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glial fibrillary acidic protein astrocytopathy

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: - median age of onset: 44 years [7] - 54% women 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]

General

neuroinflammatory disease

References

  1. Kunchok A, Zekeridou A, McKeon A. Autoimmune glial fibrillary acidic protein astrocytopathy. Curr Opin Neurol. 2019 Jun;32(3):452-458. PMID: 30724768 Free PMC article. Review.
  2. 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: 30568655 Free PMC article. Review.
  3. 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: 27618707
  4. 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: 30991306
  5. 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: 31927153
  6. 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: 33814491 Free PMC article. Review.
  7. 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: 28120349