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

Eric K. Chin, Gerard Hershewe, John L Keltner

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)54-58
Number of pages5
JournalNeuro-Ophthalmology
Volume36
Issue number2
DOIs
StatePublished - Apr 2012

Keywords

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

ASJC Scopus subject areas

  • Ophthalmology
  • Clinical Neurology

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