Frequency and Phenotype of Coexisting Aquaporin‐4 and Autoimmune Encephalitis Autoantibodies
Among 9457 aquaporin-4 (AQP4) immunoglobulin G-positive patients, 2071 underwent autoimmune encephalitis antibody testing and 85 (4%) had coexisting antibodies, most commonly cerebrospinal fluid glial fibrillary acidic protein-IgG and N-methyl-D-aspartate receptor-IgG, or serum high-titer GAD65-IgG. Compared with AQP4-IgG positive neuromyelitis optica spectrum disorder controls, dual-positive patients were younger and more frequently exhibited encephalopathy, psychiatric symptoms, seizures, ovarian teratomas, and deep gray matter, temporal lobe, or diencephalic MRI abnormalities. GAD65-IgG-positive patients developed stiff-person syndrome. These findings define a distinct mixed glial-neuronal autoimmune phenotype and support additional neural antibody testing in atypical AQP4-IgG-positive presentations.
Authors
- Amy C. Kunchok (ORCID: https://orcid.org/0000-0002-8387-6491)
- Jessica A. Sagen (ORCID: https://orcid.org/0000-0002-1986-6352)
- Thomas Shoemaker (ORCID: https://orcid.org/0000-0002-1085-6860)
- Binxia Yang
- Jacqueline A. Nicholas (ORCID: https://orcid.org/0000-0002-6328-1941)
- Αναστασία Ζεκερίδου (ORCID: https://orcid.org/0000-0002-1960-9065)
- Laura Cacciaguerra (ORCID: https://orcid.org/0000-0001-9342-2410)
- Georgios Mangioris (ORCID: https://orcid.org/0000-0001-5051-1739)
- Eoin P. Flanagan (ORCID: https://orcid.org/0000-0002-6661-2910)
- Emmanuelle L. Waubant (ORCID: https://orcid.org/0000-0001-5188-0157)
- Wendy G. Mitchell (ORCID: https://orcid.org/0000-0001-8082-1383)
- Kurt M. Sieloff
- Sarosh R. Irani (ORCID: https://orcid.org/0000-0002-7667-9748)
- Andrew B. McKeon (ORCID: https://orcid.org/0000-0001-6856-8143)
- Vanda A. Lennon (ORCID: https://orcid.org/0000-0003-1910-1576)
- Karen S. Fernández (ORCID: https://orcid.org/0000-0002-0097-9293)
- William R. Meador
- Amy M.L. Quek (ORCID: https://orcid.org/0000-0002-0569-0472)
- Nusrat Ahsan (ORCID: https://orcid.org/0000-0002-3502-8658)
- Shruti Agnihotri (ORCID: https://orcid.org/0000-0002-2461-0255)
- Sean Joseph Pittock (ORCID: https://orcid.org/0000-0002-6140-5584)
- Erin McLeod
- Matt C. Edmond
- Rebekah A. Laggis
- John J. Chen
- Kristin L. Flynn
Institutions
- University of Southern California (US)
- Rush University Medical Center (US)
- Cleveland Clinic (US)
- Mayo Clinic (US)
- Children's Hospital of Los Angeles (US)
- National University of Singapore (SG)
- University of California, San Francisco (US)
- University of Michigan (US)
- Riverside Methodist Hospital (US)
- University of Alabama at Birmingham (US)
- Center for Cancer and Blood Disorders (US)
- Mayo Clinic in Florida (US)
- UAB Medicine (US)
Publication Details
- Journal
- Annals of Clinical and Translational Neurology
- Published
- 2026-11-04
- DOI
- https://doi.org/10.1002/acn3.70541
- Primary Topic
- Autoimmune Neurological Disorders and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 0.00