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Aquaporin-4 Positive Neuromyelitis Optica in a Pat ...
Aquaporin-4 Positive Neuromyelitis Optica in a Patient With Sle Following Vaccination: A Case Report
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This case report describes a 31-year-old woman with systemic lupus erythematosus (SLE) who developed rapidly progressive neurologic symptoms after stopping mycophenolate and shortly after receiving shingles and pneumococcal vaccines. She presented with ascending numbness, weakness, urinary retention, and progressed to flaccid quadriparesis with severe sensory loss below the neck, without signs of an SLE flare.<br /><br />MRI of the spine showed a longitudinally extensive transverse myelitis lesion extending from the cervicomedullary junction through the thoracic cord, with patchy enhancement. Cerebrospinal fluid revealed marked pleocytosis and elevated protein, and both serum and CSF were positive for aquaporin-4 antibodies, confirming neuromyelitis optica spectrum disorder (NMOSD).<br /><br />The report emphasizes that NMOSD can coexist with SLE and may resemble lupus-associated myelitis, making early recognition essential. Because treatment and relapse risk differ, longitudinally extensive transverse myelitis in patients with SLE should prompt aquaporin-4 antibody testing. Rapid treatment with IV steroids, plasma exchange, and long-term immunotherapy is important to reduce the risk of irreversible neurologic disability.<br /><br />The patient was treated with 5 days of IV methylprednisolone, 7 sessions of plasma exchange, and rituximab for maintenance, then discharged to inpatient rehabilitation.
Asset Subtitle
Shane Fuentes
Meta Tag
Author List
Nadir Bhuiyan, Shane Fuentes
Category
Clinical Vignettes
Concept
Neuromyelitis Optica Spectrum Disorder
Concept
Longitudinally Extensive Transverse Myelitis
Concept
Aquaporin-4 Antibody
Concept
Spinal Cord Lesion
Concept
Lupus-associated Myelitis
Distinguished
Non-Finalist
Presenter Organization
Mayo Clinic
Presenting Author
Shane Fuentes
Track
Adult
Keywords
systemic lupus erythematosus
neuromyelitis optica spectrum disorder
aquaporin-4 antibodies
longitudinally extensive transverse myelitis
mycophenolate discontinuation
shingles vaccine
pneumococcal vaccine
quadriparesis
plasma exchange
rituximab
Neuromyelitis Optica Spectrum Disorder
Longitudinally Extensive Transverse Myelitis
Aquaporin-4 Antibody
Spinal Cord Lesion
Lupus-associated Myelitis
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