Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis.

Susac syndrome is a rare, probably immune-mediated endotheliopathy presenting with encephalopathy, sensorineural hearing loss and retinal arterial occlusions. A 33-year-old female with Susac syndrome was worsening despite high-dose steroids so a brain biopsy was performed which suggested a possible fungal infection. Treatment with amphotericin B resulted in prompt reversal of symptoms and radiological findings, and no further symptoms occurred during 8 years of follow-up. A diagnosis of fungal infection was not confirmed. The etiology of Susac syndrome is unknown and this anecdotal observation suggests that an infectious agent susceptible to amphotericin might have caused or triggered Susac syndrome in this patient.

A síndrome de Susac é uma endoteliopatia rara, provavelmente imunomediada que se apresenta com a tríade sindromática de encefalopatia, surdez neurosensorial e oclusões de ramos arteriais da retina. Apresenta-se o caso de uma mulher de 33 anos com o diagnóstico de síndrome de Susac que agravou sob terapêutica imunossupressora, pelo que se decidiu pela realização de uma biópsia cerebral, que levantou a suspeita de uma possível infeção fúngica. O tratamento com anfotericina B resultou numa recuperação clínica e imagiológica rápida e não se verificou recorrência de sintomas num período de seguimento de oito anos. O diagnóstico de meningoencefalite fúngica não foi confirmado. A etiologia da síndrome de Susac é desconhecida. A observação fortuita da resposta terapêutica deste caso sugere que um agente infecioso suscetível à anfotericina B possa ter causado ou desencadeado esta síndrome, nesta doente.

Goya's Lost Hearing: A Twenty-First Century Perspective on Its Cause, Effects and Possible Treatment.

Francisco Goya produced over 1,800 works during his long career, which earned him a reputation as one of the greatest artists of modern times. When he was 47, he developed a mysterious illness that destroyed his hearing, and for the next 35 years left him "deaf as a stump." Of the diagnoses proposed to date, Susac syndrome is the one most consistent with what little we do know of the character of that illness. If he were alive today, given the severity of his hearing loss, Goya would be treated with a cochlear implant, which might restore his ability to communicate in spoken language, though probably not to the level preceding his 1793 illness.