glial fibrillary acidic protein astrocytopathy
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Etiology
- autoimmune disorder
- neoplasms detected in 22-38%%[7]
- ovarian teratoma most common, especially with accompanying NMDA receptor or aquaporin-4 autoantibodies
- prostate cancer, gastroesophageal adenocarcinoma, multiple myeloma, melanoma, colonic carcinoid, parotid pleomorphic adenoma[3]
Epidemiology
Clinical manifestations
- headache
- encephalitis: delirium, seizures, psychiatric disorders[1]
- movement disorders: tremor, myoclonus, ataxia (>50%)
- autonomic dysfunction: urinary dysfunction (> 50%)
- myelitis: sensory symptoms. weakness
- optic disc papillitis (blurred vision) is common
Laboratory
- CSF analysis
- anti-GFAP IgG in CSF
- transient elevation of adenosine deaminase activity in CSF
- WBC in CSF elevated, predominantly lymphocytes
- serum sodium: hyponatremia
Radiology
- neuroimaging (MRI)
- T1 postgadolinium enhancement of GFAP-enriched CNS regions[1]
- bilateral hyperintensities in posterior thalamus (some patients)
Management
- high-dose glucocorticoid-responsive disorder
- relapses occur in ~20% of patients
- transition to steroid-sparing drug may be necessary
Comparative biology
- GFAP-specific cytotoxic CD8+ T cells implicated as effectors in a transgenic mouse model[3]
More general terms
References
- ↑ Jump up to: 1.0 1.1 1.2 Kunchok A, Zekeridou A, McKeon A. Autoimmune glial fibrillary acidic protein astrocytopathy. Curr Opin Neurol. 2019 Jun;32(3):452-458. PMID: https://www.ncbi.nlm.nih.gov/pubmed/30724768 Free PMC article. Review.
- ↑ Shan F, Long Y, Qiu W. Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Review of the Literature. Front Immunol. 2018 Dec 5;9:2802. eCollection 2018. PMID: https://www.ncbi.nlm.nih.gov/pubmed/30568655 Free PMC article. Review.
- ↑ Jump up to: 3.0 3.1 3.2 Fang B, McKeon A, Hinson SR, Kryzer TJ, Pittock SJ, Aksamit AJ, Lennon VA. Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Novel Meningoencephalomyelitis. JAMA Neurol. 2016 Nov 1;73(11):1297-1307. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27618707
- ↑ Kimura A, Takekoshi A, Yoshikura N, Hayashi Y, Shimohata T. Clinical characteristics of autoimmune GFAP astrocytopathy. J Neuroimmunol. 2019 Jul 15;332:91-98. Epub 2019 Apr 9. PMID: https://www.ncbi.nlm.nih.gov/pubmed/30991306
- ↑ Allen A, Gulhar S, Haidari R, Martinez JPP, Bekenstein J, DeLorenzo R, Tang Y, Oh U. Autoimmune glial fibrillary acidic protein astrocytopathy resulting in treatment-refractory flaccid paralysis. Mult Scler Relat Disord. 2020 Jan 3;39:101924. PMID: https://www.ncbi.nlm.nih.gov/pubmed/31927153
- ↑ Nakamura S, Fujioka T, Kawashima S et al Self-remitting Elevation of Adenosine Deaminase Levels in the Cerebrospinal Fluid with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report and Review of the Literature. Intern Med. 2021 Sep 15;60(18):3031-3036. Epub 2021 Apr 5. PMID: https://www.ncbi.nlm.nih.gov/pubmed/33814491 Free PMC article. Review.
- ↑ Jump up to: 7.0 7.1 7.2 Flanagan EP, Hinson SR, Lennon VA et al Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients. Ann Neurol. 2017 Feb;81(2):298-309. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28120349