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Article

Diagnosis within a Diagnosis: An Unusual Case of Intracranial Hypertension

1Department of Neurology, The Royal Melbourne Hospital, Parkville, Australia

2Department of Physiology, University of Melbourne, Parkville, Australia

3Department of Haematology, The Royal Melbourne Hospital, Parkville, Australia

4Melbourne Brain Centre at The Royal Melbourne Hospital, Department of Medicine, University of Melbourne, Parkville, Australia


Neuro-Ophthalmology & Visual Neuroscience. 2017, Vol. 2 No. 1, 1-3
DOI: 10.12691/novn-2-1-1
Copyright © 2017 Science and Education Publishing

Cite this paper:
Emma Foster, Mastura Monif, Kylie Mason, Anneke van der Walt. Diagnosis within a Diagnosis: An Unusual Case of Intracranial Hypertension. Neuro-Ophthalmology & Visual Neuroscience. 2017; 2(1):1-3. doi: 10.12691/novn-2-1-1.

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.

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