Endgames Picture Quiz

Waking up to darkness

BMJ 2011; 343 doi: https://doi.org/10.1136/bmj.d4783 (Published 09 August 2011) Cite this as: BMJ 2011;343:d4783
  1. Aoife C Lowney, senior house officer1,
  2. Stephen A Ryan, senior house officer1,
  3. Andrea Ryan, registrar in ophthalmology2,
  4. Sinead Harney, consultant rheumatologist1
  1. 1Department of Rheumatology, Cork University Hospital, Wilton, Cork, Ireland
  2. 2Department of Ophthalmology, Mater Misericordiae University Hospital, Dublin 7, Ireland
  1. Correspondence to: A C Lowney aoifelowney{at}gmail.com

An 84 year old woman was referred by her general practitioner to the emergency department with painless binocular blindness. She reported waking four days previously to find that she could no longer see. Fearful of the possibility of malignancy, she delayed presentation to hospital. On further questioning, she recalled focal right frontal headaches of four months’ duration. She denied any previous visual disturbance. On direct questioning, she admitted to pain in her jaw, which was most noticeable at mealtimes.

The neurological examination showed bilateral afferent pupillary defects and no perception of light in either eye. Funduscopy showed bilaterally swollen optic discs. On closer inspection, her temporal regions had an unusual appearance bilaterally (fig 1) and her right temporal artery was pulseless to palpation. Her erythrocyte sedimentation rate was 76 mm in the first hour and computed tomography of the brain was unremarkable for her age.

Questions

  • 1 What is the diagnosis?

  • 2 What are the important features of the clinical history?

  • 3 What should be considered on clinical examination?

  • 4 What is the pathophysiology of this condition?

  • 5 How is the condition managed?

Answers

1 What is the diagnosis?

Short answer

Giant cell arteritis.

Long answer

The American College of Rheumatology (ACR) 1990 classification criteria for systemic vasculitides1 include:

  • Age at disease onset 50 years or more

  • New headache

  • Temporal artery abnormality

  • Raised erythrocyte sedimentation rate (≥50 mm in the first hour by the Westergren method)

  • Abnormal artery biopsy.

Patients are diagnosed with giant cell arteritis if at least three of these five criteria are …

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