| Objective: Autoimmune glial fibrillary acidic protein astrocytopathy(GFAP-A)is a group of neurological syndromes that involves the meninges,brain,spinal cord and optic nerve.The main clinical manifestations are fever,headache,encephalopathy,myelitis and abnormal vision.The specific diagnostic marker is GFAP antibody positive in cerebrospinal fluid.GFAP-A pathogenesis is not clear.The clinical symptoms and cerebrospinal fluid indicators are not specific,so the diagnosis of the disease is difficult.This study was conducted by GFAP-A 、 CNS disease with only positive for serum GFAP antibodies(s-GFAP),neuromyelitis optica(NMO)and anti-N-methyl D-aspartate receptor(NMDAR)encephalitis’ Clinical characteristics of these four diseases and laboratory examination,To deepen the were compared and analyzed to deepen the understanding and differentiation of GFAP-A diseases.And to determine whether the patients with positive serum GFAP antibodies belong to GFAP-A or optic neuromyelitis lineage disease or anti NMDAR encephalitis.Methods: The demographics,clinical manifestations,cerebrospinal fluid(CSF)and blood results,brain and spinal cord magnetic resonance imaging(MRI)of 17 GFAP-A patients and 12 s-GFAP patients treated in the Department of Neurology of the First Affiliated Hospital of Guangxi Medical University from August 2018 to March 2021 were analyzed retrospectively,and compared with 30 NMO patients and 30 NMDAR patients.Result: 1.General data: the male to female ratio of GFAP-A patients was 10:7,and the average age of the first onset was(42.47 ± 14.88)years old,the course of disease was 1.0 months,m RS score was4.0.In the s-GFAP group,the ratio of male to female was 1:2,and the average age of first onset was(40.83 ± 15.91)years old,the course of disease was 2.5months,m RS score at admission was 2.5.In NMO group,the ratio of male to female was 1:14,and the average age of the first onset was(42.93 ±13.93)years old,the course of disease was 1.6 months,m RS score at admission was 3.0.In NMDAR group,the ratio of male to female was10:8.8,and the average age of the first onset was(34.43 ± 18.58)years old,the course of disease was 1.3 months,m RS score was3.0.There were significant differences in the ratio of male to female and m RS score at admission among the four groups: the ratio of female in NMO group was higher than that in GFAP-A group and NMDAR group(X2=20.757,P=0.000);m RS score at admission in GFAP-A group was higher than that in s-GFAP group,and that in NMDAR group was higher than that in s-GFAP group(F=5.831,0.001).2.The first clinical symptoms of the four groups were compared:(1)the proportion of early symptoms(headache,fever,runny nose,dizziness)in GFAP-A group was higher than that in NMO group and NMDAR group(X2=11.767,P=0.004).(2)The proportion of disturbance of consciousness in NMDAR group was higher than that in NMO group(X2=12.573,P=0.004).(3)The proportion of mental symptoms in NMDAR group was higher than that in GFAP-A group,s-GFAP group and NMO group(X2=39.994,P=0.000).(4)The proportion of limb convulsions in NMDAR group was higher than that in GFAP-A group,s-GFAP group and NMO group(X2=19.782,P=0.000).(5)The proportion of speech disorder in NMDAR group was higher than that in NMO group(X2=13.633,P=0.002).(6)The proportion of blurred vision in NMO group was higher than that in NMDAR group(X2=16.537,P=0.001).(7)The proportion of dyskinesia in GFAP-A group and NMO group was higher than that in NMDAR group(X2 = 24.676,P = 0.000).(8)The numbness rate of limbs in NMO group >GFAP-A group > NMDAR group,s-GFAP > NMDAR group(X2 = 43.035,P = 0.000).3.Comparison of clinical signs of four groups:(1)the decrease of calculating ability in NMDAR group was higher than that in GFAP-A group,s-GFAP group and NMO group,with significant difference(X2=44.783,P =0.000).(2)The ratio of tendon hyperreflexia in GFAP-A group,s-GFAP group and NMO group was higher than that in NMDAR group(X2=17.084,P=0.000).(3)The proportion of meningeal irritation in GFAP-A group was higher than that in NMO group and NMDAR group(X2=14.378,P=0.000).(4)The proportion of autonomic nerve dysfunction in GFAP-A group,s-GFAP group and NMO group was higher than that in NMDAR group(X2= 12.132,P=0.005).4.CSF and blood examinations:(1)the number of white blood cells in CSF of GFAP-A group was higher than that of NMO group(z=10.908,P=0.012).(2)The CSF protein level of GFAP-A group was higher than that of s-GFAP group,NMO group and NMDAR group(F=4.625,P=0.005).(3)There were significant differences in CSF Ig G level between GFAP-A group,NMDAR group,s-GFAP group,NMO group and s-GFAP group(z=28.75,P=0.000).(4)GFAP-A group QAlb was higher than that of s-GFAP group,NMO group and NMDAR group(F=10.515,P=0.000).5.MRI:(1)the positive rate of brain MRI lesions was 82.4% in GFAP-A group,41.7% in s-GFAP group,50% in NMO group and 40% in NMDAR group.The total positive rate of brain MRI in GFAP-A group was higher than that in NMDAR group(x2=8.560,P=0.035).(2)The positive rate of MRI lesions in spinal cord was 35.3% in GFAP-A group,33.3% in s-GFAP group,86.7% in NMO group and 0.0% in NMDAR group.The total positive rate of MRI in NMO group was higher than that in GFAP-A group,s-GFAP group and NMDAR group(X2=54.636,P=0.000).Conclusion: 1.The clinical characteristics of GFAP-A group are more severe on admission with early symptoms(headache,fever,runny nose,dizziness),limb weakness and meningeal irritation.Increased number of white blood cells,total protein,Ig G in CSF and blood-brain barrier damage are also more common in GFAP-A group.The MRI lesions are found more in brain stem,cervical spinal cord and ≥ three spinal cord segments.2.The clinical characteristics of s-GFAP group are similar those of GFAP-A group,NMO group and NMDAR group,and it is hard to distinguish s-GFAP group from NMO group and NMDAR group in clinical manifestations. |
