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Brain radial enhancement pattern in patients with negative glial fibrillary acidic protein-IgG: A cases series study
Jiajia Yue1, Peihao Lin1, Chun Lian1
1Department of Neurology, the Second Affiliated Hospital of Guangzhou Medical University, 250# Changgang east Road, Guangzhou, 510260, Guangdong Province, China; Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and The Ministry of Education of China, Institute of Neuroscience and the Second Affiliated Hospital of Guangzhou Medical University, 250# Changgang east Road, Guangzhou, 510260, Guangdong Province, China.
Background And Objectives:
Brain radial enhancement pattern on magnetic resonance imaging (MRI) has been identified as typical lesions in autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). However, the authors encountered several patients without GFAP-IgG showing that such specific imaging. In the present study, we reported the clinical pictures of 5 GFAP-IgG-negative patients with GFAP-A specific imaging pattern.
Methods:
Data was retrospectively obtained from June 2013 through April 2023, and five GFAP-IgG-negative patients with valid data were recruited. Clinical information was either obtained by the investigators or retrieved from the referring clinicians and included prodromal symptoms, neurologic manifestations, comorbidities, results of ancillary studies.
Results:
Altogether five GFAP-IgG-negative patients with "meningoencephalitis/encephalitis" manifestations and brain radial perivascular enhancement were confirmed. One patient had peripheral lymphoma. Four patients had other autoimmune antibody in serum and/or cerebrospinal fluid, of which one patient had positive aquaporin IgG. Clinical features of the five patients included headache, fever, epilepsy and abnormal behavioral symptoms. MRI of patients revealed radial perivascular gadolinium enhancement extending from the lateral ventricles to the white matter suggestive of autoimmune GFAP-A.
Conclusion:
GFAP-A-like disorders with radial perivascular enhancement could be found in GFAP-IgG-negative patients with or without neoplasm, which could provide new insight into the differential diagnosis of GFAP-A.
Insights
Glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) imaging patterns can occur in patients negative for GFAP-IgG antibodies. This study details five such cases, expanding the differential diagnosis for GFAP-A.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Brain radial enhancement on MRI is characteristic of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A).
- However, this specific imaging pattern has been observed in patients lacking GFAP-IgG antibodies.
Purpose of the Study:
- To report the clinical characteristics of five GFAP-IgG-negative patients exhibiting the GFAP-A specific imaging pattern.
- To broaden the understanding of GFAP-A differential diagnoses.
Main Methods:
- Retrospective data collection from June 2013 to April 2023.
- Recruitment of five GFAP-IgG-negative patients with valid data.
- Gathering of clinical information including prodromal symptoms, neurological manifestations, comorbidities, and ancillary study results.
Main Results:
- Five GFAP-IgG-negative patients presented with meningoencephalitis/encephalitis and brain radial perivascular enhancement.
- One patient had peripheral lymphoma; four had other autoimmune antibodies (one with positive aquaporin IgG).
- Clinical features included headache, fever, epilepsy, and behavioral changes, with MRI showing radial perivascular enhancement.
Conclusions:
- GFAP-A-like disorders with radial perivascular enhancement can occur in GFAP-IgG-negative individuals.
- These findings are relevant with or without neoplasms, offering new insights for GFAP-A differential diagnosis.
