A cross-sectional electromyography assessment in linear scleroderma patients

Abstract

BackgroundMuscle 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.MethodsWe 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.ResultsNine 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.ConclusionIn 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.

A clinical study on linear dermatoses at a teriary care teaching hospital in Davangere

“…5 In our study, segmental vitiligo was found to affect younger age group which is comparable with the studies by Van et al and Ratnam et al 6,7 A female preponderance was observed in our study of Linear Dermatoses. This finding in our study in patients with linear morphea correlated with the study by Claudia Saad et al and Mukhapadhyay et al 8,9 With Lichen striatus a similar female preponderance was observed in our study. This is similar to Kerstin T Abagge et al who observed a female: male ratio of 3: 1.10…”

Diagnostic criteria, severity classification and guidelines of localized scleroderma

“…One theory suggests that adipose tissue and muscle atrophy in linear scleroderma is caused by sympathetic nervous system disorders, and there has been exhaustive investigation on the muscle and nerve lesions that are found in this disease. Saad Magalhaes et al 126 evaluated electromyograms for nine patients with juvenile linear scleroderma (linear scleroderma/ limbs, n = 7; Parry-Romberg syndrome, n = 2) and found myogenic changes in the muscle underlying the lesions in eight patients (both patients with Parry-Romberg syndrome had myogenic changes in the masticatory muscles), while one patient had neurogenic changes. In an investigation on the conduction velocity of limb motor neurons and sensory nerves, they reported that they found abnormalities in a patient with linear scleroderma in whom a lesion extended deep into the thigh.…”

A case of overlapping adult‐onset linear scleroderma and Parry‐Romberg syndrome presenting with widespread ipsilateral neurogenic involvement

“…Therefore, their clinical manifestations were different from those of our patient, who had widespread muscle weakness. Because central and peripheral nerve involvement is frequently seen in localized scleroderma, we speculate that linear scleroderma may occasionally present with neurogenic muscle weakness independent of skin lesions, as in the one case described by Magalhães et al It remains unknown whether muscle findings in linear scleroderma differ according to the presence or absence of PRS.…”

“…Few studies have examined EMG in patients with liner scleroderma or PRS. Only one report examined needle EMG in a series of patients with linear scleroderma, including two PRS cases . In that study, eight of nine pediatric patients with linear scleroderma, including two with concomitant PRS, showed a myogenic pattern in the muscles underlying the extremity skin lesions on needle EMG; the remaining one patient with linear scleroderma alone had a neurogenic pattern.…”