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BMJ Case Rep. 2010; 2010: bcr0820092219.
Published online Oct 12, 2010. doi:  10.1136/bcr.08.2009.2219
PMCID: PMC3029921
Rare disease
Initial neuro-ophthalmological manifestations in Churg–Strauss syndrome
Anne-Evelyne Vallet,1 Adrien Didelot,1 Fitsum Guebre-Egziabher,2 Martine Bernard,3 and François Mauguière1
1Department of Functional Neurology, Hopital Neurologique, Bron Cedex, France
2Department of Nephrology, Edouard Herriot Hospital, Lyon Cedex, France
3Department of Neuro-Ophthalmology, Hopital Neurologique, Bron Cedex, France
Correspondence to Anne-Evelyne Vallet, anne-evelyne.vallet/at/chu-lyon.fr
Abstract
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.
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