the fractional compounds of glycosaminoglycane in the urine of patients differs from the parameters of chromatograms of normals by a high content of fractions of heparansulfate in a relatively low level of chondroethylsulfatolike fractions. In such states the severity of the clinical picture is accompanied by expressed metabolic disturbances.