DYNAMICS OF CLINICAL AND ELECTROPHYSIOLOGICAL CHARACTERISTICS OF CARPAL TUNNEL SYNDROME IN CHILDREN WITH MUCOPOLYSACCHARIDOSES AGAINST THE BACKGROUND OF ENZYME-REPLACEMENT THERAPY
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.
About the authorsOsipova Liliya L.
Wraith JE, Alani SM. Carpal tunnel syndrome in the mucopolysaccharidoses and related disorders. Arch Dis Child. 1990; 65(9): 962-3.
Jadhav TM, Kornberg AJ, Peters H, Lee J, Ryan MM. Carpal Tunnel Syndrome in Pediatric Mucopolysaccharidoses. JICNA. 2015; 15: 101.
Argenta AE, Davit A. Carpal Tunnel Syndrome in the Setting of Mucopolysaccharidosis II (Hunter Syndrome). Plast Reconstr Surg Glob Open. 2017; 5(8):е1477. doi: 10.1097/GOX.0000000000001477.
Yuen A, Dowling G, Johnstone B, Kornberg A, Coombs C. Carpal tunnel syndrome in children with mucopolysaccaridoses. J Child Neurol. 2007; 22(3): 260-3.
Viskochil D, Muenzer J, Guffon N, Garin C, Munoz-Rojas MV, Moy KA, Hutchinson DT. Carpal tunnel syndrome in mucopolysaccharidosis I: a registry-based cohort study. Dev Med Child Neurol. 2017; 59(12): 1269-75. doi: 10.1111/dmcn.13545.
Haddad FS, Jones DH, Vellodi A, Kane N, Pitt MC. Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses. J Bone Joint Surg Br. 1997; 79(4): 576-82.
Kurenkov A.L., Podkletnova T.V., Kuzenkova L.M., Bursagova B.I., Nikitin S.S., Gevorkyan A.K., Vashakmadze N.D. Electromyography in diagnostic of median nerve lesion in carpal tunnel syndrome of children with mucopolysaccharidoses. Nervno-myshechnye bolezni. 2013; 4: 24-9. (In Russian) DOI:10.17650/2222-8721-2013-0-4-24-29.
Vijay S, Wraith JE. Clinical presentation and follow-up of patients with the attenuated phenotype of mucopolysaccharidosis type I. Acta Paediatr. 2005; 94(7): 872-7. doi:10.1080/08035250510031584.
Link B, de Camargo Pinto LL, Giugliani R, Wraith JE, Guffon N, Eich E, Beck M. Orthopedic manifestations in patients with mucopolysaccharidosis type II (Hunter syndrome) enrolled in the Hunter Outcome Survey. Orthop Rev (Pavia). 2010; 2(2):e16. doi: 10.4081/or.2010.e16.
Mendelsohn NJ, Harmatz P, Bodamer O, Burton BK, Giugliani R, Jones SA et al. Hunter Outcome Survey Investigators. Importance of surgical history in diagnosing mucopolysaccharidosis type II (Hunter syndrome): data from the Hunter Outcome Survey. Genet Med. 2010; 12(12): 816-22. doi: 10.1097/GIM.0b013e3181f6e74d.
White K, Kim T, Neufeld JA. Clinical assessment and treatment of carpal tunnel syndrome in the mucopolysaccharidoses. J Pediatr Rehabil Med. 2010; 3(1): 57-62. doi: 10.3233/PRM-2010-0103.
Kwon JY, Ko K, Sohn YB, Kim SJ, Park SW, Kim SH 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. doi: 10.1002/ajmg.a.34013.
Jurecka A, Marucha J, Jurkiewicz E, Różdżyńska-Świątkowska A, Tylki-Szymańska A. Enzyme replacement therapy in an attenuated case of mucopolysaccharidosis type I (Scheie syndrome): a 6.5-year detailed follow-up. Pediatr Neurol. 2012; 47(6): 461-5. doi: 10.1016/j.pediatrneurol.2012.08.010.
Van Heest AE, 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. doi: 10.1016/S0363-5023(98)80120-2.
Aslam R, Hendriksz CJ, Jester A. Objective results of median nerve decompression and tenosynovectomy for carpal tunnel syndrome in patients with mucopolysaccharidoses Types I and II. J Hand Surg Eur Vol. 2015; 40(2): 216-8. doi: 10.1177/1753193414523356.
Wyffels ML, Orchard PJ, Shanley RM, Miller WP, Van Heest AE. The Frequency of Carpal Tunnel Syndrome in Hurler Syndrome After Peritransplant Enzyme Replacement Therapy: A Retrospective Comparison. J Hand Surg Am. 2017; 42(7):573.e1-573.e8. doi: 10.1016/j.jhsa.2017.03.036.
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