Case of Heidenhain Variant Creutzfeldt-Jakob Disease Presenting with Homonymous Quadrantanopia and Corresponding Nuclear Imaging Findings

Sporadic Creutzfeldt-Jakob disease (sCJD) belongs to a class of prion diseases in which prion protein forms abnormal and pathological folds. In the Heidenhain variant of Creutzfeldt-Jakob disease (HvCJD), patients present with early, prominent visual complaints prior to the onset of more typical features of sCJD. MRI brain is commonly normal, making early diagnosis of this variant particularly challenging. We present a case of a patient with impaired depth perception and homonymous inferior quadrantanopia with normal MRI, in contrast to marked focal FDG-PET and SPECT abnormalities providing clinical-anatomical correlate.