ISRA May 2022

MR Findings of Autoimmune Glial Fibrillary Acidic Protein (GFAP) Autoimmune Encephalitis: Case Report and Literature Review

Azzam Ghaben 1 Adi Wilf Yarkoni 2 Ruth Eliahou 1
1Radiology, Rabin Medical Centre - Beilinson, Israel
2Neurology, Rabin Medical Centre - Beilinson, Israel

Background: GFAP autoimmune encephalitis (AIE) is a rare type of inflammatory CNS disease caused by autoantibodies against GFAP, a hallmark protein expressed on astrocytes. Median age is 44–50 years. Characteristic MRI features are linear, radial perivascular pattern of enhancement as well as periventricular white matter abnormalities on T2-Weighted images.

Case presentation: We present a case of a 58 years old male with Behcet disease who presented with progressive functional and mental decline for the past two months. First brain MRI showed subtle bi-temporal periventricular T2\flair hyper-intensities. Follow-up MR scans demonstrated subtle perivascular radial enhancement, suggesting AIE and particularly GFAP AIE. CSF and Serum analysis confirmed GFAP-IgG. Patient was treated with corticosteroids with marked clinical improvement.

Conclusion: Increased awareness to subtle MR findings of GFAP-AIE can aid diagnosis and promote prompt treatment.