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Susac’s syndrome : 2 cases without hearing loss

Year 2015, Volume: 2 Issue: 5, 308 - 13, 06.07.2015

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.

References

  • 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
  • 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
  • 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
  • Susac JO. Susac’s Syndrome. Neurology 1994;44:591-3
  • Susac JO, Hardman MD, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979;29:313-6
  • Bitra RK, Eggenberger E. Review of Susac syndrome. Curr Opin Ophthalmol.2011;22(6):472-6
  • 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
  • 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
  • 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
  • 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
  • Oliveira P, Mayeux J. Teaching neuroimages: snowball-like lesions with sudden hearing loss. Neurology. 2014;82(12):100
  • Papo T, Biousse V, Lehoang P et al. Susac Syndrome. Medicine(Baltimore) 1998;77:3-9
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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.

Susac’s syndrome: 2 cases without hearing loss

Year 2015, Volume: 2 Issue: 5, 308 - 13, 06.07.2015

Abstract

References

  • 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
  • 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
  • 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
  • Susac JO. Susac’s Syndrome. Neurology 1994;44:591-3
  • Susac JO, Hardman MD, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979;29:313-6
  • Bitra RK, Eggenberger E. Review of Susac syndrome. Curr Opin Ophthalmol.2011;22(6):472-6
  • 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
  • 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
  • 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
  • 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
  • Oliveira P, Mayeux J. Teaching neuroimages: snowball-like lesions with sudden hearing loss. Neurology. 2014;82(12):100
  • Papo T, Biousse V, Lehoang P et al. Susac Syndrome. Medicine(Baltimore) 1998;77:3-9
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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.
There are 20 citations in total.

Details

Primary Language English
Journal Section Case Reports
Authors

Serkan Demir

Mehmet Senol

Rifat Togrol This is me

Ali Ayata This is me

Eyup Duzgun This is me

Publication Date July 6, 2015
Published in Issue Year 2015 Volume: 2 Issue: 5

Cite

APA Demir, S., Senol, M., Togrol, R., Ayata, A., et al. (2015). Susac’s syndrome : 2 cases without hearing loss. Medical Science and Discovery, 2(5), 308-13. https://doi.org/10.17546/msd.65929
AMA Demir S, Senol M, Togrol R, Ayata A, Duzgun E. Susac’s syndrome : 2 cases without hearing loss. Med Sci Discov. July 2015;2(5):308-13. doi:10.17546/msd.65929
Chicago Demir, Serkan, Mehmet Senol, Rifat Togrol, Ali Ayata, and Eyup Duzgun. “Susac’s Syndrome : 2 Cases Without Hearing Loss”. Medical Science and Discovery 2, no. 5 (July 2015): 308-13. https://doi.org/10.17546/msd.65929.
EndNote Demir S, Senol M, Togrol R, Ayata A, Duzgun E (July 1, 2015) Susac’s syndrome : 2 cases without hearing loss. Medical Science and Discovery 2 5 308–13.
IEEE S. Demir, M. Senol, R. Togrol, A. Ayata, and E. Duzgun, “Susac’s syndrome : 2 cases without hearing loss”, Med Sci Discov, vol. 2, no. 5, pp. 308–13, 2015, doi: 10.17546/msd.65929.
ISNAD Demir, Serkan et al. “Susac’s Syndrome : 2 Cases Without Hearing Loss”. Medical Science and Discovery 2/5 (July 2015), 308-13. https://doi.org/10.17546/msd.65929.
JAMA Demir S, Senol M, Togrol R, Ayata A, Duzgun E. Susac’s syndrome : 2 cases without hearing loss. Med Sci Discov. 2015;2:308–13.
MLA Demir, Serkan et al. “Susac’s Syndrome : 2 Cases Without Hearing Loss”. Medical Science and Discovery, vol. 2, no. 5, 2015, pp. 308-13, doi:10.17546/msd.65929.
Vancouver Demir S, Senol M, Togrol R, Ayata A, Duzgun E. Susac’s syndrome : 2 cases without hearing loss. Med Sci Discov. 2015;2(5):308-13.