Contributions
Abstract: EP1431
Type: ePoster
Abstract Category: Clinical aspects of MS - 10 Neuro-ophthalmology
Background: Recently proposed diagnostic criteria Susac syndrome (SuS) may not serve patients who have early or atypical SuS. A classification criteria will enable identification and facilitate research for those patients.
Objective: To propose classification criteria and rules for classification for SuS that recognize heterogeneity in limited forms of disease.
Methodology: Using reported manifestations of SuS, criteria for demonstrating Eye, Ear and Brain involvement were developed. Corpus callosum (CC) lesions were used as an independent biomarker. Classification Rules include all combinations of the presence/absence of CC lesions and the classic triad.
Results: Eye involvement can be demonstrated by:
1) Branch/Central Retinal Artery Occlusion (BRAO),
2) arterial wall hyperfluoresence on fluroscein angiogram,
3) history of purported past BRAO, without current FA evidence of diminished dye flow through any vessels but with residual scotoma (documented by visual field testing) and OCT evidence of past BRAO, or
4) chronic changes in the retinal periphery that are consistent with residual damage from past Susac retinal vasculopathy (such as capillary dropout, peripheral non-perfusion, neovascularization) and are best explained by SuS.
Brain Involvement can be demonstrated by diffuse encephalopathy and/or a neuropsychiatric syndrome such as psychosis, paranoia, personality change, or amnesia. Ear involvement can be demonstrated with subacute/acute onset of unilateral or bilateral low frequency sensorineural hearing loss, documented by audiogram. Classical CC lesions include typical “snowball” lesions (of various sizes and shapes), “spoke” lesions, “icicle” lesions, and corpus callosal “holes” that are located in the central portion of the CC. The number of lesions may vary from many to a single (but definite) lesion. Classification rules recognize all possible combinations of the above findings and with a total of 11 categories, including definite/probable/possible/isolated feature and classical/atypical depending on presence of CC lesions.
Conclusion: The proposed NASSC Classification Criteria use established manifestations of SuS including the presence/absence of the complete triad and CC lesions to provide labels for early SuS who do not meet published diagnostic criteria.
Disclosure: Robert Carruthers: Nothing to disclose
Gillian Paton: Nothing to disclose
Claire Sheldon: Nothing to disclose
Talia Vertinsky: Nothing to disclose
Anthony Traboulsee: Nothing to disclose
Mollie Carruthers: Nothing to disclose
Robert Rennebohm: Nothing to disclose
Abstract: EP1431
Type: ePoster
Abstract Category: Clinical aspects of MS - 10 Neuro-ophthalmology
Background: Recently proposed diagnostic criteria Susac syndrome (SuS) may not serve patients who have early or atypical SuS. A classification criteria will enable identification and facilitate research for those patients.
Objective: To propose classification criteria and rules for classification for SuS that recognize heterogeneity in limited forms of disease.
Methodology: Using reported manifestations of SuS, criteria for demonstrating Eye, Ear and Brain involvement were developed. Corpus callosum (CC) lesions were used as an independent biomarker. Classification Rules include all combinations of the presence/absence of CC lesions and the classic triad.
Results: Eye involvement can be demonstrated by:
1) Branch/Central Retinal Artery Occlusion (BRAO),
2) arterial wall hyperfluoresence on fluroscein angiogram,
3) history of purported past BRAO, without current FA evidence of diminished dye flow through any vessels but with residual scotoma (documented by visual field testing) and OCT evidence of past BRAO, or
4) chronic changes in the retinal periphery that are consistent with residual damage from past Susac retinal vasculopathy (such as capillary dropout, peripheral non-perfusion, neovascularization) and are best explained by SuS.
Brain Involvement can be demonstrated by diffuse encephalopathy and/or a neuropsychiatric syndrome such as psychosis, paranoia, personality change, or amnesia. Ear involvement can be demonstrated with subacute/acute onset of unilateral or bilateral low frequency sensorineural hearing loss, documented by audiogram. Classical CC lesions include typical “snowball” lesions (of various sizes and shapes), “spoke” lesions, “icicle” lesions, and corpus callosal “holes” that are located in the central portion of the CC. The number of lesions may vary from many to a single (but definite) lesion. Classification rules recognize all possible combinations of the above findings and with a total of 11 categories, including definite/probable/possible/isolated feature and classical/atypical depending on presence of CC lesions.
Conclusion: The proposed NASSC Classification Criteria use established manifestations of SuS including the presence/absence of the complete triad and CC lesions to provide labels for early SuS who do not meet published diagnostic criteria.
Disclosure: Robert Carruthers: Nothing to disclose
Gillian Paton: Nothing to disclose
Claire Sheldon: Nothing to disclose
Talia Vertinsky: Nothing to disclose
Anthony Traboulsee: Nothing to disclose
Mollie Carruthers: Nothing to disclose
Robert Rennebohm: Nothing to disclose