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Driving Simulation in the Clinic: Testing Visual Exploratory Behavior in Daily Life Activities in Patients with Visual Field Defects
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Rapid progressive visual decline and visual field defects in two patients with the Heidenhain variant of

Janine Lenk1, Kay Engellandt2, Naim Terai1

  • 1Department of Ophthalmology, University Hospital Carl Gustav Carus, TU Dresden, Fetscherstraße 74, 01307 Dresden, Germany.

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
|February 13, 2018
PubMed

Insights

The Heidenhain variant of Creutzfeldt-Jakob disease (HvCJD) causes rapid vision loss and behavioral changes. Early diagnosis via CSF analysis is crucial due to the poor prognosis.

Area of Science:

  • Neurology
  • Ophthalmology

Background:

  • The Heidenhain variant of Creutzfeldt-Jakob disease (HvCJD) is a rare prion disease.
  • HvCJD typically presents with visual disturbances, including loss of visual acuity and declining visual fields.

Observation:

  • Two patients experienced rapid vision loss and homonymous hemianopsia over weeks.
  • Cranial MRI and neurological exams were unremarkable, but CSF showed elevated protein.
  • Visual fields progressively deteriorated, followed by significant behavioral changes and cognitive decline.

Findings:

  • Cerebrospinal fluid (CSF) analysis revealed PrPSc and 14-3-3 protein, confirming Creutzfeldt-Jakob disease (CJD).
  • Despite initial normal MRI, patients rapidly declined and died within 8 weeks of diagnosis.

Implications:

  • Early suspicion of HvCJD is critical in cases of unexplained visual loss and behavioral changes.
  • Prompt diagnostic testing, including CSF analysis for specific biomarkers, is essential for timely diagnosis.
  • The poor prognosis necessitates immediate communication to patients and families for appropriate care planning.
Abstract

Keywords:
HvCJDMRIPrion diseaseVisual field defects

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