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Heidenhain Variant of Creutzfeldt-Jakob Disease: A Case Report

Nikolina Madjer1, Rahul Shaju1, Colin Vipond1

  • 1Internal Medicine, Advocate Lutheran General Hospital, Park Ridge, USA.

Cureus
|September 27, 2024
PubMed

Insights

Heidenhain variant Creutzfeldt-Jakob disease (HvCJD) is a rare prion disease. This case report details a patient with initial progressive vision loss, followed by cognitive decline, emphasizing HvCJD

Area of Science:

  • Neuroscience
  • Neurology
  • Prion Diseases

Background:

  • Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder.
  • Prions, misfolded proteins, accumulate in the central nervous system.
  • Heidenhain variant CJD (HvCJD) is a rare subtype with prominent initial visual symptoms.

Observation:

  • A patient presented with several weeks of progressive vision loss.
  • Cognitive impairment developed after the initial visual symptoms.
  • Extensive diagnostic workup was required due to the rarity of the condition.

Findings:

  • The case highlights the initial presentation of vision loss in HvCJD.
  • It underscores the diagnostic challenges posed by rare CJD variants.
  • The report focuses on the clinical course preceding cognitive decline.

Implications:

  • Increased awareness of HvCJD's visual-onset presentation is crucial.
  • Early recognition can aid in timely diagnosis and patient management.
  • This case contributes to understanding the spectrum of CJD presentations.

Keywords:
cortical ribboningcreutzfeld-jakob diseaseheidenhain variantocular symptomsprion diseases

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