Clinical Report: Optic Neuritis as Initial Presentation of IgG4 Anti-NF155-Positive CCPD

Overview

This case study presents a young male with severe optic neuritis as the initial manifestation of biopsy-confirmed combined central and peripheral demyelination (CCPD) associated with anti-NF155 antibodies. The findings emphasize the importance of anti-NF155 antibody testing in patients with optic neuritis and peripheral neuropathy.

Background

Combined central and peripheral demyelination (CCPD) is a rare neuroimmune disorder characterized by concurrent demyelination in both the central and peripheral nervous systems. The clinical manifestations can vary widely, and optic neuritis may be an initial presentation. Identifying specific autoantibodies, such as IgG4 anti-NF155, is crucial for diagnosis and management.

Data Highlights

No numerical data or trial data provided in the article.

Key Findings

• A 21-year-old male presented with severe optic neuritis and was positive for anti-NF155 antibodies.
• His clinical course included steroid dependence and relapse upon treatment discontinuation.
• Histopathological examination confirmed the diagnosis of CCPD.
• Initial treatment with intravenous methylprednisolone showed limited efficacy on visual impairment.
• Sustained remission was achieved with corticosteroid pulse therapy combined with rituximab.

Clinical Implications

The case highlights the need for anti-NF155 antibody testing in patients presenting with optic neuritis and peripheral neuropathy. Effective management strategies may require aggressive immunomodulation, particularly in cases resistant to standard treatments.

Conclusion

This case underscores the potential for optic neuritis to be the initial manifestation of CCPD and the importance of timely diagnosis and treatment.

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