Abstract
Abstract
Susac syndrome is a rare disease of unknown pathogenesis. It is caused by a microangiopathy affecting the arterioles of the brain, retina, and cochlea, giving the classic clinical triad of subacute encephalopathy, visual loss secondary to retinal branch occlusions, and sensorineural hearing loss. Magnetic resonanace imaging, retinal fluorescein angiography, and audiography findings enable diagnosis. Early therapy may reduce sequelae and improve recovery. Herein we report a case of Susac syndrome with all the findings of the disease.
Keywords: Magnetic resonance imaging, microangiopathy, Susac syndrome
Özet
Susac sendromu patogenezi bilinmeyen nadir bir hastalıktır. Beyin, retina ve kohleanın etkilendiği mikroanjiyopati sonucunda ortaya çıkar. Bu durum hastalığın tipik triadı olan subakut ensefalopati, retinal arteriollerin tıkanmasına bağlı oluşan görme kaybı ve sensorinöral işitme kaybına neden olur. Manyetik rezonans görüntüleme, retinal floresan anjiografi ve odyografi bulguları tanıya yardımcıdır. Erken tedavi sekel oluşumunu azaltabilir ve iyileşemeyi hızlandırabilir. Bu yazıda hastalığın tüm bulguları ile birlikte bir Susac sendromlu olguyu sunuyoruz.
Anahtar sözcükler: Manyetik rezonans görüntüleme, mikroanjiyopati, Susac sendromuReferences
- Susac JO, Murtagh FR, Egan RA, Berger JR, Bakshi R, Lincoff N, Gean AD, Galetta SL, Fox RJ, Costello FE, Lee AG, Clark J, Layzer RB, Daroff RB.MRI findings in Susac’s syndrome. Neurology 2003; 61: 1783-7.
- Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979; 29: 313-6.
- Susac JO. Susac’s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 1994; 44: 591-3.
- Mass M, Bourdette D, Bernstein W, Hammerstad J. Retinopathy, encephalopathy, deafness associated microangiopathy (the RED M syndrome): three new cases. Neurology 1988; 38(Suppl): 215.
- Nicolle MW, Mclachlan RS. Microangiopathy with retinopathy, encephalopathy, and deafness (RED-M) and systemic features. Semin Arthritis Rheum 1991; 21: 123-8.
- Schwitter J, Agosti R, Ott P, Kalman A, Waespe W. Small infarctions of cochlear, retinal, and encephalic tissue in young women. Stroke 1992; 23: 903-7.100
- Petty GW, Engel AG, Younge BR, Duffy J, Yanagihara T, Lucchinetti CF, Bartleson JD, Parisi JE, Kasperbauer JL, Rodriguez M. Retinocochleocerebral vasculopathy. Medicine (Baltimore) 1998; 77: 12-40.
- Papo T, Biousse V, Lehoang P, Fardeau C, N'Guyen N, Huong DL, Aumaitre O, Bousser MG, Godeau P, Piette JC. Susac syndrome. Medicine (Baltimore) 1998; 77: 3-11.
- Petty GW, Matteson EL, Younge BR, McDonald TJ, Wood CP. Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years. Mayo Clin Proc 2001; 76: 958-60.
- 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’s syndrome. J Neurol Neurosurg Psychiatry 1999; 66: 641-3.
- O'Halloran HS, Pearson PA, Lee WB, Susac JO, Berger JR. Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature. Ophthalmology 1998; 105: 1038-44.
- Do TH, Fisch C, Evoy F. Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol 2004; 25: 382-8.
- Saw VP, Canty PA, Green CM, Briggs RJ, Cremer PD, Harrisberg B, McCluskey P, O'Day J, Paine M, Wakefield D, Watson JD. Susac syndrome: microangiopathy of the retina, cochlea and brain. Clin Experiment Ophthalmol 2000; 28: 373-81.
- Yellin MW, Johnson TW. A case of Susac syndrome. J Am Acad Audiol 2000; 11: 484-8.
Abstract
Susac sendromu patogenezi bilinmeyen nadir bir hastalıktır. Beyin, retina ve kohleanın etkilendiği mikroanjiyopati sonucunda ortaya çıkar. Bu durum hastalığın tipik triadı olan subakut ensefalopati, retinal arteriollerin tıkanmasına bağlı oluşan görme kaybı ve sensorinöral işitme kaybına neden olur. Manyetik rezonans görüntüleme, retinal floresan anjiografi ve odyografi bulguları tanıya yardımcıdır. Erken tedavi sekel oluşumunu azaltabilir ve iyileşemeyi hızlandırabilir. Bu yazıda hastalığın tüm bulguları ile birlikte bir Susac sendromlu olguyu sunuyoruz.
References
- Susac JO, Murtagh FR, Egan RA, Berger JR, Bakshi R, Lincoff N, Gean AD, Galetta SL, Fox RJ, Costello FE, Lee AG, Clark J, Layzer RB, Daroff RB.MRI findings in Susac’s syndrome. Neurology 2003; 61: 1783-7.
- Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979; 29: 313-6.
- Susac JO. Susac’s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 1994; 44: 591-3.
- Mass M, Bourdette D, Bernstein W, Hammerstad J. Retinopathy, encephalopathy, deafness associated microangiopathy (the RED M syndrome): three new cases. Neurology 1988; 38(Suppl): 215.
- Nicolle MW, Mclachlan RS. Microangiopathy with retinopathy, encephalopathy, and deafness (RED-M) and systemic features. Semin Arthritis Rheum 1991; 21: 123-8.
- Schwitter J, Agosti R, Ott P, Kalman A, Waespe W. Small infarctions of cochlear, retinal, and encephalic tissue in young women. Stroke 1992; 23: 903-7.100
- Petty GW, Engel AG, Younge BR, Duffy J, Yanagihara T, Lucchinetti CF, Bartleson JD, Parisi JE, Kasperbauer JL, Rodriguez M. Retinocochleocerebral vasculopathy. Medicine (Baltimore) 1998; 77: 12-40.
- Papo T, Biousse V, Lehoang P, Fardeau C, N'Guyen N, Huong DL, Aumaitre O, Bousser MG, Godeau P, Piette JC. Susac syndrome. Medicine (Baltimore) 1998; 77: 3-11.
- Petty GW, Matteson EL, Younge BR, McDonald TJ, Wood CP. Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years. Mayo Clin Proc 2001; 76: 958-60.
- 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’s syndrome. J Neurol Neurosurg Psychiatry 1999; 66: 641-3.
- O'Halloran HS, Pearson PA, Lee WB, Susac JO, Berger JR. Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature. Ophthalmology 1998; 105: 1038-44.
- Do TH, Fisch C, Evoy F. Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol 2004; 25: 382-8.
- Saw VP, Canty PA, Green CM, Briggs RJ, Cremer PD, Harrisberg B, McCluskey P, O'Day J, Paine M, Wakefield D, Watson JD. Susac syndrome: microangiopathy of the retina, cochlea and brain. Clin Experiment Ophthalmol 2000; 28: 373-81.
- Yellin MW, Johnson TW. A case of Susac syndrome. J Am Acad Audiol 2000; 11: 484-8.
Details
| Primary Language | English |
|---|---|
| Journal Section | Case Reports |
| Authors | |
| Publication Date | March 28, 2014 |
| Published in Issue | Year 2014Volume: 36 Issue: 1 |