Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary Involvement

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 ca...

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Main Authors: Roaa Ridha Amer, Sara Mohammed Qubaiban, Eman Abdulkarim Bakhsh
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2017/2807461
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author Roaa Ridha Amer
Sara Mohammed Qubaiban
Eman Abdulkarim Bakhsh
author_facet Roaa Ridha Amer
Sara Mohammed Qubaiban
Eman Abdulkarim Bakhsh
author_sort Roaa Ridha Amer
collection DOAJ
description 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.
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spelling doaj-art-c89c4bc7b77e4327b0e6f79610b8ab5d2025-02-03T01:22:01ZengWileyCase Reports in Neurological Medicine2090-66682090-66762017-01-01201710.1155/2017/28074612807461Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary InvolvementRoaa Ridha Amer0Sara Mohammed Qubaiban1Eman Abdulkarim Bakhsh2King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi ArabiaKing Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi ArabiaKing Fahad Medical City, Riyadh, Saudi ArabiaPolyostotic 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.http://dx.doi.org/10.1155/2017/2807461
spellingShingle Roaa Ridha Amer
Sara Mohammed Qubaiban
Eman Abdulkarim Bakhsh
Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary Involvement
Case Reports in Neurological Medicine
title Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary Involvement
title_full Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary Involvement
title_fullStr Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary Involvement
title_full_unstemmed Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary Involvement
title_short Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary Involvement
title_sort homonymous superior quadrantanopia due to erdheim chester disease with asymptomatic pituitary involvement
url http://dx.doi.org/10.1155/2017/2807461
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AT saramohammedqubaiban homonymoussuperiorquadrantanopiaduetoerdheimchesterdiseasewithasymptomaticpituitaryinvolvement
AT emanabdulkarimbakhsh homonymoussuperiorquadrantanopiaduetoerdheimchesterdiseasewithasymptomaticpituitaryinvolvement