Longitudinal extensive transverse myelitis in systemic lupus erythematosus – a case report and review of literature

Abstract

Longitudinal extensive transverse myelitis (LETM) is rather rare life threatening complication of systemic lupus erythematosus (SLE). It is defined as an inflammatory spinal cord lesion that extends over three or more adjacent vertebral segments. Clinical presentation can consist of paraparesis or tetraparesis, sensory disturbances and vegetative dysfunction, and respiratory failure may follow in severe cases. A female patient aged 28 years, diagnosed with SLE 11 years ago, has been herein presented, whose disease was complicated by LETM affecting cervical and thoracic spinal cord. LETM occurred after the period of stable remission, and was followed by clinical, as well as previously serologically active disease. In the context of LETM the patient developed tetraparesis and high hypoesthesia, while respiratory muscle paralysis and consecutive respiratory failure required mechanical ventilation. After the exclusion of underlying intracranial infection, combined immunosuppressive therapy has been used resulting in a gradual but partial neurological recovery, and an overall neuroradiological improvement. LETM is an uncommon but severe complication of SLE leading to serious neurological sequelae, and often resulting even in fatal otcome. Along with herein presented case report, current available findings on pathogenesis, diagnosis and therapheutic approach in the context of neuropsychiatric lupus have been depicted through the review of the literature.

Keywords

• lupus erythematous
• systemic – complications
• diagnosis
• drug therapy; myelitis
• transverse – diagnosis
• drug therapy
• etiology; lupus vasculitis
• central nervous system – diagnosis
• etiology; immunosuppressive agents – therapeutic use; quadriplegia – etiology; respiratory insufficiency – etiology; antibodies