Eales Disease Susac Syndrome Also known as No

Eales Disease Susac Syndrome Also known as… No other names ? I’m aware of Retinocochleocerebral vasculopathy ? SICRET syndrome Process in a nutshell Occlusive retinal vasculopathy Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculopathy Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculopathy Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT What does SICRET stand for? Occlusive retinal vasculopathy Small Infarctions of Cochlear, Retinal, and Encephalic Tissue

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculopathy Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT What does SICRET stand for? Occlusive retinal vasculopathy Small Infarctions of Cochlear, Retinal, and Encephalic Tissue

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal ? vasculopathy Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules ? Arterioles ? AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes ? No ? Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization producing vitreous hemorrhage Multiple BRAOs at non-branch sites; boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization ? producing vitreous hemorrhage Multiple BRAOs at non-branch sites; ? boxcarring of arteriole blood Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations +/- epistaxis Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Susac syndrome. Note the area of infarct doesn’t correspond to a branch-point blockage 19

Retinal Vasculitis Susac syndrome. Note the multiple areas of arteriolar inflammation at non-branch points 20

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult East Asia Indian None Ethnicity If not embolic, what causes BRAO in Susac? Male Female Gender Recall Susac syndrome is an inflammatory condition—specifically, a vasculitis that TB positivity Abnormal CSF studies Key lab finding affects arterioles. It is believed the target of this inflammatory process is the endothelial cells that line the arteriolar TB? lumen. occurs when endothelial-cell (may. Occlusion be idiopathic) Unknown Cause damage compromises the adjacent lumen. Because the amount of non-branch-point Punctate white matter lesions, esp. endothelium dwarfsfinding the amount of branch-point. None endothelium, occlusion will essentially Brain imaging of the corpus callosum; always occur at non-branch points. Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult East Asia Indian None Ethnicity If not embolic, what causes BRAO in Susac? Male Female Gender Recall Susac syndrome is an inflammatory condition—specifically, a vasculitis that TB positivity Abnormal CSF studies Key lab finding affects arterioles. It is believed the target of this inflammatory process is the endothelial two words cells that line the arteriolar lumen. Occlusion occurs when endothelial-cell TB? (may be idiopathic) Unknown Cause damage compromises the adjacent lumen. Because the amount of non-branch-point Punctate white matter lesions, esp. endothelium dwarfsfinding the amount of branch-point. None endothelium, occlusion will essentially Brain imaging of the corpus callosum; always occur at non-branch points. Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult East Asia Indian None Ethnicity If not embolic, what causes BRAO in Susac? Male Female Gender Recall Susac syndrome is an inflammatory condition—specifically, a vasculitis that TB positivity Abnormal CSF studies Key lab finding affects arterioles. It is believed the target of this inflammatory process is the endothelial cells that line the arteriolar TB? lumen. occurs when endothelial-cell (may. Occlusion be idiopathic) Unknown Cause damage compromises the adjacent lumen. Because the amount of non-branch-point Punctate white matter lesions, esp. endothelium dwarfsfinding the amount of branch-point. None endothelium, occlusion will essentially Brain imaging of the corpus callosum; always occur at non-branch points. Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, Venules or arterioles? Why bother mentioning that the BRAOs are occurring at ‘non-branch’ sites? Yes AC/vitreous cell present? Because most BRAOs occur at branch sites neovascularization Classic Why doretinal most finding BRAOs occur at Peripheral branch sites? Because most BRAOs are embolic in nature +/- epistaxis Systemic associations So, putting it all together—the fact that BRAOs in Bilateral sites (may be sequential) Laterality Susac syndrome occur at non-branch suggests what about them? Uncommon How common? It suggests they are not embolic in origin Teens-young adult Age at presentation Arterioles No Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Bilateral Very rare Teens-young adult East Asia Indian None Ethnicity If not embolic, what causes BRAO in Susac? Male Female Gender Recall Susac syndrome is an inflammatory condition—specifically, a vasculitis that TB positivity Abnormal CSF studies Key lab finding affects arterioles. It is believed the target of this inflammatory process is the endothelial cells that line the arteriolar TB? lumen. occurs when endothelial-cell (may. Occlusion be idiopathic) Unknown Cause damage compromises the adjacent lumen. Because the amount of non-branch-point Punctate white matter lesions, esp. endothelium dwarfsfinding the amount of branch-point. None endothelium, occlusion will essentially Brain imaging of the corpus callosum; always occur at non-branch points. Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Systemic associations Laterality How common? Multiple BRAOs at non-branch sites and… Encephalopathy; sensorineural hearing loss There’s another noteworthy DFE finding in Susac syndrome—what is it? +/- epistaxis Boxcarring blood Bilateralof (may be sequential) Bilateral Uncommon Very rare Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT Age at presentation Ethnicity What does boxcarring of blood refer to? adultof the visible blood. Teens-young adultan arteriole, It refers to. Teens-young segmentation column within producing an appearance akin to a series of boxcars in a train East Asia Indian None

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Systemic associations Laterality How common? Multiple BRAOs at non-branch sites and… boxcarring of blood Encephalopathy; +/- epistaxis sensorineural hearing loss There’s another noteworthy DFE finding in Susac syndrome—what is it? Peripheral neovascularization Boxcarring blood Bilateralof (may be sequential) Bilateral Uncommon Very rare Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT Age at presentation Ethnicity What does boxcarring of blood refer to? adultof the visible blood. Teens-young adultan arteriole, It refers to. Teens-young segmentation column within producing an appearance akin to a series of boxcars in a train East Asia Indian None

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Systemic associations Laterality How common? Multiple BRAOs at non-branch sites and… boxcarring of blood Encephalopathy; +/- epistaxis sensorineural hearing loss There’s another noteworthy DFE finding in Susac syndrome—what is it? Peripheral neovascularization Boxcarring blood Bilateralof (may be sequential) Bilateral Uncommon Very rare Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT Age at presentation Ethnicity What does boxcarring of blood refer to? adultof the visible blood. Teens-young adultan arteriole, It refers to. Teens-young segmentation column within producing an appearance akin to a series of boxcars in a train East Asia Indian None

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Systemic associations Laterality How common? Multiple BRAOs at non-branch sites and… boxcarring of blood Encephalopathy; +/- epistaxis sensorineural hearing loss There’s another noteworthy DFE finding in Susac syndrome—what is it? Peripheral neovascularization Boxcarring blood Bilateralof (may be sequential) Bilateral Uncommon Very rare Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT Age at presentation Ethnicity What does boxcarring of blood refer to? adultof the visible blood. Teens-young adultan arteriole, It refers to. Teens-young segmentation column within producing an appearance akin to a series of boxcars in a train East Asia Indian None

Susac syndrome: Boxcarring. FA showing abrupt interruption of two arteries inferiorly (arrows). Note also the characteristic arterial wall hyperfluorescence (asterisk) 29

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Encephalopathy; +/- epistaxis Systemic associations. What sequelae result from the peripheral neo? sensorineural hearing loss Laterality Vitreous hemorrhage and tractional RD Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Encephalopathy; +/- epistaxis Systemic associations. What sequelae result from the peripheral neo? sensorineural hearing loss Laterality Vitreous hemorrhage and tractional RD Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales disease. In the right eye, neovascularization of the disc, vascular sheathing, scattered intraretinal hemorrhages in the periphery and areas of subretinal fibrosis in the macular region are present. In the left eye, multiple areas are visible of preretinal and subhyaloid hemorrhages with neovascularization of disc and sclerosis of retinal vessels extending into the periphery. 32

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations +/- epistaxis ? Encephalopathy; sensorineural? hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Age at presentation Ethnicity rare loss? What is the. Uncommon cause of the encephalopathy and Very hearing The same cause as the arteriolar occlusions—infarctions secondary Teens-young adult to an autoimmune endotheliopathy East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Age at presentation Ethnicity rare loss? What is the. Uncommon cause of the encephalopathy and Very hearing The same cause as the arteriolar occlusions—infarctions secondary Teens-young adult to an autoimmune endotheliopathy East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Peripheral neovascularization Whatfinding is the classic triad of Susac syndrome? Classic retinal producing vitreous 1) ? 2) ? +/- epistaxis Systemic associations 3) ? Multiple BRAOs at non-branch sites Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Peripheral neovascularization Whatfinding is the classic triad of Susac syndrome? Classic retinal producing vitreous 1) Retinal arteriole occlusions 2) Encephalopathy +/- epistaxis Systemic associations 3) Hearing loss 1) Multiple BRAOs at non-branch sites 2) Encephalopathy; sensorineural hearing loss 3) Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality How common? Bilateral (may be does sequential) Bilateral clinically? How the encephalopathy manifest Pts may be disoriented, and/or experience memory loss Uncommon Very rare or even personality changes Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality How common? Bilateral (may be does sequential) Bilateral clinically? How the encephalopathy manifest Pts may be disoriented, and/or experience memory loss Uncommon Very rare or even personality changes; HAs are common (80%) Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may? be sequential) Bilateral ? How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon ? Very? rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young ? adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia ? Indian None ? Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female TB positivity Abnormal CSF studies Key lab finding Note: Neither the Uveitis nor Retina book mentions an TB? for (may be idiopathic) Unknown ethnicity predilection Susac’s, but Eye. Wiki indicates it occurs mainly in white folk. Caveat Punctate emptor. white matter lesions, esp. None Brain imaging finding of the corpus callosum; Cause Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Male Gender Similarly, of the BCSC books mention. TB this ethnic positivity Keyone lab finding predilection; the other doesn’t. Eye. Wiki does. Again, caveat TB? (may be idiopathic) emptor. Cause Female Abnormal CSF studies Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male ? Female ? Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity ? Abnormal CSF ? studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be ? idiopathic) Unknown ? Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum; men Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None ? Punctate white matter lesions, esp. ? callosum; of the corpus Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. two words of the corpus callosum Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT

Susac syndrome: Classic ‘snowball’ lesions of the corpus callosum

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum Treatment Steroids early +/- anti-TB tx; later, ? as needed PRP +/- PPV Systemic: Steroids; IMT ?

Eales Disease Susac Syndrome Also known as… No other names I’m aware of SICRET syndrome Process in a nutshell Occlusive retinal vasculitis Primarily affects venules, or arterioles? Venules Arterioles AC/vitreous cell present? Yes No Classic retinal finding Peripheral neovascularization Multiple BRAOs at non-branch sites Systemic associations none Encephalopathy; sensorineural hearing loss Laterality Bilateral (may be sequential) Bilateral How common? Uncommon Very rare Age at presentation Teens-young adult Ethnicity East Asia Indian None Gender Male Female Key lab finding TB positivity Abnormal CSF studies Cause TB? (may be idiopathic) Unknown Brain imaging finding None Punctate white matter lesions, esp. of the corpus callosum Treatment Steroids early +/- anti-TB tx; later, PRP +/- PPV as needed Systemic: Steroids; IMT