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.

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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

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article

Case Report: Anti-neural cell adhesion molecule 1 antibody-positive encephalitis presenting with schizophrenia-like symptoms and an ovarian teratoma

Saori Toyoda, Masayuki Nakamori, Yukiko Motokawa, Nanami Yamanaka et al.
Frontiers in Immunology
Autoimmune Neurological Disorders and Treatments
article

Case Report: Anti-neural cell adhesion molecule 1 antibody-positive encephalitis presenting with schizophrenia-like symptoms and an ovarian teratoma

Saori Toyoda, Masayuki Nakamori, Yukiko Motokawa, Nanami Yamanaka, Makoto Hara, Fumitaka Shimizu, Masaya Honda, Hiroki Shiwaku, Michiaki Koga, Shusaku Tomita
article en

Abstract

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.

Frontiers in ImmunologyVol. 17
Nihon University (JP), Yamaguchi University (JP), Institute of Behavioral Sciences (JP), National Hospital Organization (JP)
Japan Agency for Medical Research and Development, Japan Society for the Promotion of Science
Good health and well-being
Openalex Percentile: Top 72%
Autoimmune Neurological Disorders and Treatments
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