Unilateral central retinal artery occlusion as the sole presenting sign of Susac syndrome in a young man: case report.
We report the case of a 24-year-old man presenting with sudden visual loss in the left eye from a central retinal artery occlusion. An extensive clinical investigation revealed no etiology. Three weeks later, however, the patient developed hearing loss followed by encephalopathy and multiple branch retinal artery occlusions in the right eye. Fluorescein angiography confirmed retinal vascular occlusions with no sign of vasculitis. The neurological examination revealed a diffuse encephalopathy while the MRI scan disclosed several small areas of infarcts in the brain. Bilateral sensorineural hearing loss was confirmed on audiometry. The patient was diagnosed with Susac syndrome and treated with methylprednisolone and cyclophosphamide, resulting in slight improvement and stabilization. This case shows that Susac syndrome may be diagnosed late due to the absence at onset of one or more of the symptoms of the classic triad (encephalopathy, multiple branch retinal artery occlusions and hearing loss). This case also serves to emphasize that Susac syndrome should be considered in the differential diagnosis of central retinal artery occlusion, even in apparently healthy young men.
Department of Neurology, Universidade de São Paulo - USP - São Paulo (SP), Brazil.
Samira Luiza dos
SL
Apóstolos-Pereira
Lúcia B Passos
LB
Kara-José
Paulo Euripedes
PE
Marchiori
Mário Luiz Ribeiro
ML
Monteiro
D003520:Cyclophosphamide / Q000627:therapeutic use; D005451:Fluorescein Angiography; D034381:Hearing Loss / Q000209:etiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D018696:Neuroprotective Agents / Q000627:therapeutic use; D015356:Retinal Artery Occlusion / Q000209:etiology*; D055955:Susac Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D014786:Vision Disorders / Q000209:etiology*; D055815:Young Adult
D007166:Immunosuppressive Agents; D018696:Neuroprotective Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone