Initial neuro-ophthalmological manifestations in Churg–Strauss syndrome

Churg–Strauss syndrome (CSS) is a systemic vasculitis with frequent respiratory tract involvement. It can also affect the nervous system, notably the optic tract. The present work reports the case of a 65-year-old man diagnosed as having CSS in the context of several acute onset neurological symptom...

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Bibliographic Details
Published in:BMJ Case Reports 2010, Vol.2010
Main Authors: Vallet, Anne-Evelyne, Didelot, Adrien, Guebre-Egziabher, Fitsum, Bernard, Martine, Mauguière, François
Format: Report
Language:English
Online Access:Get full text
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Summary:Churg–Strauss syndrome (CSS) is a systemic vasculitis with frequent respiratory tract involvement. It can also affect the nervous system, notably the optic tract. The present work reports the case of a 65-year-old man diagnosed as having CSS in the context of several acute onset neurological symptoms including muscle weakness and signs of temporal arteritis, including bilateral anterior ischaemic optic neuropathy (ON). Electroretinograms (ERGs) and visual evoked potentials (VEPs) were performed. Flash ERGs were normal whereas VEPs were highly abnormal, showing a dramatic voltage reduction, thus confirming the ON. The vision outcome was poor. Ophthalmological presentations of CSS have rarely been reported, but no previous case of sudden blindness documented by combined ERG and VEP investigations were found in the literature. The present case strongly suggests that the occurrence of visual loss in the context of systemic inflammation with hypereosinophilia should lead to considering the diagnosis of CSS.
ISSN:1757-790X
DOI:10.1136/bcr.08.2009.2219