Hybrid 18F-florbetapir PET/MRI for assessing myelin recovery in GFAP-A patients

Abstract

Glial fibrillary acidic protein astrocytopathy (GFAP-A) is a rare autoimmune disease of the central nervous system that was newly reported in 2016. Previous studies have speculated that the pathological mechanism and clinical outcome of GFAP-A lie in the demyelination of the central nervous system, but due to the limitations of MR, this conclusion has not been further confirmed from the perspective of neuroimaging. A non-invasive, quantitative measurement of demyelination would be clinically valuable, given its critical role in mediating GFAP-A. Here, we report a case in which we use 18F-florbetapir positron emission tomography-magnetic resonance imaging (PET/MRI) to evaluate myelin recovery with follow-up in the patient with GFAP-A. Our patient displayed a decreased uptake of PET tracer 18F-florbetapir in the brain lesions and lower distribution volume ratio in the damaged white matter lesions compared to the normal-appearing white matter, indicating significant intracranial demyelination. After treatment, the 18F-florbetapir PET/MRI examination showed a significant increase in the uptake of 18F-florbetapir in the brain lesions, along with a reduced Expanded Disability Status Scale score. Although only a small number of patients have been validated, this case first reported 18F-florbetapir PET/MRI could quantitatively and non-invasively assess the myelin recovery in GFAP-A patients, which may lead to improvements in the early diagnosis and long-term prognosis.

Keywords

• glial fibrillary acidic protein
• neuroinflammation
• 18f-florbetapir pet/mri
• autoimmune encephalitis
• myelin recovery