Karen L. Salzman, MD; Jeffrey S. Anderson, MD, PhD
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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Retinocochleocerebral vasculopathy, formerly known as small infarctions of cochlear, retinal, and encephalic tissue (SICRET syndrome)
Definitions
Microangiopathy of brain, retina, and cochlea
IMAGING
General Features
Imaging Recommendations
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bersano A et al: Heritable and non-heritable uncommon causes of stroke. J Neurol. ePub, 2020
Haralur Y et al: Neuroimaging of Multiple Sclerosis Mimics. Neurol Clin. 38(1):149-70, 2020
Machado S et al: Cognitive dysfunction and brain atrophy in Susac syndrome. J Neurol. 267(4):994-1003, 2020
Ramos-Ruperto L et al: Susac syndrome and pregnancy: a relationship to clarify. About two cases and review of the literature. J Matern Fetal Neonatal Med. 1-6, 2020
Agamanolis DP et al: Brain microvascular pathology in Susac syndrome: an electron microscopic study of five cases. Ultrastruct Pathol. 43(6):229-36, 2019
Coulette S et al: Diagnosis and prediction of relapses in susac syndrome: a new use for MR postcontrast FLAIR leptomeningeal enhancement. AJNR Am J Neuroradiol. 40(7):1184-90, 2019
Egan RA: Diagnostic criteria and treatment algorithm for Susac syndrome. J Neuroophthalmol. 39(1):60-7, 2019
Gross CC et al: CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 10(1):5779, 2019
Law LY et al: The spectrum of immune-mediated and inflammatory lesions of the brainstem: clues to diagnosis. Neurology. 93(9):390-405, 2019
Onat Demirci N et al: Susac's syndrome as a rare arterial stroke mimic. Neuroradiol J. 32(3):200-2, 2019
Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
Marrodan M et al: Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system. J Neurol Sci. 395:29-34, 2018
Rennebohm RM et al: Guidelines for treatment of Susac syndrome - an update. Int J Stroke. 1747493017751737, 2018
Sarbu N et al: White matter diseases with radiologic-pathologic correlation. Radiographics. 36(5):1426-47, 2016
Filippi CG et al: Lesions of the corpus callosum and other commissural fibers: diffusion tensor studies. Semin Ultrasound CT MR. 35(5):445-58, 2014
Greco A et al: Susac's syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 13(8):814-21, 2014
Allmendinger AM et al: Diffuse cauda equina enhancement in a middle aged male with Susac syndrome and symptomatic cauda equina syndrome. J Neurol Sci. 333(1-2):25-8, 2013
Dörr J et al: Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 9(6):307-16, 2013
Mateen FJ et al: Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol. 19(6):800-11, 2012
Bitra RK et al: Review of Susac syndrome. Curr Opin Ophthalmol. 22(6):472-6, 2011
Kleffner I et al: Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology. 70(19 Pt 2):1867-9, 2008
Susac JO et al: Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 257(1-2):270-2, 2007
Do TH et al: Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol. 25(3):382-8, 2004
White ML et al: Evolution of lesions in Susac syndrome at serial MR imaging with diffusion-weighted imaging and apparent diffusion coefficient values. AJNR Am J Neuroradiol. 25(5):706-13, 2004
Xu MS et al: Susac syndrome: serial diffusion-weighted MR imaging. Magn Reson Imaging. 22(9):1295-8, 2004
Susac JO et al: MRI findings in Susac's syndrome. Neurology. 61(12):1783-7, 2003
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Retinocochleocerebral vasculopathy, formerly known as small infarctions of cochlear, retinal, and encephalic tissue (SICRET syndrome)
Definitions
Microangiopathy of brain, retina, and cochlea
IMAGING
General Features
Imaging Recommendations
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bersano A et al: Heritable and non-heritable uncommon causes of stroke. J Neurol. ePub, 2020
Haralur Y et al: Neuroimaging of Multiple Sclerosis Mimics. Neurol Clin. 38(1):149-70, 2020
Machado S et al: Cognitive dysfunction and brain atrophy in Susac syndrome. J Neurol. 267(4):994-1003, 2020
Ramos-Ruperto L et al: Susac syndrome and pregnancy: a relationship to clarify. About two cases and review of the literature. J Matern Fetal Neonatal Med. 1-6, 2020
Agamanolis DP et al: Brain microvascular pathology in Susac syndrome: an electron microscopic study of five cases. Ultrastruct Pathol. 43(6):229-36, 2019
Coulette S et al: Diagnosis and prediction of relapses in susac syndrome: a new use for MR postcontrast FLAIR leptomeningeal enhancement. AJNR Am J Neuroradiol. 40(7):1184-90, 2019
Egan RA: Diagnostic criteria and treatment algorithm for Susac syndrome. J Neuroophthalmol. 39(1):60-7, 2019
Gross CC et al: CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 10(1):5779, 2019
Law LY et al: The spectrum of immune-mediated and inflammatory lesions of the brainstem: clues to diagnosis. Neurology. 93(9):390-405, 2019
Onat Demirci N et al: Susac's syndrome as a rare arterial stroke mimic. Neuroradiol J. 32(3):200-2, 2019
Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
Marrodan M et al: Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system. J Neurol Sci. 395:29-34, 2018
Rennebohm RM et al: Guidelines for treatment of Susac syndrome - an update. Int J Stroke. 1747493017751737, 2018
Sarbu N et al: White matter diseases with radiologic-pathologic correlation. Radiographics. 36(5):1426-47, 2016
Filippi CG et al: Lesions of the corpus callosum and other commissural fibers: diffusion tensor studies. Semin Ultrasound CT MR. 35(5):445-58, 2014
Greco A et al: Susac's syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 13(8):814-21, 2014
Allmendinger AM et al: Diffuse cauda equina enhancement in a middle aged male with Susac syndrome and symptomatic cauda equina syndrome. J Neurol Sci. 333(1-2):25-8, 2013
Dörr J et al: Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 9(6):307-16, 2013
Mateen FJ et al: Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol. 19(6):800-11, 2012
Bitra RK et al: Review of Susac syndrome. Curr Opin Ophthalmol. 22(6):472-6, 2011
Kleffner I et al: Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology. 70(19 Pt 2):1867-9, 2008
Susac JO et al: Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 257(1-2):270-2, 2007
Do TH et al: Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol. 25(3):382-8, 2004
White ML et al: Evolution of lesions in Susac syndrome at serial MR imaging with diffusion-weighted imaging and apparent diffusion coefficient values. AJNR Am J Neuroradiol. 25(5):706-13, 2004
Xu MS et al: Susac syndrome: serial diffusion-weighted MR imaging. Magn Reson Imaging. 22(9):1295-8, 2004
Susac JO et al: MRI findings in Susac's syndrome. Neurology. 61(12):1783-7, 2003
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