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 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.
Department of Functional Neurology, Hopital Neurologique, Bron Cedex, France. anne-evelyne.vallet@chu-lyon.fr
Anne-Evelyne
AE
Vallet
Adrien
A
Didelot
Fitsum
F
Guebre-Egziabher
Martine
M
Bernard
François
F
Mauguière
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001766:Blindness / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D004172:Diplopia / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D004359:Drug Therapy, Combination; D004576:Electromyography; D004596:Electroretinography; D005074:Evoked Potentials, Visual / Q000502:physiology; D005451:Fluorescein Angiography; D013700:Giant Cell Arteritis / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D020423:Median Neuropathy / Q000175:diagnosis / Q000503:physiopathology; D008775:Methylprednisolone / Q000627:therapeutic use; D018908:Muscle Weakness / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D018917:Optic Neuropathy, Ischemic / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D011241:Prednisone / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone; D008775:Methylprednisolone