Neuromyelitis optica

Clinical vignette
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By Tiffani Stroup DO and Adil Javed MD PhD

An 18-year-old African-American woman with a past medical history of sickle cell trait and asthma presented 1 month postpartum with acute triparesis with bladder involvement and a T4 sensory level. She was found to have a longitudinally extensive transverse myelitis extending from the medulla to C7. Her brain MRI was normal. Acutely, she was treated with IV steroids and plasma exchange, and she had some residual weakness. She was placed on a prolonged steroid taper and azathioprine for prophylaxis. The following year, she developed 2 episodes of right optic neuritis and another episode of transverse myelitis. MRI showed enhancement of the right optic nerve as well as enhancement and increased T2 signal from C3-T11 on spinal MRI. Three years later, she developed optic neuritis affecting the optic chiasm and both optic nerves. The following year, she developed another transverse myelitis. Two years later, she had recurrent thoracic transverse myelitis. She was treated each time with IV steroids, IVIG, and/or plasma exchange, with minimal recovery. Her clinical course was complicated by neurogenic bladder, constipation, and frequent urinary tract infections, which often preceded her relapses. Prophylactically, she has been on azathioprine, mycophenolate mofetil, mitoxantrone, IVIG, and rituximab. Eleven years after her initial presentation, she is wheelchair-bound due to paraplegia, is blind in the right eye, and has severely decreased visual acuity in the left eye.

This case illustrates that neuromyelitis optica is a syndrome that leads to significant disability. Many of the patient’s attacks were prompted by urinary tract infections, which is very common. She was treated with appropriate acute and prophylactic therapies. However, she continued to have disease activity. Clinical trials for new therapeutic options are needed for patients such as the one described above who continue to have disease activity despite current therapies.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited