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.

Highlights

  • A 56 year old male presented to his family doctor with frequent headaches and blurred vision

  • He was diagnosed with possible idiopathic intracranial hypertension (IIH) and commenced on acetazolamide 250mg twice daily

  • Biopsy of a sclerotic bone lesion in the pelvis revealed histiocytes that were CD68 positive, CD1a negative, and BRAF V600E mutation positive. These findings were consistent with non-Langerhans histiocytosis, or Erdheim-Chester disease (ECD)

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Summary

Introduction

A 56 year old male presented to his family doctor with frequent headaches and blurred vision. Cite This Article: Emma Foster, Mastura Monif, Kylie Mason, and Anneke van der Walt, “Diagnosis within a Diagnosis: An Unusual Case of Intracranial Hypertension.” Neuro-Ophthalmology & Visual Neuroscience, vol 2, no. His past medical history consisted of the following: (1) systemic hypertension, (2) surgically treated left retinal detachment 28 years prior with residual visual acuity of 6/24 in that eye, and (3) hyperopia managed with reading glasses. Relevant examination findings included Frisen grade 1-2 papilledema (Figure 1) and reduced visual acuity of 6/9 in the right eye and 6/24-1 in the left eye.

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