Neuromyelitis optica spectrum disorder with ultra-longitudinally extensive transverse myelitis: A case report and literature review.

BACKGROUND

Neuromyelitis optica spectrum disorders (NMOSD) is characterized by inflammatory demyelinating events in the central nervous system (CNS), primarily affecting the spinal cord and optic nerve, with a significant influence of astrocytes. Longitudinal extensive transverse myelitis (LETM) is a distinct and relatively rare spinal cord syndrome, commonly associated with NMOSD.

CASE PRESENTATION: This report describes a unique case of myelitis in a patient diagnosed with NMOSD. The patient exhibited an uncommon manifestation of ultra- LETM (u-LETM), coexisting with connective tissue disorders including Sjögren's syndrome and autoimmune hepatitis-primary cholestatic cirrhosis. In the acute phase, high-dose methylprednisolone pulse therapy was administered in combination with intravenous human immunoglobulin, while prednisone was gradually tapered and discontinued upon stabilization of the patient's condition. Simultaneously, sequential disease-modifying therapy was initiated, starting with long-term oral administration of mycophenolate mofetil, followed by cyclophosphamide, telitacicept, and Inebilizumab. During follow-up visits conducted every three months, the patient showed gradual improvement, eventually achieving the ability to stand and walk independently.

CONCLUSIONS

Early and comprehensive evaluation of autoimmune diseases is crucial in patients with NMOSD presenting with u-LETM as the initial symptom. Prompt treatment initiation, followed by disease-modifying therapy, is essential for improving patient prognosis.