Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary InvolvementReport as inadecuate

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Case Reports in Neurological Medicine - Volume 2017 2017, Article ID 2807461, 4 pages - https:-doi.org-10.1155-2017-2807461

Case Report

King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia

King Fahad Medical City, Riyadh, Saudi Arabia

Correspondence should be addressed to Roaa Ridha Amer

Received 13 April 2017; Accepted 8 May 2017; Published 23 May 2017

Academic Editor: Samuel T. Gontkovsky

Copyright © 2017 Roaa Ridha Amer et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Polyostotic sclerosing histiocytosis, also known as Erdheim-Chester disease ECD, is a rare form of non-Langerhans histiocytosis. ECD has wide clinical spectrums which mainly affect skeletal, neurological, dermatological, retroperitoneal, cardiac, and pulmonary manifestations. Here we describe a case of ECD in a 45-year-old female who presented initially with bilateral knee pain and homonymous superior quadrantanopia progressed to ophthalmoplegia and complete visual loss of the left eye over a period of one year. Plain X-ray of both knees showed bilateral patchy sclerosis of the distal femur and upper parts of the tibiae. Initial brain magnetic resonance imaging MRI showed bilateral enhancing masses in the temporal lobes anterior to the temporal horns, thickening of the pituitary stalk, partially empty sella, and involvement of the left cavernous sinus one year later. Our case is a peculiar case of ECD initially presented with unilateral homonymous superior quadrantanopia due to involvement of the visual apparatus in the mesial temporal lobe which progressed to unilateral ophthalmoplegia and total visual loss secondary to involvement of the cavernous sinus. Thus, the diagnosis of ECD should be kept in mind in the presence of bilateral bone sclerotic lesions.

Author: Roaa Ridha Amer, Sara Mohammed Qubaiban, and Eman Abdulkarim Bakhsh

Source: https://www.hindawi.com/


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