Muscular atrophy and weakness in proximal parts of lower extremities in Behçet’s disease: A case report and review of the literature

Abstract

Background: In Behçet’s disease (BD), very few cases associated with muscular involvement had been reported previously. The natural history and therapeutic protocol for muscular involvement in BD is obscure due to the low incidence of peripheral neuropathy or myopathy in BD. The purpose of our study was to report a rare case of BD with chronic, focal forms of neuromyopathy and review the relevant literature. Materials and methods: We herein report a 54-year-old male patient, who presented with progressive muscular atrophy and weakness in both thighs two years after the presentation of the cardinal symptoms of BD. The past history taking, electrophysiological study, neurological examination, laboratory test of blood sample, magnetic resonance imaging study, and histological study were performed for differential diagnosis. The relevant literature on muscular involvement in BD was reviewed. Results: Neurological examination revealed that the muscular involvement was predominantly localized to the proximal parts of lower extremities. Heterogeneous enhancement of several thigh muscles was observed upon magnetic resonance imaging study which corresponded with the clinical manifestations. Histological study of one of the enhanced muscles showed denervation atrophy of the muscle with superimposed myopathic changes while electrophysiological study only suggested denervation. Conclusion: To our knowledge, this is the first case of neurogenic muscular atrophy with this specific set of clinical, radiological, electrophysiological and histological findings reported in BD. Despite the rare involvement of muscles, it should be considered particularly in BD patients that muscular symptoms with or without the cardinal symptoms of BD might be correlated with BD.