Gastrointestinal dysfunction associated with autoimmune glial fibrillary acidic protein astrocytopathy.

Authors

Li Xiao, Bing Qin, Yuefei Guo, Ping Zhang, Yaxin Lu, Yuge Wang, Wendong Lai, Jianning Chen, Bilun Ke, Lijun Huang, Jie Sun, Wei Qiu, Yuhang Pan, Youming Long, Yaqing Shu

Published in

Journal of neurology. Volume 273. Issue 9. Aug 09, 2026. Epub Aug 09, 2026.

Abstract

Gastrointestinal (GI) dysfunction has been reported in autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). However, GI dysfunction in GFAP-A has not been sufficiently investigated. This study aimed to analyze the clinical features of GFAP-A patients with GI dysfunction and explore potential pathogenic mechanisms.
From January 1, 2016, to April 30, 2024, patients diagnosed with GFAP-A based on CSF GFAP-IgG positivity at the Third Affiliated Hospital of Sun Yat-sen University were enrolled. Clinical parameters and GI dysfunction assessed by the I-FEED scoring system were collected. Pathological examination was performed in a subset of patients. And the preliminary role of GFAP-IgG in the enteric plexus was investigated.
Among 92 enrolled individuals (male:female = 68:24; median age 39.5 years), gastrointestinal dysfunction occurred in 72 cases (78.3%). Manifestations included constipation in 38 cases (41.3%), paralytic ileus in 30 cases (32.6%), fecal incontinence in 3 cases (3.26%), and diarrhea in 1 case (1.09%). Severe GI dysfunction (I-FEED score ≥ 6) was associated with higher disease severity (admission mRS 5.0 vs. 3.0, p < 0.001), longer hospitalization (21.5 vs. 15.5 days, p = 0.029), increased ICU admission (50.0% vs. 10.5%, p < 0.001), higher rates of respiratory failure, autonomic dysfunction, and infections, worse CSF profiles (elevated protein, leukocytosis, hypoglycorrhachia). Pathological analysis revealed prominent CD8⁺ and CD4⁺ T-cell infiltration in the colonic wall and a specific loss of GFAP⁺ enteric glia. Furthermore, we demonstrated that anti-GFAP-IgG derived from the cerebrospinal fluid (CSF) of GFAP-A patients binds specifically to the enteric plexus within mouse colonic tissues.
Our findings collectively suggest that severe GI dysfunction could be a marker of disease severity and poor prognosis in GFAP-A, and indicate that its potential mechanism may involve the specific binding of anti-GFAP-IgG to the enteric plexus and subsequent enteric glia loss.

PMID:
42571679
Bibliographic data and abstract were imported from PubMed on 09 Aug 2026.

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