ABSTRACT
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.
ABSTRACT
Objective Our study aimed to delineate the clinical and radiological features of patients with anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab)positive neuromyelitis optica spectrum disorder (NMOSD). Methods Fifty-seven patients with NMOSD and 29 patients with multiple sclerosis (MS) were collected. Data on clinical and radiological features of MOG-Ab positive patients with were analyzed retrospectively. Results MOG-Abs were present in 9/57 (15.8%) NMOSD patients and 2/29 (6.9%) MS patients. Both MOG and aquaporin-4 (AQP4) antibodies were positive in one case of NMOSD. There was no significant difference between the two groups (P＞0.05). There were more females than males having MOG-Ab positive NMOSD (females: males=7:1) and the average onset age was 41.4 ± 11.5 years. There was no significant difference in gender and age between MOG-Ab negative and AQP4-Ab positive groups(P＞0.05). The durations of disease were significantly shorter in either MOG-Ab positive NMOSD patients or MOG-Ab negative NMOSD patients than in AQP4-Ab positive group (P<0.05). Recurrence was the main disease pattern of all three groups and the frequency of recurrence was not significant different among three groups (P＞0.05). The incidence of optic neuritis was 62.5% in NMOSD patients with MOG-Ab positive and 43.5% in AQP4-Ab positive NMOSD patients (P＞0.05). There was no significant difference in the morphology and location of brain lesions among the three groups (P＞0.05). MOG-Ab positive NMOSD patients had long segment spinal cord lesions. The median length of the spinal cord lesions in the MOG-Ab positive group was similar to the other two groups (P＞0.05). Conclusions MOG-Ab positive NMOSD patients have higher proportion of females with shorter recurrence course, more likely complicated with optic neuritis. And the radiological features of brain and spinal cord were not specific to patients with AQP4-Ab positive.