Diagnosis within a Diagnosis: An Unusual Case of Intracranial Hypertension

Emma Caroline Foster, Mastura Monif, Kylie Mason, Anneke Van Der Walt

Research output: Contribution to journalArticleOther

Abstract

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
JournalNuro-Ophthalmology and Visual Neuroscience
Volume2
Issue number1
DOIs
Publication statusPublished - 2017
Externally publishedYes

Keywords

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

Cite this

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abstract = "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.",
keywords = "papilloedema, Erdheim-Chester Disease,, proto-oncogene protein B-raf, histiocytosis, optic nerve fenestration, elevated intracranial pressure",
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Diagnosis within a Diagnosis : An Unusual Case of Intracranial Hypertension. / Foster, Emma Caroline; Monif, Mastura; Mason, Kylie; Van Der Walt, Anneke.

In: Nuro-Ophthalmology and Visual Neuroscience, Vol. 2, No. 1, 2017, p. 1-3.

Research output: Contribution to journalArticleOther

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AU - Mason, Kylie

AU - Van Der Walt, Anneke

PY - 2017

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AB - 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.

KW - papilloedema

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