Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP)

Abstract

Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP) is a rare condition associated with the presence of antibodies against an intermediate protein located between the finer microfilaments and the larger microtubules in astrocytes. The condition remains poorly understood, necessitating further research to improve diagnosis and treatment. This article presents a clinical case of encephalomyelitis associated with antibodies to GFAP. In a 40-year-old female patient, the disease presented with the onset of numbness and weakness in the lower limbs, accompanied by pelvic organ dysfunction. The detection of antibodies to GFAP in serum and cerebrospinal fluid, the clinical picture of encephalomyelitis, and an MRI pattern of linear radial perivascular enhancement on T1-weighted images, along with an extensive (extending from TI to TXI) hyperintense lesion on T2-weighted images, led to a diagnosis of autoimmune anti-GFAP astrocytopathy. By the time the diagnosis was confirmed, the patient presented with marked gait disturbance requiring the use of bilateral support, as well as neurogenic bladder dysfunction necessitating intermittent catheterisation. No clinical or radiological progression of the disease was observed during anti-B-cell therapy with rituximab. This case report adds to the existing evidence on the clinical features of anti-GFAP astrocytopathy and demonstrates the importance of timely diagnosis and early initiation of pathogenetic therapy for the disease.