Xiao Yang1, Chenyang Zhang1, Jun Zhang2, Guisheng Chen1, Li Zhao1, Ping Yang1, Huilu Li3, Youming Long4. 1. Neuroscience Center, General Hospital of Ningxia Medical University, Key Laboratory of Craniocerebral Diseases of Ningxia Hui Autonomous Region, Yinchuan 75004, China. 2. Department of Intensive Care Unit, General Hospital of Ningxia Medical University, Yinchuan 75004, China. 3. Department of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou 510260, China. 4. Department of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou 510260, China. Electronic address: youminglong@126.com.
Abstract
BACKGROUND: Autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A) has been recently characterized as a novel autoimmune central nervous system (CNS) disorder with GFAP antibody as the biomarker. However, nonspecific symptoms of A-GFAP-A contribute to misdiagnosis. CASE PRESENTATION: The patients presented with initial symptoms of fever, headache, and nuchal rigidity. Case 1 exhibited mild signs of irritability, active tendon reflexes, and dysuria; case 2 had transient loss of consciousness. Cerebrospinal fluid (CSF) revealed lymphocytosis, elevated protein level, and decreased glucose level. Magnetic resonance imaging (MRI) revealed radial gadolinium enhancement perpendicular to the lateral ventricle. Viral meningitis or tubercular meningitis was suspected. However, their inflammatory and pathogenic indicators showed no abnormal changes, and empirical antibiotic and antiviral drugs did not result in remarkable recovery. Subsequently, cases were detected with a strongly positive expression of GFAP antibody in CSF and the symptoms improved dramatically after high-dose methylprednisolone pulse treatment. CONCLUSION: A-GFAP-A with meningitis-like symptoms could initially masquerade as intracranial infection, and prompt detection of GFAP antibody is essential for differentiation.
BACKGROUND:Autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A) has been recently characterized as a novel autoimmune central nervous system (CNS) disorder with GFAP antibody as the biomarker. However, nonspecific symptoms of A-GFAP-A contribute to misdiagnosis. CASE PRESENTATION: The patients presented with initial symptoms of fever, headache, and nuchal rigidity. Case 1 exhibited mild signs of irritability, active tendon reflexes, and dysuria; case 2 had transient loss of consciousness. Cerebrospinal fluid (CSF) revealed lymphocytosis, elevated protein level, and decreased glucose level. Magnetic resonance imaging (MRI) revealed radial gadolinium enhancement perpendicular to the lateral ventricle. Viral meningitis or tubercular meningitis was suspected. However, their inflammatory and pathogenic indicators showed no abnormal changes, and empirical antibiotic and antiviral drugs did not result in remarkable recovery. Subsequently, cases were detected with a strongly positive expression of GFAP antibody in CSF and the symptoms improved dramatically after high-dose methylprednisolone pulse treatment. CONCLUSION: A-GFAP-A with meningitis-like symptoms could initially masquerade as intracranial infection, and prompt detection of GFAP antibody is essential for differentiation.