Transverse myelitis in Beh?et's disease: A series of four cases and review of the literature

摘要

Objectives. Transverse myelitis (TM) is an uncommon neurologic condition characterised by the segmental involvement of the spinal cord. Although its etiology is unknown, a well established list of associations have been described, many items of which point towards an autoimmune and vasculitic process. TM is also a rare complication of Beh?et's disease (BD), an autoimmune process. Herein we present 4 cases of TM associated with BD. Methods. Retrospective chart reviews of 104 patients were done. Diagnosis of BD was established in each case according to the diagnostic criteria established by the International Study Group for Beh?et's Disease. Demographic data, clinical and radiologic presentations of TM, treatment strategies and outcomes were obtained from hospital records. Results. Among the 15 patients with neurological involvement, four cases (3 male, 1 female) of acute TM associated with BD were confirmed. TM associated with BD affected cervical and thoracic levels of the spinal cord. Myelitis involved multiple segments (4 cases). TM developed at any stage of the disease, even as a dramatic initial presentation, leading to the diagnosis of BD (1 case). The longest time elapsed since the diagnosis of BD prior to the development of TM was 10 years. A major association was the history of panuveitis in all four cases. Cyclophosphamide and steroid therapy were the mainstay of the treatment once the TM had developed. Treatment outcomes were variable, depending mostly on the severity of the neurologic involvement at presentation, timing of the initiation of the therapy and patients adherence with the treatment. Two of 4 cases recovered without major sequela. Conclusion. Although rare, the presented association and its detailed clinical discussions would serve to enhance our understanding of both TM and the spectrum of neurologic complications that BD may harbour. Early recognition and initiation of therapy are crucial for successful outcome.