Case Report: Anti-neural cell adhesion molecule 1 antibody-positive encephalitis presenting with schizophrenia-like symptoms and an ovarian teratoma
Autoimmune encephalitis (AE) has been linked to various autoantibodies, with anti-neural cell adhesion molecule 1 (NCAM1) antibodies recently identified in patients with schizophrenia. Their pathogenic potential has been demonstrated in passive transfer experiments in mice. We report the first case of AE associated with anti-NCAM1 antibodies in a 15-year-old female who presented with acute-onset psychiatric symptoms, including delusions, hallucinations, and catalepsy, along with fever and impaired consciousness. Cerebrospinal fluid analysis showed a high IgG index, and electroencephalography revealed generalized slow-wave activity. However, brain magnetic resonance imaging was unremarkable. A pelvic tumor was identified, and adnexectomy confirmed a right mature cystic teratoma. Given the clinical presentation, anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was initially suspected, and the patient was treated with intravenous methylprednisolone and intravenous immunoglobulin, resulting in significant improvement. Despite a subsequent relapse of hallucinations, additional immunotherapy led to complete resolution of symptoms. Notably, anti-NMDAR and other well-known neuronal surface antibodies were negative in both serum and cerebrospinal fluid, whereas anti-NCAM1 antibodies were detected. Immunohistochemical analysis confirmed NCAM1 expression in the excised ovarian tissue, and post-treatment serum tests showed the disappearance of anti-NCAM1 antibodies. The concurrent resolution of clinical symptoms and the disappearance of these antibodies support their pathogenic role. The clinical presentation and paraneoplastic association with teratoma closely resemble anti-NMDAR encephalitis, suggesting that anti-NCAM1 antibodies may define a novel form of AE with psychotic manifestations. Further research is needed to clarify their role in neuroinflammatory disorders.
Authors
- Saori Toyoda
- Masayuki Nakamori (ORCID: https://orcid.org/0000-0003-4714-3044)
- Yukiko Motokawa
- Nanami Yamanaka (ORCID: https://orcid.org/0009-0007-5017-8711)
- Makoto Hara (ORCID: https://orcid.org/0000-0001-8893-6847)
- Fumitaka Shimizu (ORCID: https://orcid.org/0000-0003-4163-2939)
- Masaya Honda
- Hiroki Shiwaku
- Michiaki Koga
- Shusaku Tomita
Institutions
- Nihon University (JP)
- Yamaguchi University (JP)
- Institute of Behavioral Sciences (JP)
- National Hospital Organization (JP)
Publication Details
- Journal
- Frontiers in Immunology
- Published
- 2026-01-30
- DOI
- https://doi.org/10.3389/fimmu.2026.1587199
- Primary Topic
- Autoimmune Neurological Disorders and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Japan Agency for Medical Research and Development
- Japan Society for the Promotion of Science