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Purkinje cell (PC) antibody positivity in a patient with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy
- Source :
- International Journal of Neuroscience. 132:1043-1048
- Publication Year :
- 2020
- Publisher :
- Informa UK Limited, 2020.
-
Abstract
- This case report is the first to describe the detection of antibodies against inositol 1,4,5-trisphosphate receptor 1 (ITPR1, I3PR) in a patient diagnosed with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. ITPR1 is known as one of the Purkinje cell antibodies present in autoimmune cerebellar ataxia (ACA). Here, we described the association between autoimmune GFAP astrocytopathy and autoimmune cerebellar disease (ACD).Demographic features, clinical characteristics, cerebrospinal fluid (CSF) parameters and neuroimaging findings were collected from this patient. Specifically, antibodies against GFAP and other proteins associated with neurological disorders were measured by immunofluorescence staining in both serum and CSF samples.A 52-year-old woman was diagnosed with autoimmune inflammatory meningoencephalitis. She presented with cognitive dysfunction, psychiatric/behavioral abnormalities and serious insomnia with subacute onset. Brain magnetic resonance imaging (MRI) showed bilateral hyperintensity in the semioval centers on axial images and perivascular linear enhancement oriented radially to the ventricles on sagittal images. GFAP-IgG, oligoclonal bands (OBs), N-methyl-D-aspartate receptor (NMDAR)-IgG and ITPR1-IgG co-existed in her CSF. She responded well to immunoglobulin and steroid treatments.Here, we describe the case of a patient with autoimmune GFAP astrocytopathy whose CSF was positive for ITPR1-IgG; however, she did not show typical ataxia manifestations or cerebellar lesions on her MRI scan. This suggests that ITPR1-IgG is not pathogenic, and the positivity of this antibody in CSF is probably associated with the presence of autoimmune inflammation.
- Subjects :
- 0301 basic medicine
Purkinje cell
Autoimmune Diseases
Purkinje Cells
03 medical and health sciences
chemistry.chemical_compound
Autoimmune Diseases of the Nervous System
0302 clinical medicine
Glial Fibrillary Acidic Protein
medicine
Humans
Inositol
Receptor
Autoantibodies
biology
Glial fibrillary acidic protein
General Neuroscience
Meningoencephalitis
General Medicine
Middle Aged
medicine.disease
Molecular biology
030104 developmental biology
medicine.anatomical_structure
chemistry
Astrocytes
Immunoglobulin G
biology.protein
Female
Antibody
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 15435245 and 00207454
- Volume :
- 132
- Database :
- OpenAIRE
- Journal :
- International Journal of Neuroscience
- Accession number :
- edsair.doi.dedup.....1ce1c65b7800320380e2df6cb095001d