Susac’s syndrome : 2 cases without hearing loss

Abstract

Susac’s syndrome is a disease of the microvasculature in the brain, inner ear (cochlea and vestibular apparatus) and retina that consists of a clinical triad of encephalopathy, hearing loss and visual disturbance. It is usually seen in young women. It is first defined by Susac et al. in 1979. Diagnosis is often difficult because the classic triad may take up to 2 years to develop and radiological findings are easily confused with multiple sclerosis. Also, encephalopathy may mask some other findings. We present two cases that we think are particularly instructive because they were male patients, had somewhat unusual presenting symptoms, had no accompanying hearing loss, required relatively less aggressive and less sustained immunosuppression than usual, and followed a relatively short and benign course, without relapse and with excellent outcome.  They also serve as good examples of the apparent efficacy and sufficiency (in their cases) of IVIG.

Keywords

• Susac's Syndrome; Retinal Artery Occlusion; Hearing Loss; IVIG

Susac’s syndrome: 2 cases without hearing loss

Öz

References

1. Barker RA, Anderson JR, Meyer P, Dick DJ, Scolding NJ. Microangiopathy of the brain and retina with hearing loss in a 50 year old woman: extending the spectrum of Susac’syndrome. Journal of Nurology, Neurosurgery and Psychiatry 1999;66:641-3
2. O’Halloran HS, Pearson PA, Lee WB, Susac JO, Berger JR. Microangiopathy of the brain, retine and cochlea (Susac syndrome). A report of five cases and a review of the literature. Ophthalmology 1998;105:1038-42
3. Schwitter J, Agosti R, Ott P, Kalman A, Waespe W. Small infarctions of cochlear, retinal and encephalic tissue in young women. Stroke 1992;23:903-7
4. Susac JO. Susac’s Syndrome. Neurology 1994;44:591-3
5. Susac JO, Hardman MD, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979;29:313-6
6. Bitra RK, Eggenberger E. Review of Susac syndrome. Curr Opin Ophthalmol.2011;22(6):472-6
7. Greco A, De Virgilio A, Gallo A, Fusconi M, Turchetta R, Tombolini M, Rizzo MI, de Vincentiis M. Susac's syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 2014;13(8):814-21
8. Kleffner I, Duning T, Lohmann H, Deppe M, Basel T, Promesberger J, et al. A brief review of Susac syndrome. J Neurol Sci. 2012;322(1-2):35-40

9. Saw VP, Canty PA, Green CM et al. : Susac Syndrome: microangiopathy of the retina, cochlea and brain. Clinical and Experimental Ophthalmology 2000;28:373-80
10. Dörr J, Radbruch H, Bock M, Wuerfel J, Brüggemann A, Wandinger KP, et al. Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome. Nat Rev Neurol. 2009;5(12):683-8
11. Oliveira P, Mayeux J. Teaching neuroimages: snowball-like lesions with sudden hearing loss. Neurology. 2014;82(12):100
12. Papo T, Biousse V, Lehoang P et al. Susac Syndrome. Medicine(Baltimore) 1998;77:3-9
13. Petty GW, Matteson EL, Younge BR, Mcdonald TJ, Wood CP. Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years. Mayo Clinic Proceedings 2001;76:958-60
14. Susac JO, Egan RA, Rennebohm RM, Lubow MJ. Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 2007;257(1-2):270-2
15. Feresiadou A, Eriksson U, Larsen HC, Raininko R, Nygren I, Melberg A. Recurrence of Susac Syndrome following 23 Years of Remission. Case Rep Neurol. 2014 May 21;6(2):171-5
16. Mateen FJ, Zubkov AY, Muralidharan R, Fugate JE, Rodriguez FJ, Winters JL, et al. Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol. 2012 ;19(6):800-11
17. Dörr J, Ringelstein M, Duning T, Kleffner I. Update on Susac Syndrome: New Insights in Brain and Retinal Imaging and Treatment Options. J Alzheimers Dis. 2014; 42(3):99-108
18. Erer S, Boz M, Taskapilioglu O, Hakyemez B, Kaderli B, Zarifoglu M. The first Susac's syndrome case in Turkey. J Neurol Sci. 2006 Dec 21;251(1-2):134-7
19. Ozturk A, Degirmenci Y, Tunc M, Kececi H. Susac's syndrome: a case of simultaneous development of all three components of the triad. J Neurol Sci. 2013 Jan 15;324(1-2):187-9
20. Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO. Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model. Pediatr Rheumatol Online J. 2008 Jan 29;6:3.