# Paraneoplastic neuromyelitis optica spectrum disorder with dual AQP4-IgG and CRMP5 antibodies following thymectomy: a case report

**Authors:** Yucai Chuai, Tao Jin, Lizhi Zhang, Honglu Song

PMC · DOI: 10.3389/fnins.2026.1747586 · Frontiers in Neuroscience · 2026-03-09

## TL;DR

A rare case of a neurological disorder linked to a thymoma showed dual antibodies, suggesting a unique autoimmune mechanism.

## Contribution

Highlights a rare case of paraneoplastic NMOSD with dual AQP4-IgG and CRMP5 antibodies following thymoma.

## Key findings

- Patient showed high AQP4-IgG titer and positive CRMP5 antibodies without other paraneoplastic markers.
- Treatment led to significant visual recovery in a patient with optic neuritis.
- Suggests a unique autoimmune mechanism involving both AQP4 and CRMP5 antibodies in thymoma-associated NMOSD.

## Abstract

We present a rare case of paraneoplastic neuromyelitis optica spectrum disorder (NMOSD) in a 50-year-old woman with a history of B3 thymoma, marked by dual positivity for AQP4-IgG and CV2/CRMP5 antibodies. The patient developed sequential bilateral optic neuritis, with orbital MRI revealing enhancement of the left optic nerve. Serological analysis showed a high AQP4-IgG titer (1:320) and positive CV2/CRMP5 antibodies, while other paraneoplastic markers were absent. Treatment with glucocorticoids and mycophenolate mofetil led to substantial visual recovery. This case underscores the association between thymoma and paraneoplastic NMOSD and suggests a potential interaction between AQP4- and CRMP5-related autoimmune responses. Although CV2/CRMP5 antibodies are typically linked to lung cancer and thymoma, their coexistence with AQP4-IgG in NMOSD points to a distinct paraneoplastic mechanism. These findings support the need for thorough tumor screening in NMOSD patients with atypical features, particularly those with CV2/CRMP5 seropositivity.

## Linked entities

- **Diseases:** thymoma (MONDO:0006456)

## Full-text entities

- **Genes:** AQP4 (aquaporin 4) [NCBI Gene 361] {aka MIWC, MLC4, WCH4, hAQP4}, DPYSL5 (dihydropyrimidinase like 5) [NCBI Gene 56896] {aka CRAM, CRMP-5, CRMP5, CV2, RTSC4, Ulip6}, BMPER (BMP binding endothelial regulator) [NCBI Gene 168667] {aka CRIM3, CV-2, CV2}
- **Diseases:** paraneoplastic (MESH:D010257), optic neuritis (MESH:D009902), autoimmune (MESH:D001327), lung cancer (MESH:D008175), tumor (MESH:D009369), NMOSD (MESH:D009471), thymoma (MESH:D013945)
- **Chemicals:** mycophenolate mofetil (MESH:D009173)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC13006573/full.md

## References

15 references — full list in the complete paper: https://tomesphere.com/paper/PMC13006573/full.md

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Source: https://tomesphere.com/paper/PMC13006573