A pediatric case of autoimmune glial fibrillary acidic protein astrocytopathy with unique brain imaging patterns and increased cytokines/chemokines

Abstract

Background: Autoimmune anti-glial fibrillary acidic protein (GFAP) astrocytopathy represents a new spectrum of autoimmune inflammatory central nervous system disorders. In recent years, there have been an increasing number of reports on pediatric patients with this disease other than those in Japan.

Case report: A 6-year-old previously healthy boy presented with fever persisting for approximately 10 days, consciousness disturbance, anorexia, and hyponatremia (Na, 121 mEq/L). Even after appropriate correction of hyponatremia, consciousness disturbance was prolonged and was accompanied by gait disturbance, visual hallucinations, and autonomic dysfunction (bradycardia and urinary dysfunction). On a plain MRI, T2-weighted and fluid-attenuated inversion recovery images showed abnormal hyperintense lesions in the bilateral basal ganglia, thalamus, and periventricular white matter. The cerebrospinal fluid was positive for anti-GFAP antibody before treatment, and cytokines/chemokines were increased. He received three courses of intravenous methylprednisolone, followed by gradually tapered oral prednisolone for 6 months, without relapse after 1 year of observation.

Conclusion: In cases of autoimmune encephalitis with prolonged consciousness disturbance, hyponatremia, urinary dysfunction, and MRI findings with hyperintensities in the bilateral basal ganglia, thalamus, and periventricular white matter, anti-glial fibrillary acidic protein antibodies should be examined.

Keywords: Astrocytopathy; Autoantibody; Autoimmune encephalitis; Glial fibrillary acidic protein (GFAP); Hyponatremia; Pediatric; Urinary dysfunction.