"The primary pathology is likely to be an autoimmune endothelialopathy (microangiopathy) affecting pre-capillary arterioles, with subsequent embolisation, of the brain, retina, and inner ear 1,3,9,19,20. Hence, microinfarcts associated with endothelial proliferation and hypertrophy, basement membrane thickening, and thickening of precapillary arteriolar adventitia with loss of internal elastic lamina 16. Demyelination is not a pathological hallmark of Susac syndrome, in contrast with multiple sclerosis 16."
"MRI "
"The disorder was initially described by John O Susac (1940-2012), an American neurologist and neuro-ophthalmologist, and his colleagues in their 1979 seminal paper on two patients with the classic clinical triad 3. The eponym was famously first used in 1986, when a patient was presented in a neuro-ophthalmology conference with the same triad that Susac et al. had described, prompting one member of the conference to exclaim “this is Susac’s syndrome” 15."
"Susac syndrome, also known as retinocochleocerebral vasculopathy or SICRET syndrome (small infarctions of cochlear, retinal and encephalic tissue), is a rare syndrome typically affecting young to middle-aged women that is clinically characterised by the triad of acute or subacute encephalopathy, bilateral sensorineural hearing loss, and branch retinal arterial occlusions."
"acute or subacute encephalopathy which can manifest in a broad spectrum of symptoms, e.g. memory impairment, confusion, behavioural disturbances, ataxia, dysarthria, paranoid psychosis, and headaches 1-8"
Expected headings
"MRI "
"Susac syndrome, also known as retinocochleocerebral vasculopathy or SICRET syndrome (small infarctions of cochlear, retinal and encephalic tissue), is a rare syndrome typically affecting young to middle-aged women that is clinically characterised by the triad of acute or subacute encephalopathy, bilateral sensorineural hearing loss, and branch retinal arterial occlusions."
"headache (within 6 months of diagnosis; migrainous or oppressive in character)"
"A definite diagnosis of Susac syndrome requires fulfilment of all three criteria and their subcriteria, in other words, 1-i, 1-ii, 2-ii, 3-i, and 3-ii."
"acute or subacute encephalopathy which can manifest in a broad spectrum of symptoms, e.g. memory impairment, confusion, behavioural disturbances, ataxia, dysarthria, paranoid psychosis, and headaches 1-8"
"The primary pathology is likely to be an autoimmune endothelialopathy (microangiopathy) affecting pre-capillary arterioles, with subsequent embolisation, of the brain, retina, and inner ear 1,3,9,19,20. Hence, microinfarcts associated with endothelial proliferation and hypertrophy, basement membrane thickening, and thickening of precapillary arteriolar adventitia with loss of internal elastic lamina 16. Demyelination is not a pathological hallmark of Susac syndrome, in contrast with multiple sclerosis 16."