Following the examination and laboratory testing, the patient was admitted under our family medicine service for neuromyelitis optica (NMO) affecting the area postrema. NMO, also known as Devic’s disease, is an autoimmune disorder that affects the spinal cord and optic nerves. Autoantibodies against AQP4 are created in the periphery and are directed against astrocytes in the central nervous system. These antibodies bind to the foot processes of astrocytes, inducing complement-mediated cell damage and granulocyte infiltration.1-5
Intravenous methylprednisolone was initiated at 250 mg every 6 hours for 3 days. A repeat brain MRI demonstrated nonspecific multiple scattered foci of hyperintense signal involving the subcortical supratentorial white matter without abnormal enhancement, most likely representing nonactive demyelinating plaques (FIGURES 1B and 1C).
Dx is revisited. Our patient was referred to an NMO clinic for evaluation. After further testing (including a repeat MRI based on the neurologist’s specifications, anti-aquaporin antibody testing, and MOG-antibody testing) and case discussion, it was determined that the patient had MOG-antibody disease. This disease, along with NMO, comprise a spectrum of disorders referred to as neuromyelitis optica spectrum disorder (NMOSD).
The patient was subsequently prescribed a rituximab infusion, 500 mg/50 mL, to treat the current attack. One infusion was to be completed weekly for 2 weeks with plans to repeat treatment every 6 months to prevent flares of NMO. During the first dose, the patient had a reaction to the treatment, which caused pruritus and chest tightness. She was able to complete the infusion after being treated with diphenhydramine.
Tx continued. In order to complete the second of 2 infusions of rituximab, the patient was pretreated with oral methylprednisolone the night before the infusion, along with diphenhydramine and acetaminophen on the day of treatment. Fortunately, the patient tolerated the infusion well with no adverse effects or reactions.
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