Susac’s Syndrome: A Case Report

Öz

Susac’s syndrome (SS) is a common infarct arteriolar occlusive disease. It consists of a triad of symptoms: encephalopathy, branch retinal artery occlusions (BRAO), and hearing loss. It usually affects women aged 20 to 40. The precise cause is not known, but the symptoms usually include hearing loss and vision loss. We report a case of SS in a 52-year-old woman. She initially was admitted to the hospital with signs of loss of consciousness and hearing. An audiogram taken for the patient showed bilateral severe sensorineural hearing loss which affected all frequencies (right 82/80; left 110/78 dB). Speech reception thresholds (SDS) of 65 dB to 70 dB on the left to the right were observed and speech scores could not be obtained. A tympanogram of Curve Type A was obtained. The patient experienced sudden sensorineural hearing loss (SSHL) and treatment of SSHL was started immediately. Patients of long-duration stays at the clinic often experienced decreased vision and reduced coordination. Cranial magnetic resonance imaging (MRI), Axial T2-FLAİR images showed multiple hyperintense lesions involving cerebral white matter, and left internal-external capsule; sagittal T2W images showed hyperintense lesions involving periventriculer cerebral white matter. No significant changes in hearing, but improvements in the patient’s coordiantion and vision, were observed at a six-month follow up. The patient was given hearing aids at the follow up as well. For all those patients with sudden hearing loss, confusion, and blurred vision, retinocohleocerebral disease that Susac’s syndrome should be considered. Funduscopic eye examination, audiometry, and MRI are the first steps in diagnosis. Case report (J Int Dent Med Res 2013; 6: (3), pp. 136-139)

Anahtar Kelimeler

• Susac’s syndome, branch retinal artery occlusions, retinocochleocerebral Vasculopathy

Susac’s Syndrome: A Case Report

Öz

Anahtar Kelimeler

• -

Kaynakça

1. Petty GW, Engel AG, Younge BR, Duffy J, Yanagihara JE, Kasperbauer T, Lucchinetti
2. JL, Rodriguez M. Retinocochleocerebral vasculopathy. Medicine
3. (Baltimore) Jan 1998, 77(1): 12–40. JD, Parisi
4. Susac JO, Hardman JM, Selhorst JB: Microangiopathy of the brain and retina. Neurology 1979, 29(3): 313–316.
5. Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M,
6. Duning T, Aktas O, Ringelstein EB, Paul F, Kleffner I. Characteristics of Susac’s syndrome: a review of all reported cases. doi:10.1038/nrneurol.2013.82. publication, 30 April 2013;
7. Petty GW, Engel AG, Younge BR, Duffy J, Yanagihara T, Lucchinetti CF, Bartleson JD, Parisi JE, Kasperbauer JL, Rodriguez M: Retinocochleocerebral vasculopathy.
8. O’Halloran HS, Pearson PA, Lee WB, Susac JO, Berger JR:

9. Microangiopathy of the brain, retina, and cochlea (Susac’s
10. syndrome). A report of five cases and a review of the literature.
11. Ophthalmology 1998, 105(6): 1038–1044
12. Petty GW, Engel AG, Younge BR, et al. Retinocochleocerebral vasculopathy. Medicine (Baltimore) 1998, 77: 12–40.
13. Monteiro ML, Swanson RA, Coppeto JR, Cuneo RA, DeArmond SJ, encephalopathy, hearing loss, and retinal arteriolar occlusions. Neurology 1985, 35: 1113–1121.
14. microangiopathic syndrome of
15. Petty GW, Yanagihara T, Bartleson JD, Younge BR, Mokri B. Retinocochleocerebral vasculopathy [abstract P30]. Ann. Neurol. 30 1991, 245.
16. O’Halloran HS, Pearson PA, Lee WB, Susac JO, Berger JR.
17. Microangiopathy of the brain, retina, and cochlea (Susac’s syndrome): a report of five cases and a review of the literature. Ophthalmology 1998, 105: 1038–1044. 1994, 44: 591–593. –1690. 1975–2005
18. microangiopathy/autoimmune
19. endotheliopathy. J. Neurol. Sci. 2007, 257: 270–272. –1611.