Clinical characteristics of 11 children with autoimmune glial fibrillary acidic protein astrocytopathy / 中华神经医学杂志

ABSTRACT

Objective:To explore the clinical characteristics of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children.Methods:Eleven children with autoimmune GFAP astrocytopathy with positive GFAP antibody in serum and/or cerebrospinal fluid were collected in our hospital from January 2020 to February 2022. The clinical data of these children were analyzed retrospectively.Results:Among the 11 children, there were 6 males and 5 females, and the age of onset ranged from 3 years old and 10 months to 12 years old. The main clinical manifestations included fever ( n=8), headache ( n=5), vomiting ( n=6), ataxia ( n=2), limb weakness ( n=4), cranial nerve involvement ( n=6), disturbance of consciousness ( n=4), abnormal mental behavior ( n=3), seizures ( n=1), and autonomic nervous dysfunction ( n=3). Meningoencephalomyelitis was noted in one child, meningoencephalitis in one, encephalomyelitis in 7, and encephalitis was noted in two children. MRI showed brain involvement in all children, spinal cord involvement in 8 children, and optic nerve involvement in one child. Abnormal enhancement in different parts of cerebral lobe, meninges, sulcus, optic nerve and spinal cord were found in 3 children. Four children were positive for GFAP antibody in cerebrospinal fluid and serum, 3 patients were positive for GFAP antibody in cerebrospinal fluid, and 4 children were positive for GFAP antibody in serum. Four children were complicated with multiple antibodies, mainly myelin oligodendrocyte glycoprotein antibody. Tumor screening was all negative. All of the 11 children responded to immunotherapy, but two of them relapsed; one left visual and motor function impairment. Conclusions:The clinical manifestations of autoimmune GFAP astrocytopathy in children are diverse and non-specific, and the lesions mainly involve meninx, brain, spinal cord and optic nerve. Most children respond well to glucocorticoid treatment and have a good prognosis; but there is still a certain recurrence rate, and some children may leave neurological damage.