Electromyography in diagnostic of median nerve lesion in carpal tunnel syndrome of children with mucopolysaccharidoses

Cover Page

Cite item

Full Text

Abstract

Carpal tunnel syndrome (CTS) is a frequent syndrome in adults, but is very rare in children. CTS was described in children with mucopolysaccharidoses (MPS) as condition due to the deformation of carpal bones, deposition of glycosaminoglycans in tendosynovial tissue and connective tissue of flexor retinaculum. Electromyography is essential method for diagnostic CTS in children because typical symptoms of CTS (paresthesia, numbness of hand and fingers, atrophy and paresis of certain muscles) seen in adults are absent or not realized by children with MPS because of cognitive deficit despite the presence of nerve involvement. EMG results from 40 children with different types of MPS (age 1 year 8 months to 18 years) are presented. Neurophysiologic abnormalities related to CTS were found in every child with MPS I, in 80,9 % of cases – with MPS II and in every case – with MPS VI; no EMG signs of median nerve lesions in
carpal channel were detected in patients with MPS III and MPS IV. CTS was bilateral in children with MPS I, II and VI, but usually
there was an asymmetry of changes. We revealed CTS in one patient with MPS II as early as at the age 2 years 11 months. All children with MPS II had already CTS at the age of 4 years except one patient. Children with MPS I and MPS VI were not investigate before the age 4 years old, but one child 4 years old with MPS I had severe CTS. In children with MPS atrophy of thenar eminence muscles developed rapidly as complication of CTS. Therefore we recommend repeating of EMG regularly to identify earliest signs of median nerve disturbance in carpal channel and opportune surgical decompression of the entrapped nerve. It allows preserving normal function of hand that it is very important for adequate child development and quality of life.

About the authors

A. L. Kurenkov

Center of Child Health, Russian Academy of Medical Sciences, Moscow

Author for correspondence.
Email: alkurenkov@gmail.com
Russian Federation

T. V. Podkletnova

Center of Child Health, Russian Academy of Medical Sciences, Moscow

Email: fake@neicon.ru
Russian Federation

L. M. Kuzenkova

Center of Child Health, Russian Academy of Medical Sciences, Moscow

Email: fake@neicon.ru
Russian Federation

B. I. Bursagova

Center of Child Health, Russian Academy of Medical Sciences, Moscow

Email: fake@neicon.ru
Russian Federation

S. S. Nikitin

Institute of General Pathology and Pathophysiology, Russian Academy of Medical Sciences, Moscow

Email: fake@neicon.ru
Russian Federation

A. K. Gevorkyan

Center of Child Health, Russian Academy of Medical Sciences, Moscow

Email: fake@neicon.ru
Russian Federation

N. D. Vashakmadze

Center of Child Health, Russian Academy of Medical Sciences, Moscow

Email: fake@neicon.ru
Russian Federation

References

Supplementary files

Supplementary Files
Action
1. JATS XML

Copyright (c) 2013 Kurenkov A.L., Podkletnova T.V., Kuzenkova L.M., Bursagova B.I., Nikitin S.S., Gevorkyan A.K., Vashakmadze N.D.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ЭЛ № ФС 77 - 85909 от  25.08.2023.