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

BACKGROUND: In Behçet’s disease (BD), very few cases of muscular involvement have been reported previously. The natural history and therapeutic protocol for muscular involvement in BD are 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. CASE SUMMARY: We herein report the case of a 54-year-old man who presented with progressive muscular atrophy and weakness of both thighs 2 years after the presentation of the cardinal symptoms of BD. The past medical history, electrophysiological study, neurological examination, blood tests, magnetic resonance imaging study, and histological exam were performed for the differential diagnosis. Relevant literature on muscular involvement in BD was reviewed. Neurological examination revealed that muscular involvement was predominantly localized in the proximal parts of the lower extremities. Heterogeneous enhancement of several thigh muscles was observed on magnetic resonance imaging, 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 studies only suggested denervation. CONCLUSION: To our knowledge, this is the first case of neurogenic muscular atrophy with a specific set of clinical, radiological, electrophysiological, and histological findings reported in BD.