DYNAMICS OF CLINICAL AND ELECTROPHYSIOLOGICAL CHARACTERISTICS OF CARPAL TUNNEL SYNDROME IN CHILDREN WITH MUCOPOLYSACCHARIDOSES AGAINST THE BACKGROUND OF ENZYME-REPLACEMENT THERAPY

Abstract

Introduction. Mucopolysaccharidoses are the most common cause of the carpal tunnel syndrome in children. Enzyme-replacement therapy, which is available in clinical practice within last 10-15 years for mucopolysaccharidoses I, II and VI demonstrated the efficacy regarding somatic symptoms of the diseases, however, the impact of this new pathogenetic treatment on neuropathy of median nerve, arising from compression in the carpal tunnel, is poorly described. Objective. To study the dynamics of clinical and electrophysiological characteristics of carpal tunnel syndrome in children with mucopolysaccharidoses during enzyme-replacement therapy. Materials and methods. 18 children with mucopolysaccharidoses I, II and VI, received enzyme-replacement therapy, are included in the study. Dynamics of clinical symptoms of the carpal tunnel syndrome and EMG indices of median nerve testing are described against the background of enzyme-replacement therapy. Results. Objective and subjective clinical manifestations of the carpal tunnel syndrome persisted or appeared in children, receiving enzyme-replacement therapy for 21±12 months. However, there were no statistically significant changes in EMG-characteristics of median nerves testing during enzyme-replacement therapy in children with mucopolysaccharidoses Conclusion. Enzyme-replacement therapy in children with mucopolysaccharidoses I, II and VI may suspend and/or delay progressive damage of median nerves in the carpal canal, thus indicating prevention and slowing down in glycosaminoglycans storage in the carpal tunnel.