Delayed diagnosis and misdiagnosis of autoimmune glial fibrillary acidic protein astrocytopathy: a dual-center retrospective cohort study in China
- 1Department of Neurology, Yuebei People's Hospital
- 2Department of Neurology, Shantou Central Hospital
Abstract
Objective: To analyze the misdiagnosis and delayed diagnosis of patients with GFAP-Astrocytopathy and to identify the core underlying reasons.
Methods: A total of 42 patients diagnosed with GFAP-Astrocytopathy from July 2020 to March 2026 in two tertiary hospitals were retrospectively included. The patients were defined as having delayed diagnosis if the time from the onset of the initial symptom to the diagnosis was >2 weeks. The misdiagnosis was defined as an incorrect initial diagnosis before the final diagnosis that prompted disease-specific treatment or caused clinically meaningful delay (>7 days) before GFAP-Astrocytopathy was considered, unless autoimmune etiologies were documented and GFAP-IgG testing was ordered within 72 h. Based on this, the patients were divided into the delayed diagnosis group ( n = 27) and the non-delayed diagnosis group ( n = 15).
Results: Among the 42 patients, 64.29% (27/42) experienced diagnostic delay, and 80.95% (34/42) were misdiagnosed. The most common misdiagnosis was infectious meningoencephalitis, accounting for 23 cases (54.76%). The proportion of patients first diagnosed in the neurology department was 25.93%(7/27) in the delayed diagnosis group and 53.33%(8/15) in the non-delayed diagnosis group, χ ² = 3.115, P = 0.039. The non-delayed diagnosis group had significantly higher proportions of neck stiffness and impaired consciousness ( P < 0.05). The proportion of patients presenting with only isolated fever and/or headache without any localizing neurological syndrome at onset was higher in the delayed diagnosis group [59.26% (16/27) vs. 33.33% (5/15); Fisher's exact test, two-sided P = 0.124], but the difference was not statistically significant between the groups. At the time of initial medical consultation, the absence of any localizing neurological syndrome (meningeal, encephalic, myelitic, autonomic, or optic) remained significantly more frequent in the delayed diagnosis group [59.26% (16/27)] compared with the non-delayed diagnosis group [13.33% (2/15), Fisher's exact test, two-sided P = 0.004].
Conclusion: Patients with GFAP-Astrocytopathy have a high rate of delayed diagnosis and are frequently misdiagnosed, most commonly with infectious meningoencephalitis. Initial presentation to a non-neurology department was a key factor contributing to diagnostic delay. The absence of localizing neurological syndromes at onset and initial consultation, delayed GFAP-IgG testing, overlapping CSF inflammatory profiles with infectious etiologies, and absence of perivascular radial enhancement on early brain MRI were significantly associated with delayed diagnosis. Concurrent GFAP-IgG testing at first lumbar puncture was associated with a shorter time to diagnosis, though causality cannot be inferred from this observational association.
Related
- Treatment-refractory autoimmune glial fibrillary acidic protein astrocytopathy: Clinical features, predictive models, therapy
- Glial Fibrillary Acidic Protein Astrocytopathy Mimicking Wernicke's Encephalopathy
- Case report: When encephalitis is not an infection: two cases of autoimmune GFAP astrocytopathy mimicking infectious encephalitis
- Scabies Diagnosis: A Narrative Review of Diagnostic Methods, Clinical Misdiagnosis, and Emerging Directions
- A Rapid Pathological Diagnosis Based on Unfixed Frozen Tissue Section Leads to the Misdiagnosis of Eosinophilic Myocarditis as Lymphocytic Myocarditis
BibTeX
@article{lin2026delayed,
title = {{Delayed diagnosis and misdiagnosis of autoimmune glial fibrillary acidic protein astrocytopathy: a dual-center retrospective cohort study in China}},
author = {Sheng Lin and Yiqian Huang and Chuming Huang and Xiujun Zheng},
journal = {Frontiers in Neurology},
year = {2026},
month = oct,
doi = {10.3389/fneur.2026.1968815},
url = {https://www.frontiersin.org/articles/10.3389/fneur.2026.1968815/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Delayed diagnosis and misdiagnosis of autoimmune glial fibrillary acidic protein astrocytopathy: a dual-center retrospective cohort study in China
AU - Sheng Lin
AU - Yiqian Huang
AU - Chuming Huang
AU - Xiujun Zheng
PY - 2026
DA - 2026/10/23
JO - Frontiers in Neurology
DO - 10.3389/fneur.2026.1968815
UR - https://www.frontiersin.org/articles/10.3389/fneur.2026.1968815/full
AB - Objective: To analyze the misdiagnosis and delayed diagnosis of patients with GFAP-Astrocytopathy and to identify the core underlying reasons. Methods: A total of 42 patients diagnosed with GFAP-Astrocytopathy from July 2020 to March 2026 in two tertiary hospitals were retrospectively included. The patients were defined as having delayed diagnosis if the time from the onset of the initial symptom to the diagnosis was >2 weeks. The misdiagnosis was defined as an incorrect initial diagnosis before the final diagnosis that prompted disease-specific treatment or caused clinically meaningful delay (>7 days) before GFAP-Astrocytopathy was considered, unless autoimmune etiologies were documented and GFAP-IgG testing was ordered within 72 h. Based on this, the patients were divided into the delayed diagnosis group ( n = 27) and the non-delayed diagnosis group ( n = 15). Results: Among the 42 patients, 64.29% (27/42) experienced diagnostic delay, and 80.95% (34/42) were misdiagnosed. The most common misdiagnosis was infectious meningoencephalitis, accounting for 23 cases (54.76%). The proportion of patients first diagnosed in the neurology department was 25.93%(7/27) in the delayed diagnosis group and 53.33%(8/15) in the non-delayed diagnosis group, χ ² = 3.115, P = 0.039. The non-delayed diagnosis group had significantly higher proportions of neck stiffness and impaired consciousness ( P < 0.05). The proportion of patients presenting with only isolated fever and/or headache without any localizing neurological syndrome at onset was higher in the delayed diagnosis group [59.26% (16/27) vs. 33.33% (5/15); Fisher's exact test, two-sided P = 0.124], but the difference was not statistically significant between the groups. At the time of initial medical consultation, the absence of any localizing neurological syndrome (meningeal, encephalic, myelitic, autonomic, or optic) remained significantly more frequent in the delayed diagnosis group [59.26% (16/27)] compared with the non-delayed diagnosis group [13.33% (2/15), Fisher's exact test, two-sided P = 0.004]. Conclusion: Patients with GFAP-Astrocytopathy have a high rate of delayed diagnosis and are frequently misdiagnosed, most commonly with infectious meningoencephalitis. Initial presentation to a non-neurology department was a key factor contributing to diagnostic delay. The absence of localizing neurological syndromes at onset and initial consultation, delayed GFAP-IgG testing, overlapping CSF inflammatory profiles with infectious etiologies, and absence of perivascular radial enhancement on early brain MRI were significantly associated with delayed diagnosis. Concurrent GFAP-IgG testing at first lumbar puncture was associated with a shorter time to diagnosis, though causality cannot be inferred from this observational association.
SN - 1664-2295
ER -