Neuromyelitis optica spectrum disorder in a patient with Kikuchi-Fujimoto disease.

Abstract

A 26-year-old African American woman was admitted for intractable nausea and vomiting during her third trimester of pregnancy. The patient had been diagnosed with Kikuchi-Fujimoto disease (KFD) a year prior, when she presented with high fevers and cervical lymphadenopathy. Lymph node biopsy revealed necrotic foci of predominantly CD8-positive T cells and myeloperoxidase-positive histiocytes consistent with necrotizing lymphadenitis. The patient became pregnant within months of the diagnosis. At 33 weeks' gestation, the patient was given a short course of steroids for myalgias and joint pains that were thought to be due to KFD. This improved her symptoms. Three weeks later, she was admitted for headaches, hypertension, nausea, and intractable vomiting. A diagnosis of preeclampsia was made, labor was induced, and she had an uncomplicated vaginal delivery. The following day, the patient complained of “shaky” and “jumpy” vision, severe headache, weakness, and paresthesias in the lower extremities. Her blood pressure was 170/100 mm Hg. Examination revealed rapid, involuntary eye movements with both horizontal and vertical components consistent with opsoclonus, severe paraparesis, absent reflexes in the lower extremities, and a possible T10 sensory level. Brain MRI revealed hyperintensities in bilateral occipital lobes suggestive of posterior reversible encephalopathy syndrome (PRES) along with a lesion in the medulla (figure, A and B). Spine MRI revealed an extensive cord lesion spanning from C5 to T10 spinal segments consistent with transverse myelitis (figure, C). CSF and serum IgG–neuromyelitis optica antibodies, otherwise known as aquaporin-4 antibodies, were positive; rheumatologic markers, serum and CSF viral markers, cultures, and paraneoplastic autoantibodies were negative. The patient's complaints of abnormal vision and headache resolved with blood pressure control. However, the paraparesis did not improve despite treatment with high-dose IV Solu-Medrol and IVIg. She slowly regained strength over several weeks after receiving treatment with plasmapheresis and continued high-dose prednisone 60 mg/d.