Coexisting Autoimmune Glial Fibrillary Acidic Protein （GFAP）-IgG and Aquaporin4 （AQP4）-IgG in Patients with Myelitis
10.13471/j.cnki.j.sun.yat-sen.univ（med.sci）.20220414.001
- VernacularTitle:抗体双阳性伴脊髓炎的自身免疫性星形细胞病临床特点
- Author:
Hai-yan YAO
1
;
Hui-lu LI
1
;
Li-hong JIANG
1
;
You-ming LONG
1
;
Xin-guang YANG
2
Author Information
1. Department of Neurology， the Second Affiliated Hospital of Guangzhou Medical University， Guangzhou 510260， China
2. Department of Neurology， Sun Yat-sen Memorial Hospital， Sun Yat-sen University， Guangzhou 510235， China
- Publication Type:Journal Article
- Keywords:
glial fibrillary acidic protein;
GFAP-A;
antibody
- From:
Journal of Sun Yat-sen University(Medical Sciences)
2022;43(4):607-612
- CountryChina
- Language:Chinese
-
Abstract:
ObjectiveTo analyze the clinical features of patients with coexisting GFAP-IgG and AQP4-IgG in myelitis. MethodsWe performed a retrospective analysis of patients with myelitis and GFAP-IgG. ResultsTotally 55 cases of autoimmune GFAP astrocytopathy （GFAP-A） were collected. The clinical manifestations included headache， fever， myelitis， abnormal vision， abnormal behavior， ataxia， disturbance of consciousness， epilepsy， dyskinesia， cognitive dysfunction， and other manifestations. Thirty-one cases were accompanied by myelitis， and 8 cases were GFAP/AQP4 double positive with myelitis. The 8 double-positive cases all showed dysuria and sensory plane disturbance. The MRI of spinal cord showed lesions in eight patients， seven of which had spinal cord lesions more than three segments， and three of which had Gadolinium enhancement. CSF showed the increasing level of white blood cell count or protein in six patients and decreasing level of glucose signally in one patient. In this study， 8 cases of GFAP/AQP4 IgG double positive with myelitis， 16 cases of GFAP IgG single positive （except AQP4 IgG negative， neuron antibody， oligodendrocyte antibody and glial cell antibody are negative） with myelitis， and 47 cases of AQP4 IgG single positive （without other neural related antibodies） with myelitis NMOSD were selected. There was statistically difference between single positive group with GFAP-IgG （sGFAP-A） and double positive group with GFAP-IgG （dGFAP-A） and AQP4-IgG in fever and CSF protein level（P<0.05）. ConclusionsGFAP/AQP4-IgG double-positive myelitis is relatively rare， which is different from the AQP4 single-positive myelitis in clinical features. The clinical manifestations include urination and defecation difficulties， sensory dysfunction. Spinal cord MRI usually manifests as long lesions extending over three vertebral segments， and cerebrospinal fluid examinations often indicate increased levels of white blood cells or protein.
