Rapidly progressive homonymous hemianopia in the Heidenhain variant of Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease is a rare degenerative disease of the brain characterized by rapid irreversible neurological decline. The Heidenhain variant describes any case of Creutzfeldt-Jakob disease in which visual symptoms predominate in the early stages. The authors report a 63-year-old female who had rapidly progressive bilateral visual field loss since 2 months prior to evaluation. Over the following 2 weeks, the patient developed rapidly progressive visual changes and eventually died. Her case has several unusual features: (1) lack of neurological deficits classically seen with Creutzfeldt-Jakob disease such as myoclonus and ataxia; (2) rapid progression of a clearly documented consecutive homonymous hemianopia over less than 2.5 months from onset of symptoms to death; (3) early symmetric temporal atrophy of the optic nerves and paracentral retinal thinning with optical coherence tomography imaging; (4) negative single-photon emission computed tomography imaging; and (5) negative magnetic resonance imaging changes until late in her course.