Autoimmune glial fibrillary acidic protein astrocytopathy in children: a retrospective study

Abstract

Objective To describe the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children. Method Data from 11 pediatric patients with autoimmune GFAP astrocytopathy were retrospectively analyzed. Results All of the patients showed encephalitis and meningoencephalitis or meningoencephalomyelitis with or without myelitis. 45.4% of the patients had fever, 27.3% headaches, 18.2% dizziness, 18.2% drowsiness, and 18.2% mental disorders. Cerebrospinal fluid (CSF) was detected in all patients. The white blood cell counts (WBC) (90.9%), lactic dehydrogenase levels (72.7%), protein level (36.4%), and adenosine deaminase activity (ADA) level (27.3%) were elevated, and the CSF glucose levels (72.7%) were slightly reduced. Nine patients (90%) were found to have brain abnormalities, of which five (50.0%) patients had abnormal symmetrical laminar patterns or line patterns hyperintensity lesions on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the basal ganglia, hypothalamus, subcortical white matter and periventricular white matter. The linear radial enhancement pattern of the cerebral white matter was only seen in two patients, with the most common being abnormal enhancement of leptomeninges (50%). Five patients had longitudinally extensive spinal cord lesions. Conclusion The findings of pediatric patients with autoimmune GFAP astrocytopathy are different from previous reports.

Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy: An Emerging Cause of Meningoencephalomyelitis in Children and Adolescents

“…2–4 Pediatric patients have been successfully treated with varying combinations of therapies, often including intravenous methylprednisolone and intravenous immune globulin, with variable inclusion of PLEX and/or long-term immunosuppressants. 2–10,13,14 Our experience demonstrates that pediatric patients may require combination acute treatment with intravenous methylprednisolone, intravenous immune globulin, and PLEX, as 4 of our 5 patients were not responsive to intravenous methylprednisolone and intravenous immune globulin alone, supporting consideration of early initiation of PLEX.…”

“…Interestingly, this imaging finding may be less common in pediatric patients, with only 11 prior pediatric patients being described as having radial linear enhancement. 2,6,10,11 Among our cohort, linear perivascular enhancement was seen in only 1 patient, and he was 17 years of age, raising the question of whether this finding may be age dependent.…”

“…Although oncologic processes have been reported as a common potential trigger of GFAP astrocytopathy in adults, 7 among pediatric patients, a potential oncologic trigger has only rarely been identified. 6,9,10,13,14 This suggests that other factors may trigger GFAP autoimmunity in children. Infectious triggers have also been linked to GFAP astrocytopathy.…”

“…Most patients with GFAP astrocytopathy described to date are adults, [2][3][4] with few pediatric patients reported. [2][3][4][5][6][7][8][9][10][11][12][13][14] Regardless of age, patients tend to present with a flulike prodrome followed by neurologic symptoms, ranging from encephalopathy and seizures to headache, tremor, and ataxia. 2,6 Many patients are steroid-responsive, although relapsing and fatal cases have been described.…”

Autoimmune glial fibrillar acidic protein astrocytopathy mimicking tuberculous meningitis

“…A CSF ADA level of around 10 units/L is a relatively reliable cut-off for differentiating tuberculous meningitis from other meningitis types 16. However, elevated CSF ADA levels in autoimmune GFAP astrocytopathy are common and can reach as high as 13 units/L 2 17. The CSF ADA level can exceed 10 units/L in bacterial or cryptococcal meningitis 18–21.…”