Systemic Lupus Erythematous and Neuromyelitis Optica Causing Hypercapnic Respiratory Failure

BACKGROUND Neuromyelitis optica (NMO) is a rare neurological disease characterized by attacks of transverse myelitis and optic neuritis, contiguous spinal cord lesions on more than 3 vertebral segments on magnetic resonance imaging (MRI), and seropositivity for AQP-4 Ab. The tissue destruction from NMO is immune mediated and results in demyelination and axonal damage. Optic and spinal nerve involvement can eventually lead to blindness, weakness, and altered consciousness, and bladder and bowel involvement in some cases. CASE REPORT A 54-year-old Black woman presented with chest pain, dysphagia, generalized weakness, diplopia, and paresthesias in her bilateral feet. A brain MRI revealed an area of hyperintensity in the cervical medullary junction. A diagnosis of NMO was made after the treatment response was poor for systemic lupus erythematous (SLE) myelitis. She eventually developed acute hypercapnic respiratory failure, became encephalopathic, and was emergently intubated. She was extubated but had poor recovery and was eventually discharged home. CONCLUSIONS NMO is a rare immune-mediated disease that is often delayed in diagnosis and treatment. Clinical suspicion is important since there is a tendency for the disease to overlap concomitant autoimmune diseases in 25% of cases. Progressive and permanent tissue damage can occur despite the use of high-dose steroids, long-term immunosuppressant agents, immunomodulators, exchange transfusions, and even autologous hematopoietic stem cell bone marrow transplantation.