Diagnosis within a Diagnosis: An Unusual Case of Intracranial Hypertension

Research output: Contribution to journalArticleOther


A 56 year old man presented with several months of new onset headaches and blurred vision. Lumbar puncture revealed elevated intracranial pressure, but multiple cerebral imaging studies failed to identify a secondary cause. Eventually a formal venogram revealed a transverse venous sinus thrombosis, and an occult malignancy screen led to a diagnosis of non-Langerhans histiocytosis, or Erdheim-Chester Disease. This case highlights two important learning points: 1) secondary causes for raised intracranial pressure should be vigorously sought in atypical cases of idiopathic intracranial hypertension; 2) ECD is a rare but important cause of ophthalmologic and neurologic presentations, and should be a differential for orbital infiltration, intra- and extracranial disease.
Original languageEnglish
Pages (from-to)1-3
Number of pages3
JournalNeuro-Ophthalmology & Visual Neuroscience
Issue number1
Publication statusPublished - 2017
Externally publishedYes


  • papilloedema
  • Erdheim-Chester Disease,
  • proto-oncogene protein B-raf
  • histiocytosis
  • optic nerve fenestration
  • elevated intracranial pressure

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