Diagnosis and classification of Susac syndrome

Abstract

Susac syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and neuro-sensorial hearing loss; it is due to a microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear. SS is characterized by typical radiological features on magnetic resonance imaging (MRI) which, together with clinical symptoms, may permit a diagnosis. Branch retinal artery occlusions (BRAOs) are best evaluated using fluorescein angiography (FA) which may show the typical multifocal fluorescence. SS is an autoimmune endotheliopathy that requires treatment with immunosuppressive agents: steroids, azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide and intravenous immunoglobulin, usually in combination. Plasma exchange is also useful. In addition, antiplatelet agents may be a useful adjunct. Correct immunosuppressive therapy results in significant clinical and radiological improvement. An early diagnosis and treatment are important to delay the disease progression and prevent permanent disability.

Increased Incidence Of Susac Syndrome: A Case Series Study

“…3. Punctuate DWI hyperintense lesions with corresponding ADC hypointensity (restricted diffusion) were associated with disease activity in all of our patients ( Figure 1A) [9]. 4.…”

Increased Incidence Of Susac Syndrome: A Case Series Study

“…3. Punctuate DWI hyperintense lesions with corresponding ADC hypointensity (restricted diffusion) were associated with disease activity in all of our patients ( Figure 1A) [9]. 4.…”

Increased incidence of Susac syndrome: a case series study

“…Punctuate DWI hyperintense lesions with corresponding ADC hypointensity (restricted diffusion) were associated with disease activity in all of our patients (Fig. 1 a) [ 9 ]. 4.…”

“…Audiometry showed low-frequency sensorineural hearing loss in five cases (Fig. 1 c) [ 1 , 6 , 9 , 18 , 26 ], three were bilateral and two were unilateral. Of note, two patients had SNHL on audiometry with no auditory symptoms.…”

A panel of clinical and neuropathological features of cerebrovascular disease through the novel neuroimaging methods

“…Regarding the study of vascular-related pathology, a major development is underway, particularly with the advent of 7 T MRI; previous attempts have been made to distinguish between vascular occlusion and microinfarction versus demyelinating disease, for instance in the differential diagnosis at 7 T between Susac syndrome and Multiple Sclerosis. 56 , 57 Accordingly, the anatomic modifications involving dilated perivascular spaces have been investigated and quantified with greater accuracy using 7 T MRI, 58 showing different patterns and quantification, for instance, in stroke, migraine, CADASIL, dementia, AD, and mild cognitive impairment. Furthermore, 7 T studies may be more successful in providing a more detailed picture of the neuropathology of closely related conditions, such as the case of cerebral microinfarcts, whose regional distribution (intracortical and juxtacortical location) 59 may indicate chronic or acute lesions, with the latter being described as gliotic cerebral microinfarcts with hemorrhagic components.…”

Increased Incidence Of Susac Syndrome: A Case Series Study

“…3. Punctuate DWI hyperintense lesions with corresponding ADC hypointensity (restricted diffusion) were associated with disease activity in all of our patients ( Figure 1A) [9]. 4.…”

Increased incidence of Susac syndrome: a case series study

“…Punctuate DWI hyperintense lesions with corresponding ADC hypointensity (restricted diffusion) were associated with disease activity in all of our patients (Fig. 1 a) [ 9 ]. 4.…”

“…Audiometry showed low-frequency sensorineural hearing loss in five cases (Fig. 1 c) [ 1 , 6 , 9 , 18 , 26 ], three were bilateral and two were unilateral. Of note, two patients had SNHL on audiometry with no auditory symptoms.…”

A panel of clinical and neuropathological features of cerebrovascular disease through the novel neuroimaging methods

“…Regarding the study of vascular-related pathology, a major development is underway, particularly with the advent of 7 T MRI; previous attempts have been made to distinguish between vascular occlusion and microinfarction versus demyelinating disease, for instance in the differential diagnosis at 7 T between Susac syndrome and Multiple Sclerosis. 56 , 57 Accordingly, the anatomic modifications involving dilated perivascular spaces have been investigated and quantified with greater accuracy using 7 T MRI, 58 showing different patterns and quantification, for instance, in stroke, migraine, CADASIL, dementia, AD, and mild cognitive impairment. Furthermore, 7 T studies may be more successful in providing a more detailed picture of the neuropathology of closely related conditions, such as the case of cerebral microinfarcts, whose regional distribution (intracortical and juxtacortical location) 59 may indicate chronic or acute lesions, with the latter being described as gliotic cerebral microinfarcts with hemorrhagic components.…”

Increased Incidence Of Susac Syndrome: A Case Series Study

“…3. Punctuate DWI hyperintense lesions with corresponding ADC hypointensity (restricted diffusion) were associated with disease activity in all of our patients ( Figure 1A) [9]. 4.…”

Increased incidence of Susac syndrome: a case series study

“…Punctuate DWI hyperintense lesions with corresponding ADC hypointensity (restricted diffusion) were associated with disease activity in all of our patients (Fig. 1 a) [ 9 ]. 4.…”

“…Audiometry showed low-frequency sensorineural hearing loss in five cases (Fig. 1 c) [ 1 , 6 , 9 , 18 , 26 ], three were bilateral and two were unilateral. Of note, two patients had SNHL on audiometry with no auditory symptoms.…”

A panel of clinical and neuropathological features of cerebrovascular disease through the novel neuroimaging methods

“…Regarding the study of vascular-related pathology, a major development is underway, particularly with the advent of 7 T MRI; previous attempts have been made to distinguish between vascular occlusion and microinfarction versus demyelinating disease, for instance in the differential diagnosis at 7 T between Susac syndrome and Multiple Sclerosis. 56 , 57 Accordingly, the anatomic modifications involving dilated perivascular spaces have been investigated and quantified with greater accuracy using 7 T MRI, 58 showing different patterns and quantification, for instance, in stroke, migraine, CADASIL, dementia, AD, and mild cognitive impairment. Furthermore, 7 T studies may be more successful in providing a more detailed picture of the neuropathology of closely related conditions, such as the case of cerebral microinfarcts, whose regional distribution (intracortical and juxtacortical location) 59 may indicate chronic or acute lesions, with the latter being described as gliotic cerebral microinfarcts with hemorrhagic components.…”