title: Recurrence of Susac Syndrome following 23 Years of Remission.
abstract: Susac syndrome is an autoimmune microangiopathy affecting the brain, retina and inner ear (cochlea and semicircular canals), leading to encephalopathy, branch retinal artery occlusions (BRAOs) and asymmetric neurosensory hearing loss, respectively. The natural history and long-term prognosis are variable as the disease has been shown to be monophasic and self-limiting, polycyclic or chronic continuous. We describe a 35-year-old woman who presented with a sudden hearing loss in the left ear in the 37th week of her second pregnancy. She subsequently developed BRAO in the right eye 2.5 months after having given birth. MRI findings included round lesions in the corpus callosum which are pathognomonic for Susac syndrome. Previous patient records documented encephalopathy, sudden deafness of the right ear and visual field defects in the left eye at the age of 12, followed by permanent hearing and visual defects. We expand on the variability in the course of Susac syndrome as recurrence may occur after as long as 23 years. Cases of monophasic self-limiting Susac syndrome may in fact turn polycyclic with an interval of more than 2 decades between the bouts of the disease. In these cases, suspecting the development of exacerbation early is important in order to start the treatment promptly.
authors.affiliation: Neurology, Department of Neuroscience, Oncology and Radiation Science, Uppsala University, Uppsala, Sweden.
authors.forename: Amalia
authors.initials: A
authors.lastname: Feresiadou
authors.affiliation: Ophthalmology Units, Department of Neuroscience, Oncology and Radiation Science, Uppsala University, Uppsala, Sweden.
authors.forename: Urban
authors.initials: U
authors.lastname: Eriksson
authors.affiliation: Otolaryngology Unit, Department of Surgical Sciences, Oncology and Radiation Science, Uppsala University, Uppsala, Sweden.
authors.forename: Hans-Christian
authors.initials: HC
authors.lastname: Larsen
authors.affiliation: Radiology Unit, Department of Radiology, Oncology and Radiation Science, Uppsala University, Uppsala, Sweden.
authors.forename: Raili
authors.initials: R
authors.lastname: Raininko
authors.affiliation: Neurology, Department of Neuroscience, Oncology and Radiation Science, Uppsala University, Uppsala, Sweden.
authors.forename: Ingela
authors.initials: I
authors.lastname: Nygren
authors.affiliation: Neurology, Department of Neuroscience, Oncology and Radiation Science, Uppsala University, Uppsala, Sweden.
authors.forename: Atle
authors.initials: A
authors.lastname: Melberg
keywords: Branched retinal artery occlusion; Corpus callosum; Encephalopathy; Hearing loss; Recurrence; Retinal vasculopathy; Susac syndrome; Visual loss