Autoimmune glial fibrillary acidic protein astrocytopathy in Chinese patients: a retrospective study

• Published in: European journal of neurology Vol. 25; no. 3; pp. 477 - 483
• Main Authors: Long, Y., Liang, J., Xu, H., Huang, Q., Yang, J., Gao, C., Qiu, W., Lin, S., Chen, X.
• Format: Journal Article
• Language: English
• Published: England John Wiley & Sons, Inc 01-03-2018
• Subjects: Abnormalities; Adult; Aged; Anti-Inflammatory Agents - therapeutic use; antibody; Asian Continental Ancestry Group; astrocyte; Astrocytes; Astrocytes - pathology; Ataxia; Autoimmune Diseases - cerebrospinal fluid; Autoimmune Diseases - diagnostic imaging; Autoimmune Diseases - psychology; Brain; Brain - abnormalities; Brain - diagnostic imaging; Cerebellum; Cerebrospinal fluid; Cortex; Dementia disorders; Dyskinesia; Encephalitis; Female; Fever; Glial fibrillary acidic protein; Glial Fibrillary Acidic Protein - cerebrospinal fluid; Glial Fibrillary Acidic Protein - chemistry; Glial Fibrillary Acidic Protein - genetics; Glial Fibrillary Acidic Protein - immunology; Headache; Humans; Hypothalamus; Immunoglobulin G; Immunoglobulin G - immunology; Immunoglobulins; Immunoglobulins, Intravenous - therapeutic use; Immunohistochemistry; Lesions; Lymphocytes; Lymphocytes B; Lymphocytes T; Magnetic Resonance Imaging; Male; Medulla oblongata; Meninges; Meningitis; meningoencephalitis; Mental disorders; Mesencephalon; Methylprednisolone - therapeutic use; Middle Aged; Myelitis; Neurons; Patients; Pons; Psychosis; Retrospective Studies; Spinal cord; Treatment Outcome; Young Adult; antibody; meningoencephalitis; astrocyte; glial fibrillary acidic protein
• ISSN: 1351-5101; 1468-1331
• DOI: 10.1111/ene.13531

Background and purpose
The aim was to describe the clinical, radiological and pathological features of an autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.
Methods
Data from 19 patients with positive GFAP‐immunoglobulin G in cerebrospinal fluid (CSF) were retrospectively analyzed.
Results
The main disease manifestations included myelitis (68.4%), headache (63.2%), abnormal vision (63.2%), fever (52.6%), ataxia (36.8%), psychosis (31.6%), dyskinesia (15.8%), dementia (15.8%) and seizure (10.5%). Seventeen patients had brain abnormalities (89.5%), of which eight (42.1%) revealed the characteristic radial enhancing and laminar patterns. Cortical abnormalities were found in four patients (21.1%). Other abnormalities were found in the hypothalamus, midbrain, pons, medulla cerebellum, meninges and skull. Eleven patients had longitudinally extensive spinal cord lesions. CSF abnormalities were detected in all patients. Pathological examinations of four patients revealed extensive inflammation, with prominent perivascular B cells and T cells. Abundant antibody‐secreting cells were noted in the interstitial and perivascular spaces. Immunohistochemical analysis showed loss of astrocytes and neurons.
Conclusion
The present patients with positive GFAP‐immunoglobulin G are highly similar to autoimmune GFAP astrocytopathy, described in a recent report. The features of the neuropathology and immunopathology of GFAP astrocytopathies were perivascular inflammation and loss of astrocytes and neurons.
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