Thinking at a Spinal Level? A Novel Case of Longitudinal Myelopathy as First Presentation of NPSLE in a Child

Abstract

Introduction: Neuropsychiatric systemic lupus erythematosus (NPSLE) involves central and peripheral nervous systems, producing neurological and psychiatric symptoms in 25–95% of cases. Extensive spinal cord involvement such as longitudinal myelopathy (LM) is exceedingly rare, particularly in pediatric SLE. Case Report: A 13-year-old girl with normal development presented with intermittent fever, arthralgia, malaise, and a malar rash for two months. She subsequently developed progressive weakness, urinary retention, and constipation. Examination revealed pallor, malar rash, oral ulcers, and evolving spastic paraparesis with brisk reflexes and extensor plantars, but intact sensation and coordination. MRI spine demonstrated a T2 hyperintense lesion extending from the cervicomedullary junction to the conus medullaris, consistent with longitudinally extensive myelopathy. Serology was positive for ANA, anti-smith, anti-RNP, and SS-A/RO-52 antibodies, with elevated ESR and CRP. CSF analysis showed mild protein elevation with negative NMO and MOG antibodies. Fundus examination revealed retinal vasculitis with bilateral optic neuritis, while other systemic workups were normal. The patient was diagnosed with SLE-associated longitudinal myelopathy and treated with intravenous methylprednisolone (30 mg/kg/day for 5 days), followed by oral prednisolone and hydroxychloroquine. She demonstrated significant neurological improvement within a week, and at one-month follow-up, urinary retention and joint pains had resolved. Conclusion: Longitudinal myelopathy is an uncommon but severe manifestation of pediatric SLE. Earlyrecognition and aggressive immunosuppressive therapy are crucial for neurological recovery and improved outcomes.