A cross-sectional electromyography assessment in linear scleroderma patients

• Published in: Pediatric rheumatology online journal Vol. 12; no. 1; p. 27
• Main Authors: Saad Magalhães, Claudia, Fernandes, Taciana de Albuquerque Pedrosa, Fernandes, Thiago Dias, Resende, Luis Antonio de Lima
• Format: Journal Article
• Language: English
• Published: London BioMed Central 12.07.2014; BioMed Central Ltd
• Subjects: Biopsy; Cell adhesion & migration; Child, Preschool; Colleges & universities; Cross-Sectional Studies; Early Diagnosis; Early Medical Intervention; Electrodes; Electromyography - methods; Female; Foot Deformities, Acquired - etiology; Foot Deformities, Acquired - prevention & control; Hand Deformities, Acquired - etiology; Hand Deformities, Acquired - prevention & control; Humans; Male; Medical imaging; Medical research; Medicine; Medicine & Public Health; Medicine, Experimental; Muscle, Skeletal - innervation; Muscle, Skeletal - pathology; Muscle, Skeletal - physiopathology; Muscular Atrophy - diagnosis; Muscular Atrophy - etiology; Muscular Atrophy - physiopathology; Nervous system; Neural Conduction - physiology; NMR; Nuclear magnetic resonance; Outcome Assessment (Health Care); Pediatrics; Retrospective Studies; Rheumatology; Scleroderma; Scleroderma, Localized - complications; Scleroderma, Localized - diagnosis; Scleroderma, Localized - physiopathology; Scleroderma, Localized - therapy; Skin; Peripheral nervous system; Electromyography; Muscle; Linear scleroderma
• ISSN: 1546-0096; 1546-0096
• DOI: 10.1186/1546-0096-12-27

Background Muscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcutaneous and muscle atrophy. The aim was investigate nerve conduction and muscle involvement by electromyography in pediatric patients with LS. Methods We conducted a retrospective review of LS pediatric patients who had regular follow up at a single pediatric center from 1997–2013. We selected participants if they had consistently good follow up and enrolled consecutive patients in the study. We examined LS photos as well as clinical, serological and imaging findings. Electromyograms (EMG) were performed with bilateral symmetric technique, using surface and needle electrodes, comparing the affected side with the contralateral side. Abnormal muscle activity was categorized as a myopathic or neurogenic pattern. Results Nine LS subjects were selected for EMG, 2 with Parry-Romberg/Hemifacial Atrophy Syndrome, 7 linear scleroderma of an extremity and 2 with mixed forms (linear and morphea). Electromyogram analysis indicated that all but one had asymmetric myopathic pattern in muscles underlying the linear streaks. Motor and sensory nerve conduction was also evaluated in upper and lower limbs and one presented a neurogenic pattern. Masticatory muscle testing showed a myopathic pattern in the atrophic face of 2 cases with head and face involvement. Conclusion In our small series of LS patients, we found a surprising amount of muscle dysfunction by EMG. The muscle involvement may be possibly related to a secondary peripheral nerve involvement due to LS inflammation and fibrosis. Further collaborative studies to confirm these findings are needed.