Atypical brain MRI findings in a patient with treatment responsive anti-IgLON5 disease

Anti-IgLON5 disease is a rare autoimmune neurological condition which was relatively recently described in the literature. This syndrome encompasses a range of clinical manifestations with most cases showing unremarkable findings on brain magnetic resonance imaging (MRI). Here, we report a case of a 61-year-old female patient with unique brain MRI features that, to the best of our knowledge, has not been reported in the literature before. Following treatment including immunotherapy, the patient experienced significant improvement clinically accompanied by radiological improvement on the follow-up imaging.