Neuronal-specific antibody in patients with multiple system atrophy and progressive supranuclear palsy
Anti-neuronal antibodies can manifest with diverse movement disorders, including parkinsonism and ataxia, and may mimic neurodegeneration. The prevalence of neuronal-specific antibodies in atypical parkinsonism remains unclear. We examined 166 patients, including 136 patients with clinically diagnosed progressive supranuclear palsy (PSP) and 30 patients with multiple system atrophy (MSA) from the Taiwan PSP/CBS Consortium using an integrated set of tissue-based immunofluorescence assays and a subsequent fixed cell-based assay as well as confirmatory tests to detect neuronal-specific antibodies. We observed one PSP-Richardson syndrome patient (0.74 %) was CASPR2-IgG positive, and one MSA-cerebellar subtype patient (3.33 %) showed GAD65 positivity with a hot-cross bun sign on MRI. The latter showed clinical improvement after immunotherapy. Neither patient had sleep-related symptoms. These findings indicate that neuronal-specific antibodies may mimic MSA or PSP and warrant antibody testing in atypical parkinsonism to identify reversible autoimmune causes.
Authors
- Pin‐Shiuan Chen
- Ing‐Tsung Hsiao (ORCID: https://orcid.org/0000-0002-5255-7498)
- Min‐Yu Lan (ORCID: https://orcid.org/0000-0001-7361-8475)
- Jung‐Lung Hsu (ORCID: https://orcid.org/0000-0001-6422-8870)
- Han‐Lin Chiang (ORCID: https://orcid.org/0000-0002-0768-0059)
- Chin‐Hsien Lin (ORCID: https://orcid.org/0000-0001-8566-7573)
- Yung‐Yee Chang (ORCID: https://orcid.org/0000-0001-6840-8537)
- Friederike A. Arlt (ORCID: https://orcid.org/0000-0002-0112-8685)
- Hsiu-Chen Chang (ORCID: https://orcid.org/0000-0002-1837-5041)
- Yih‐Ru Wu (ORCID: https://orcid.org/0000-0003-1191-2542)
- Sung‐Pin Fan (ORCID: https://orcid.org/0000-0001-5332-1922)
- Chun‐Yu Chen (ORCID: https://orcid.org/0000-0003-4284-8918)
- Cheng‐Hsuan Li (ORCID: https://orcid.org/0000-0001-9707-1929)
- Ming‐Chen Tsai (ORCID: https://orcid.org/0000-0002-3452-6567)
- Tsung-Lin Lee (ORCID: https://orcid.org/0009-0001-0076-0363)
- Chin‐Song Lu
- Mingyue Fang (ORCID: https://orcid.org/0009-0000-0237-7731)
Institutions
- Mayo Clinic (US)
- Chang Gung University (TW)
- Taipei Veterans General Hospital (TW)
- Chang Gung Memorial Hospital (TW)
- Taipei Municipal YangMing Hospital (TW)
- Landseed Hospital (TW)
- Kaohsiung Chang Gung Memorial Hospital (TW)
- Linkou Chang Gung Memorial Hospital (TW)
- Mayo Clinic in Arizona (US)
- National Taiwan University Hospital (TW)
- Mayo Clinic in Florida (US)
- National Cheng Kung University Hospital (TW)
Publication Details
- Journal
- Journal of the Formosan Medical Association
- Published
- 2025-10-01
- DOI
- https://doi.org/10.1016/j.jfma.2025.10.036
- Primary Topic
- Autoimmune Neurological Disorders and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 0.00