Anti-GFAP Antibody-Associated Hypertrophic Pachymeningitis

Abstract

Background Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an inflammatory central nervous system (CNS) disorder that usually presents as steroid responsive encephalitis, meningitis, myelitis, or meningoencephalomyelitis. Hypertrophic pachymeningitis (HP) is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Depending on the etiology, HP can be idiopathic or secondary to a wide variety of other diseases. There are no reports of autoimmune GFAP astrocytopathy presenting as HP. Methods In this case report, we describe a rare case of pediatric HP possibly associated with anti-GFAP antibody. Results A 13-year-old previously healthy girl presented with headache for nearly 8 months with left-sided peripheral facial palsy and left-sided abductor nerve palsy in the second month of course. Magnetic resonance imaging (MRI) of the brain revealed contrast enhancement of hypertrophic dura mater. Anti-GFAPα antibodies were positive in serum and cerebrospinal fluid. The patient improved clinically after steroid treatment with partial resolution of abnormal intracranial MRI lesions. Conclusion The present study suggests that HP may be one of the clinical phenotypes for autoimmune GFAP astrocytopathy or GFAP antibody is a biomarker for HP.

Autoimmune glial fibrillary acidic protein astrocytopathy associated with breast cancer: a case report

“…In the patient described in this study, gadolinium contrast-enhanced brain MRI scans showed a heterogeneous thickening of the dura mater. Similarly, a recent report described a pediatric patient with hypertrophic pachymeningitis associated with GFAP-A, and the authors indicate that hypertrophic pachymeningitis may be one of the clinical phenotypes for GFAP-A [19], which is also corroborated by our findings.…”

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.…”

“…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.…”

Glial fibrillary acidic protein astrocytopathy and tuberculous meningoencephalitis occurring in a patient with Legionella pneumonia: a case report

“…A 2016 study by Flanagan et al found that 17 out of 38 patients with GFAP astrocytopathy had a typical linear perivascular radial gadolinium pattern enhancement, while 7 had normal imaging results [ 3 ]. Recently, some case reports have shown that, in addition to common site lesions, such as the subcortical white matter, basal ganglia, hypothalamus, brainstem, cerebellum, and spinal cord, GFAP astrocytopathy can also manifest as hypertrophic meningoencephalitis [ 5 ] or mild encephalitis with reversible splenial corpus callosum lesions [ 6 ]. This patient had no typical linear lesions, but the CSF GFAP antibodies were highly specific and sensitive and led to the diagnosis of GFAP astrocytopathy.…”