Rapidly Progressive Homonymous Hemianopia in the Heidenhain Variant of Creutzfeldt-Jakob Disease

Abstract

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.

Keywords::

• 14-3-3 protein
• Creutzfeldt-Jakob disease
• cortical blindness
• Heidenhain variant
• magnetic resonance imaging
• optical coherence tomography

ACKNOWLEDGEMENTS

This work has been presented in part as a poster at the annual meeting of the Association for Research in Vision and Ophthalmology (ARVO), Ft Lauderdale, Florida, USA, in May 2011. This work was sponsored in part by an unrestricted grant from Research to Prevent Blindness.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

Note: Figures 1–3 of this article are available in colour online at www.informahealthcare.com/oph