title: Photo essay. MRI and positron emission tomography findings in Heidenhain variant Creutzfeldt-Jakob disease.
abstract: The typical presentation of Heidenhain variant Creutzfeldt-Jakob disease (CJD) is a rapidly progressive visual loss in the setting of a relatively normal ophthalmologic examination. At presentation, patients with this uniformly fatal illness frequently demonstrate only minor cortical abnormalities on MRI. Here, we document the clinical presentation and imaging results of a patient with Heidenhain variant CJD in whom abnormalities on positron emission tomographic imaging were more evident than changes on MRI. These changes were present in striate cortex and visual association areas, providing clinical-anatomical correlation with our patient's visual deficits. Nuclear imaging provides a considerably more sensitive measure of neural dysfunction early in the course of this disease.
authors.affiliation: Departments of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA. sashank.prasad@uphs.upenn.edu
authors.forename: Sashank
authors.initials: S
authors.lastname: Prasad
authors.forename: Edward B
authors.initials: EB
authors.lastname: Lee
authors.forename: John H
authors.initials: JH
authors.lastname: Woo
authors.forename: Abass
authors.initials: A
authors.lastname: Alavi
authors.forename: Steven L
authors.initials: SL
authors.lastname: Galetta
mesh_terms: D000368:Aged; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications* / Q000000981:diagnostic imaging* / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D049268:Positron-Emission Tomography / Q000379:methods*; D014786:Vision Disorders / Q000209:etiology