Related Experiment Video
Updated: Apr 26, 2026
08:16
Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
33.3K
A cross-sectional electromyography assessment in linear scleroderma patients
Claudia Saad Magalhães1, Taciana de Albuquerque Pedrosa Fernandes1, Thiago Dias Fernandes2
1Pediatric Rheumatology Unit, Pediatrics Division, Botucatu Medical School, São Paulo State University (UNESP), Botucatu, São Paulo, Brazil.
Pediatric Rheumatology Online Journal
|July 24, 2014
Summary
Electromyography revealed significant muscle dysfunction in pediatric linear scleroderma (LS) patients, indicating potential secondary peripheral nerve involvement. This study highlights the importance of assessing muscle and nerve function in children with LS.
Area of Science:
- Pediatric Rheumatology
- Neuromuscular Disorders
- Dermatology
Background:
- Linear scleroderma (LS) commonly presents with muscle atrophy and asymmetric limb growth.
- Extra-cutaneous manifestations are frequent in LS, with neurological involvement suggested in muscle atrophy.
- Sympathetic dysfunction may play a role in the pathogenesis of subcutaneous and muscle atrophy in LS.
Purpose of the Study:
- To investigate nerve conduction and muscle involvement using electromyography (EMG) in pediatric patients diagnosed with linear scleroderma.
- To assess for myopathic or neurogenic patterns in affected muscles and nerves.
Main Methods:
- Retrospective review of pediatric LS patients from 1997-2013 with consistent follow-up.
- Electromyography (EMG) performed bilaterally on affected and contralateral sides using surface and needle electrodes.
- Analysis of muscle activity patterns (myopathic vs. neurogenic) and nerve conduction studies.
Main Results:
- EMG analysis in nine LS subjects showed asymmetric myopathic patterns in muscles underlying linear streaks in all but one patient.
- Motor and sensory nerve conduction revealed a neurogenic pattern in one subject.
- Masticatory muscle testing demonstrated a myopathic pattern in two cases with head and face involvement.
Conclusions:
- A significant degree of muscle dysfunction was observed in this small series of pediatric LS patients via EMG.
- Muscle involvement in LS may be secondary to peripheral nerve involvement stemming from inflammation and fibrosis.
- Further collaborative studies are necessary to validate these findings and elucidate the relationship between LS, nerve involvement, and muscle dysfunction.
