Electromyography assessment in pediatric localized scleroderma

Abstract

Scleroderma is a rare disease. About 1-3% of all Scleroderma patients have disease onset before 16 years of age. It is categorized in two different subtypes, Systemic and Localized, being the last predominant in the pediatric age range. Localized Scleroderma encompasses different skin lesions like Morphea, Linear Scleroderma, En coup de sabre and Facial hemiatrophy also called Parry-Romberg syndrome. Previously published work indicated neurologic inolvement in all the subtypes, related to either central or peripheric nervous system. Besides, myopathic activity close to the lesions is also reported. Muscle and nerve involvement needs a broader assessment and this is presented under the current proposal by a comprehensive multidisciplinary protocol with clinical and electrophysiologic assessments in order to estimate Localized Scleroderma neurologic impact and outcome. Significance and long term goals: This project represents multidisciplinary efforts aiming at a systematic clinical and neurologic assessment to contemplate medical needs of pediatric patients with Localized Scleroderma. Besides, it would bring about research oportunities for undergraduates engagement, for technical training in electromyography performance and for clinical assessment and imaging in this rare pediatric disease. Further opportunity of resarch about pathogeneses, treatment and outcome, is expected. (AU)