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.

Keywords

autoimmune glial fibrillary acidic protein astrocytopathy - hypertrophic pachymeningitis - autoimmune inflammation - MRI

Publication History

Received: 22 July 2021

Accepted: 29 December 2021

Article published online:
11 February 2022

• References

• 1 Flanagan EP, Hinson SR, Lennon VA. et al. Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients. Ann Neurol 2017; 81 (02) 298-309
• 2 Kupersmith MJ, Martin V, Heller G, Shah A, Mitnick HJN. Idiopathic hypertrophic pachymeningitis. Neurology 2004; 62 (05) 686-694
• 3 Shan F, Long Y, Qiu W. Autoimmune glial fibrillary acidic protein astrocytopathy: a review of the literature. Front Immunol 2018; 9: 2802
• 4 Kunchok A, Zekeridou A, McKeon A. Autoimmune glial fibrillary acidic protein astrocytopathy. Curr Opin Neurol 2019; 32 (03) 452-458
• 5 Kon T, Nishijima H, Haga R. et al. Hypertrophic pachymeningitis accompanying neuromyelitis optica spectrum disorder: a case report. J Neuroimmunol 2015; 287: 27-28
• 6 Papathanasiou A, Yeo JM, Humberstone M, Hosseini AA. MOG-antibody-associated hypertrophic pachymeningitis. Mult Scler Relat Disord 2020; 42: 102074
• 7 Ueno T, Kon T, Kaneko K, Takahashi T, Tomiyama M. Contrast enhancement of hypertrophic dura mater in MOG antibody-associated disease. Neurology 2019; 93 (06) 271-272
• 8 Tamura R, Yoshida K, Toda M. Current understanding of lymphatic vessels in the central nervous system. Neurosurg Rev 2020; 43 (04) 1055-1064
• 9 Alves de Lima K, Rustenhoven J, Kipnis J. Meningeal immunity and its function in maintenance of the central nervous system in health and disease. Annu Rev Immunol 2020; 38: 597-620
• 10 Budka H. Non-glial specificities of immunocytochemistry for the glial fibrillary acidic protein (GFAP). Triple expression of GFAP, vimentin and cytokeratins in papillary meningioma and metastasizing renal carcinoma. Acta Neuropathol 1986; 72 (01) 43-54
• 11 Hainfellner JA, Voigtländer T, Ströbel T. et al. Fibroblasts can express glial fibrillary acidic protein (GFAP) in vivo. J Neuropathol Exp Neurol 2001; 60 (05) 449-461