RESUMO
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.
Assuntos
Deformidades Adquiridas do Pé/prevenção & controle , Deformidades Adquiridas da Mão/prevenção & controle , Músculo Esquelético , Atrofia Muscular , Esclerodermia Localizada , Pré-Escolar , Estudos Transversais , Diagnóstico Precoce , Intervenção Médica Precoce , Eletromiografia/métodos , Feminino , Deformidades Adquiridas do Pé/etiologia , Deformidades Adquiridas da Mão/etiologia , Humanos , Masculino , Músculo Esquelético/inervação , Músculo Esquelético/patologia , Músculo Esquelético/fisiopatologia , Atrofia Muscular/diagnóstico , Atrofia Muscular/etiologia , Atrofia Muscular/fisiopatologia , Condução Nervosa/fisiologia , Avaliação de Resultados em Cuidados de Saúde , Estudos Retrospectivos , Esclerodermia Localizada/complicações , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/fisiopatologia , Esclerodermia Localizada/terapiaAssuntos
Deformidades Adquiridas do Pé/classificação , Deformidades Adquiridas do Pé/diagnóstico , Deformidades Adquiridas do Pé/fisiopatologia , Deformidades Adquiridas do Pé/patologia , Deformidades Adquiridas do Pé/prevenção & controle , Deformidades Adquiridas do Pé/reabilitação , Deformidades Adquiridas do Pé/terapia , Hanseníase/reabilitaçãoRESUMO
En el presente trabajo, después de una breve introduccion sobre la rehabilitacion del enfermo con lepra, se hace referencia a generalidades de la enfermedad, como: origen de la enfermedad en México, número de enfermos en la Republica Mexicana (se consideran de 50 a 60,000), clasificacion, y dos grupos: indeterminado y dimorfo. Se estudiam las incapacidades físicas y psicosociales que la enfermedad produce a las personas afectadas; las físicas de localizacion en las manos, pies y cara, principalmente y las incapacidades psicosociales desde el punto de vista de como afectam a la persona y sus familiares en los diferentes aspectos: psiquico, economico y social