Dados do Trabalho

Título

Catastrophic Lupus Myelitis - a rare case report.

Descrição do caso

Myelopathys are diagnosis criteria for Systemic Lupus Erythematosus (SLE) and occurs in 2 out 100 patients. Extensive myelitis is a rare manifestation with few cases in the literature. We report a catastrophic presentation of lupus myelitis. A 45-years-old women presented with severe leg pain and flaccid paraplegia. She was investigating inflammatory arthritis, but without diagnosis. The disease ascended cranial in the first seventy two hours, with bulbar involvement and acute respiratory failure. Advanced airway acces was obtained. Magnetic Ressonance Image showed myelitis with involvement of cauda equina, extensive involvement of cervical segments and in rostral part of the medulla oblongata, with high signal intensities in T2-weighted and FLAIR sagittal midline sequences, as well as in focals areas of cerebellum. Cerebrospinal fluid contained 440 cells (54% neutrophils), glucose 20 mg/dl and protein 291. She was treated empirically with ceftriaxone, ampicillin and methyprednisolone. Leukopenia, positive antinuclear antibodies 1/640, low complement and high titer serum anti-DNA positivity allowed the diagnoses of active systemic lupus erythematosus. Plasma exchange for five sessions was added. Cyclophosphamide was postponed due to worsening infection. The hyperacute, catastrophic onset with the development of total neurological deficit predictes a poor outcome, but the patient improve with capacity of breathe without assistance and partially recovered strength in the upper and lower limbs.