Neuromuscular DiseasesNeuromuscular DiseasesНервно-мышечные болезни2222-87212413-0443Publishing House ABV Press6410.17650/2222-8721-2013-0-4-24-29ORIGINAL REPORTSОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯElectromyography in diagnostic of median nerve lesion in carpal tunnel syndrome of children with mucopolysaccharidosesЭлектромиография в диагностике поражения срединного нерва при синдроме запястного канала у детей с мукополисахаридозамиKurenkovA. L.КуренковА. Л.
Russian Federation
alkurenkov@gmail.comPodkletnovaT. V.ПодклетноваТ. В.
Russian Federation
KuzenkovaL. M.КузенковаЛ. М.
Russian Federation
BursagovaB. I.БурсаговаБ. И.
Russian Federation
NikitinS. S.НикитинС. С.
Russian Federation
GevorkyanA. K.ГеворкянА. К.
Russian Federation
VashakmadzeN. D.ВашакмадзеН. Д.
Russian Federation
Center of Child Health, Russian Academy of Medical Sciences, MoscowФГБУ «Научный центр здоровья детей» РАМНInstitute of General Pathology and Pathophysiology, Russian Academy of Medical Sciences, MoscowНИИ общей патологии и патофизиологии РАМН, Москва201220133424292002201520022015Copyright ©; 2013, Kurenkov A.L., Podkletnova T.V., Kuzenkova L.M., Bursagova B.I., Nikitin S.S., Gevorkyan A.K., Vashakmadze N.D.Copyright ©; 2013, Куренков А.Л., Подклетнова Т.В., Кузенкова Л.М., Бурсагова Б.И., Никитин С.С., Геворкян А.К., Вашакмадзе Н.Д.2013Kurenkov A.L., Podkletnova T.V., Kuzenkova L.M., Bursagova B.I., Nikitin S.S., Gevorkyan A.K., Vashakmadze N.D.Куренков А.Л., Подклетнова Т.В., Кузенкова Л.М., Бурсагова Б.И., Никитин С.С., Геворкян А.К., Вашакмадзе Н.Д.https://creativecommons.org/licenses/by/4.0https://nmb.abvpress.ru/jour/article/view/64

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 incarpal channel were detected in patients with MPS III and MPS IV. CTS was bilateral in children with MPS I, II and VI, but usuallythere 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.

mucopolysaccharidosis Imucopolysaccharidosis IImucopolysaccharidosis IIImucopolysaccharidosis IVmucopolysaccharidosis VIenzyme-replacement therapycarpal tunnel syndromeelectromyographydistal latency CMAPconduction velocityмукополисахаридоз I–IV типовмукополисахаридоз VI типаферментзаместительная терапиясиндром запястного каналаэлектромиографиядистальная латентность М-ответаскорость распространения возбуждения по периферическому нерву1. Van Meir N., De Smet L. Carpal tunnel syndrome in children. J Pediatr Orthop B 2005;14(1):42–5.Van Meir N., De Smet L. Carpal tunnel syndrome in children. J Pediatr Orthop B 2005;14(1):42–5.2. Кузенкова Л.М., Подклетнова Т.В. Неврологические аспекты мукополисахаридозов. Труды II Национального конгресса «Неотложные состояния в неврологии» (под. ред. З.А. Суслиной, М.А. Пирадова). Москва, 2011. С. 158–163.Кузенкова Л.М., Подклетнова Т.В. Неврологические аспекты мукополисахаридозов. Труды II Национального конгресса «Неотложные состояния в неврологии» (под. ред. З.А. Суслиной, М.А. Пирадова). Москва, 2011. С. 158–163.3. Yuen A., Dowling G., Johnstone B. et al. Carpal tunnel syndrome in children with mucopolysaccaridoses. J Child Neurol 2007; 2(3):260–3.Yuen A., Dowling G., Johnstone B. et al. Carpal tunnel syndrome in children with mucopolysaccaridoses. J Child Neurol 2007; 2(3):260–3.4. Haddad F.S., Jones D.H., Vellodi A. et al. Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses. J. Bone Joint Surg. Br. 1997;79(4):576–582.Haddad F.S., Jones D.H., Vellodi A. et al. Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses. J. Bone Joint Surg. Br. 1997;79(4):576–582.5. Van Heest A.E., House J., Krivit W., Walker K. Surgical treatment of carpal tunnel syndrome and trigger digits in children with mucopolysaccharide storage disorders. J Hand Surg Am 1998;23(2):236–43.Van Heest A.E., House J., Krivit W., Walker K. Surgical treatment of carpal tunnel syndrome and trigger digits in children with mucopolysaccharide storage disorders. J Hand Surg Am 1998;23(2):236–43.6. Katirji B. Electromyography in clinical practice. Philadelphia: MOSBY Elsevier, 2007. 417 p.Katirji B. Electromyography in clinical practice. Philadelphia: MOSBY Elsevier, 2007. 417 p.7. Meyer-Marcotty M.V., Kollewe K., Dengler R. et al. Carpal tunnel syndrome in children with mucopolysaccharidosis type 1H: diagnosis and therapy in an interdisciplinary centre. Handchir Mikrochir Plast Chir 2012;44(1):23–8.Meyer-Marcotty M.V., Kollewe K., Dengler R. et al. Carpal tunnel syndrome in children with mucopolysaccharidosis type 1H: diagnosis and therapy in an interdisciplinary centre. Handchir Mikrochir Plast Chir 2012;44(1):23–8.8. White K., Kim T., Neufeld J.A. Clinical assessment and treatment of carpal tunnel syndrome in the mucopolysaccharidoses. J Pediatr Rehabil Med 2010;3(1):57–62.White K., Kim T., Neufeld J.A. Clinical assessment and treatment of carpal tunnel syndrome in the mucopolysaccharidoses. J Pediatr Rehabil Med 2010;3(1):57–62.9. Muenzer J., Beck M., Eng C.M. et al. Multidisciplinary management of Hunter syndrome. Pediatrics 2009;124(6): e1228–39.Muenzer J., Beck M., Eng C.M. et al. Multidisciplinary management of Hunter syndrome. Pediatrics 2009;124(6): e1228–39.10. Kimura J. Electrodiagnosis in Diseases of Nerve and Muscle: Principles and Practice. New York: Oxford University Press, 2013. 1146 p.Kimura J. Electrodiagnosis in Diseases of Nerve and Muscle: Principles and Practice. New York: Oxford University Press, 2013. 1146 p.11. Van Meir N., De Smet L. Carpal tunnel syndrome in children. Acta Orthop Belg 2003;69(5):387–95.Van Meir N., De Smet L. Carpal tunnel syndrome in children. Acta Orthop Belg 2003;69(5):387–95.12. Kwon J.Y., Ko K., Sohn Y.B. et al. High prevalence of carpal tunnel syndrome in children with mucopolysaccharidosis type II (Hunter syndrome). Am J Med Genet A 2011;155A(6):1329–35.Kwon J.Y., Ko K., Sohn Y.B. et al. High prevalence of carpal tunnel syndrome in children with mucopolysaccharidosis type II (Hunter syndrome). Am J Med Genet A 2011;155A(6):1329–35.13. Norman-Taylor F., Fixsen J.A., Sharrard W.J. Hunter's syndrome as a cause of childhood carpal tunnel syndrome: a report of three cases. J Pediatr Orthop B 1995;4(1): 106–9.Norman-Taylor F., Fixsen J.A., Sharrard W.J. Hunter's syndrome as a cause of childhood carpal tunnel syndrome: a report of three cases. J Pediatr Orthop B 1995;4(1): 106–9.14. Daube J.R. Nerve conduction studies. In book: Electrodiagnosis in Clinical Neurology (ed. M.J. Aminoff). Philadelphia: Churchill Livingstone Elsevier, 2005. P. 285–320.Daube J.R. Nerve conduction studies. In book: Electrodiagnosis in Clinical Neurology (ed. M.J. Aminoff). Philadelphia: Churchill Livingstone Elsevier, 2005. P. 285–320.