Brain histopathology in three cases of Susac's syndrome: implications for lesion pathogenesis and treatment: Figure 1

Hardy, Todd A.; O’Brien, Billy; Gerbis, Natasha; Barnett, Michael; Reddel, Stephen W.; Brewer, Janice; Herkes, Geoffrey; Silberstein, Paul; Garsia, Roger; Watson, J. D. G.; Gupta, Ruta; Parratt, John; Buckland, Michael E.

doi:10.1136/jnnp-2014-308240

Cited by 57 publications

(41 citation statements)

References 7 publications

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“…Evidence that the pathological pathway of Susac Syndrome maybe associated with complement pathway was further disputed by brain biopsy series performed by Hardy and coworkers that did not show evidence to support complement deposition. 29 In this study, only one out of three brain biopsies showed C4d deposition in microvasculature, but similar immunoreactivity was present in the control samples.…”

Section: Accepted Manuscriptsupporting

confidence: 53%

A review and update on the ophthalmic implications of Susac syndrome

Heng

Bailey

Lee

et al. 2019

Survey of Ophthalmology

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Susac syndrome is a rare condition presumed to be immune-mediated occlusion of small arterial vasculature principally of the brain, inner ear, and retina. Clinically the syndrome manifests as a pathognomonic triad of encephalopathy, hearing loss, and branch retinal artery occlusion. Early recognition and diagnosis is important as delayed treatment may be profound and result in deafness, blindness, dementia and other neurological deficits. The plethora of imaging technology, including magnetic resonance imaging, retinal fluorescein angiography, optical coherence tomography (OCT) and OCT-angiography allows deeper and more discrete anatomical-physiological correlation of underlying pathology, early diagnosis, and imaging biomarkers for early detection of relapse during follow up. We highlight the current clinical classification of Susac syndrome, available investigations, treatment and care pathways.

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Section: Accepted Manuscriptsupporting

confidence: 53%

A review and update on the ophthalmic implications of Susac syndrome

Heng

Bailey

Lee

et al. 2019

Survey of Ophthalmology

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“…Findings from brain, muscle and skin biopsies1 13 14 are vastly unspecific, differ widely and may rather be helpful to exclude other diseases. In our opinion, the diagnosis of SuS can be established with less-invasive techniques, and biopsy findings need not necessarily be supportive, thus leading to false-negative results.…”

Section: Resultsmentioning

confidence: 99%

Diagnostic criteria for Susac syndrome

Kleffner

Dörr

Ringelstein

et al. 2016

J Neurol Neurosurg Psychiatry

207

151

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“…Sagittal T1 image (D) demonstrating a residual centrally-located ‘black hole’ in the splenium of the corpus callosum (thin black arrow). (A) is reproduced from Hardy et al ,55 with permission.…”

Section: Non-demyelinating Neuroinflammatory Diseasesmentioning

confidence: 99%

“…Susac's syndrome is considered to have an autoimmune basis with histopathological studies showing scant periarteriolar inflammatory T-cell predominant infiltrates within a thickened vessel wall without evidence of true vasculitis 55. Antiendothelial cell antibodies have been demonstrated in immunohistochemical studies, but it is not known whether these are pathogenic or an epiphenomenon, as similar antibodies have been also demonstrated in other autoimmune conditions 56 57…”

Section: Non-demyelinating Neuroinflammatory Diseasesmentioning

confidence: 99%

The corpus callosum in the diagnosis of multiple sclerosis and other CNS demyelinating and inflammatory diseases

Garg

Miller

et al. 2015

J Neurol Neurosurg Psychiatry

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Lesions in the corpus callosum (CC) are important radiological clues to the diagnosis of multiple sclerosis (MS), but may also occur in other neuroinflammatory and non-neuroinflammatory conditions. In this article, we discuss the radiological features of lesions within the CC in MS and other central nervous system inflammatory and acquired demyelinating diseases. An understanding of the appearance and location of lesions in the CC is important not only for accurate diagnosis and treatment of these various conditions, but as it also provides insights into pathogenesis.

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Brain histopathology in three cases of Susac's syndrome: implications for lesion pathogenesis and treatment: Figure 1

Cited by 57 publications

References 7 publications

A review and update on the ophthalmic implications of Susac syndrome

A review and update on the ophthalmic implications of Susac syndrome

Diagnostic criteria for Susac syndrome

The corpus callosum in the diagnosis of multiple sclerosis and other CNS demyelinating and inflammatory diseases

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