Abstract
Background Glial Fibrillary Acidic Protein Astrocytopathy (GFAP-A) is a recently described T-cell mediated inflammatory Central Nervous System (CNS) disorder. Whilst still rare, recent studies suggest that GFAP-A is not as uncommon as previously thought, comprising about 6.2% of all cases of meningoencephalitis of unknown aetiology.1 The clinical presentation is heterogeneous, with meningoencephalitis, or a form thereof, predominating the clinical picture. Linear periventricular gadolinium enhancement is a characteristic radiological finding seen in many cases.
Methods We describe two cases of GFAP-A. Our cases highlight the clinical heterogeneity and characteristic radiological findings of this disorder.
Results The first case is a 70-year-old male who presented with meningoencephalitis associated with radial periventricular gadolinium enhancement on MRI brain characteristic of GFAP-A. He had a chronic course and slow progression despite immunotherapy. The second case is a 30-year-old female who presented with meningoencephalomyelitis three weeks following acute COVID-19 infection. In addition to the characteristic radial periventricular gadolinium enhancement, she also had multifocal T2 hyperintensities affecting the basal ganglia, thalami, mesiotemporal lobes and cervical cord. GFAP-A in the second patient occurred in a para-infectious setting and had a monophasic illness responsive to corticosteroids.
Conclusion These two cases illustrate the clinical heterogeneity of GFAP-A. The diagnosis ought to be considered in all patients presenting with inflammatory/non-infective meningoencephalitis or meningoencephalomyelitis, especially when associated with the characteristic linear periventricular gadolinium enhancement on MRI.
Reference
Kimura A, Takekoshi A, Yoshikura N, Hayashi Y, Shimohata T. Clinical characteristics of autoimmune GFAP astrocytopathy. Journal of Neuroimmunology 2019 Jul 15;332:91–98.
