User:Lukelahood/Autoimmune glial fibrillary acidic protein astropathy
| Autoimmune glial fibriallary acidic protein astropathy | |
|---|---|
| Specialty | Neurology |
| Symptoms | Headache, confusion, weakness |
| Medication | Steroids, or disease modifying agents |
Autoimmune glial fibrillary acidic protein (GFAP) astropathy is a rare, autoimmune disease of the brain and spinal cord (central nervous system).[1] The name denotes that the body's immune system attacks itself (autoimmune), specifcally the protein GFAP, which is predominantly found in astrocytes found in the CNS, causing disease of the astrocytes (astropathy). It can cause headache and neckache, psychiatric symptoms, weakness, and blurred vision.[1] The disease is diagnosed with detection of antibodies against GFAP in the CSF. Characteristic MRI findings can further support diagnosis. The role of GFAP-IgG is still unknown; it might only mark the disease, rather than contribute to the disease process. It was established as a disease in 2016 with the discovery of the antibody directed against GFAP.[2][3]
Signs and symptoms
edit- Meningitis, manifesting as headache and neck ache[1]
- Encephalitis, manifesting as delirium, tremor, seizures, psychiatric symptoms[1]
- Myelitis, manifesting as sensory symptoms and weakness[1]
- Optic disc papillitis, a type of optic nerve inflammation (optic neuritis), manifesting as blurred vision[1][4]
Pathophysiology
editThe immune system attacks GFAP. Specifically, the CD8+ T cells directly induce cell death of astrocytes.
Diagnosis
editPresence of GFAP-IgG in the fluid of the CNS (cerebrospinal fluid) is diagnostic.[1]
Presence of CNS inflammation supports diagnosis. CNS inflammation can be visualized with magnetic resonance imaging (MRI) with contrast. In autoimmune GFAP astropathy, the areas of the CNS containing astrocytes enhances with the use of contrast, meaning those areas of the brain absorb contrast and hold on to it longer than unaffected tissue.[1] Also, there is enhancement of the areas around blood vessels (pervascular enhancement).[4]
Treatment
editAutoimmune GFAP astropathy is treatable.[1] Corticosteroids usually halt the disease, and usually recovery results.[1][4] In those who do not fully respond to steroids, steroid-sparing therapies can be used.[1]
History
editDiscovered at Mayo Clinic.
Research directions
edit- ^ a b c d e f g h i j k Kunchok, A; Zekeridou, A; McKeon, A (June 2019). "Autoimmune glial fibrillary acidic protein astrocytopathy". Current opinion in neurology. 32 (3): 452–458. doi:10.1097/WCO.0000000000000676. PMID 30724768.
- ^ Fang, B; McKeon, A; Hinson, SR; Kryzer, TJ; Pittock, SJ; Aksamit, AJ; Lennon, VA (1 November 2016). "Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Novel Meningoencephalomyelitis". JAMA neurology. 73 (11): 1297–1307. doi:10.1001/jamaneurol.2016.2549. PMID 27618707.
- ^ Shan, F; Long, Y; Qiu, W (2018). "Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Review of the Literature". Frontiers in immunology. 9: 2802. doi:10.3389/fimmu.2018.02802. PMID 30568655.
{{cite journal}}: CS1 maint: unflagged free DOI (link) - ^ a b c Horton, L; Bennett, JL (September 2018). "Acute Management of Optic Neuritis: An Evolving Paradigm". Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society. 38 (3): 358–367. doi:10.1097/WNO.0000000000000700. PMID 30106803.