Magnetic resonance imaging of the spinal cord and clinical characteristics in patients with autoimmune glial fibrillary acidic protein astrocytopathy

Objective To analyze the magnetic resonance imaging (MRI) of the spinal cord and clinical characteristics in patients with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. Methods A total of 1 040 samples of cerebrospinal fluid (CSF) and sera collected in the Second Affiliated Hospital of Guangzhou Medical University from March 2013 to June 2018 were tested with tissue- and cell-based assays, and 42 patients were found positive for GFAP-IgG. The clinical data and MRI characteristics of the spinal cord of 19 patients who were positive for GFAP-IgG in CSF with autoimmune GFAP astrocytopathy and lesions in the spinal cord were retrospectively reviewed. Results There were 12 females and seven males among the 19 patients, with onset age of (44±17) years. The main manifestations of these patients included limb weakness (14/19), abnormal vision (5/19), headache (4/19), seizure (4/19), dementia (3/19), etc. On MRI of the spinal cord, five patients showed involvement in the cervical cord alone, eight showed involvement in the thoracic cord alone and six had both cervical and thoracic segment involvement. Fifteen patients had longitudinally extensive myelitic abnormalities (≥3 vertebral segments long). Seven enhancement patterns were encountered. Lesions were displayed in the spinal cord and brain in eight patients. Central gray matter involvement in the spinal cord was found in all the 19 patients. Conclusions Autoimmune GFAP astrocytopathy more frequently presents in females than in males. MRI of the spinal cord has complex presentations and longitudinally extensive myelitic abnormalities usually. Patients often show central gray matter involvement in the spinal cord. Myelitic abnormalities present more often in thoracic segment than in cervical segment. Abnormalities in lumbar segment are less encountered. Key words: Autoimmune diseases; Glial fibrillary acidic protein; Astrocytes; Spinal cord; Magnetic resonance imaging