Longitudinally extensive transverse myelitis in systemic lupus erythematosus: case report and review of the literature

Abstract

Objective: To report a case of longitudinally extensive transverse myelitis (LETM), a rare but disabling condition defined as a lesion of the spinal cord that extends over four or more vertebrae on MRI, in association with systemic lupus erythematosus (SLE).

Methods: We present a rare case of LETM involving the cervical and thoracic spinal cord in a patient with SLE and review the existing literature on the association of lupus-associated myelitis.

Results: LETM is included within the diagnostic criteria for Neuromyelitis Optica (NMO), but is also known to be associated with a wide range of auto-immune diseases. Only 37 cases of LETM in patients with SLE have been previously described. We performed an updated review on epidemiology, pathophysiology, clinical features, diagnosis, management, and prognosis of LETM in the setting of SLE.

Conclusion: Due to the generally poor prognosis of LETM in SLE patients, prompt diagnosis and treatment is of critical importance for a positive clinical outcome. We provide a comprehensive perspective of past and current literature in order to aid diagnosis and management of this rare phenomenon.

Keywords: Longitudinally extensive transverse myelitis; Neuromyelitis optica; Neuromyelitis optica spectrum disorders; Systemic lupus erythematosus; Trasverse myelitis.