Susac’s syndrome: an update

Sauma, Johanna; Rivera, Daniela; Wu, Andres; Donate-López, J.; Gallego-Pinazo, Roberto; Chilov, Michael; Wu, Max; Wu, Lihteh

doi:10.1136/bjophthalmol-2019-315597

Cited by 17 publications

(18 citation statements)

References 66 publications

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“…Some or all parts of the triad must be present with correlating objective diagnostic data to diagnose SuS. Classically, magnetic resonance (MRI) imaging of the brain reveals lesions within the corpus callosum; however, additional lesions throughout white matter are not uncommon [ 6 ]. Limbic encephalitis and flavivirus-mediated encephalitides commonly affect subcortical structures, including the basal ganglia [ 7 - 8 ].…”

Section: Discussionmentioning

confidence: 99%

Susac Syndrome With Livedo Reticularis: Pathogenesis and Literature Review

Srichawla¹

2022

Cureus

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Susac syndrome (SuS) is a rare autoimmune endotheliopathy that affects the retina, cochlea, and central nervous system (CNS). Even fewer cases of SuS have been reported with dermatological findings, including livedo reticularis and racemosa. The case of SuS reported here presents with encephalopathy, visual disturbances, hearing loss, and a diffuse rash on the abdomen and flank. Magnetic resonance imaging (MRI) of the brain confirmed lesions within the corpus callosum, and an audiogram revealed a unilateral biphasic sensorineural hearing loss in the right ear. A skin biopsy was completed that revealed congested dermal vessels with lymphocytic perivascular infiltrates consistent with livedo reticularis and vasculopathy. Management included intravenous methylprednisolone (IVMP) and a tapering oral dose of prednisone. The patient was also administered 1000 mg of cyclophosphamide with a two-week follow-up for a repeat infusion.Cytotoxic T-lymphocytes (CTLs) and auto-endothelial cell antibodies (AECAs) are hypothesized to play a key role in the pathogenesis of SuS. Livedo reticularis occurs due to congestion of dermal vessels and can be both physiological and pathological in etiology. Pathological etiologies include autoimmune vasculopathies, connective tissue disorders, and drugs (catecholaminergic agents, amantadine, quinidine, etc.). A literature review of SuS cases with associated dermatologic findings is included. Five cases were identified, and neurologic manifestations, dermatologic manifestations, and interventions are described.

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Section: Discussionmentioning

confidence: 99%

Susac Syndrome With Livedo Reticularis: Pathogenesis and Literature Review

Srichawla¹

2022

Cureus

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“…In some patients, the encephalopathy could be severe and visual symptoms may not be referred even though ophthalmologic examination is mandatory if Susac syndrome is suspected, even in asymptomatic patients [19]. The fundus examination may reveal the presence of Gass plaques as yellow reflective lesions due to an autoimmune local reaction in the retinal arterial wall and characteristic retinal whitening in the area of BRAO [20][21][22][23]. However, Gass plaques are characteristic but not pathognomonic of Susac syndrome and could be observed in various retinal disorders such as Eale's disease and retinal lymphoma.…”

Section: Discussionmentioning

confidence: 99%

Multimodal Imaging in Susac Syndrome: A Case Report and Literature Review

Bagaglia

Passani

Oliverio

et al. 2021

IJERPH

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Susac syndrome (SS) is a rare microangiopathy that involves arterioles of the brain, retina, and cochlea. Diagnosis is extremely difficult because of the rarity of the disease and because the signs and symptoms often occur at different times. Multidisciplinary approaches and multimodal images are mandatory for diagnosis and prompt therapy. In this report, we describe a case of SS and the application of multimodal retinal imaging to evaluate the ophthalmologic changes and to confirm diagnosis. Early diagnosis and therapy based on the associations of steroids and immunosuppressants are necessary to limit the sequelae of the disease.

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“…In these cases that present ocular involvement first, the ophthalmologist's role is crucial and acts as a whistle blower, by recognising and treating the patients and so avoiding severe cochlear and cerebral complications. Rapid identification of the disease by the ophthalmologist is crucial, as in ophthalmological series, unlike in neurological series, MRI is often negative and ophthalmic manifestations may be the sole presenting sign [29][30][31][32]. This is one more reason to have good ophthalmological disease defining criteria.…”

Section: Discussion-conclusionmentioning

confidence: 99%

Susac syndrome (Retino-cochleo-cerebral vasculitis), the ophthalmologist in the role of the whistleblower

Papasavvas¹,

Teuchner

Herbort³

2020

J Ophthal Inflamm Infect

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Background/purpose Susac syndrome is a rare microangiopathy of suspected autoimmune origin affecting arteries of the retina, the cochlea and the brain. The aim of the study was to give a review of the disease entity and determine the proportion of cases and their characteristics in a uveitis referral centre. Patients and methods Charts of patients with the diagnosis of Susac syndrome seen in the Uveitis Clinic of the Centre for Ophthalmic Specialised Care (COS), Lausanne, Switzerland were reviewed retrospectively to determine the frequency of such cases in a uveitis referral centre. Clinical symptoms and signs, functional data, imaging signs and evolution were analysed in the 3 COS cases and one case shared with the Uveitis Clinic of the Department of Ophthalmology, University of Innsbruck, Austria. Characteristic signs were searched possibly allowing a prompt diagnosis. Results During the period from 1994 to 2019 (24 years, 2045 patients), 3 charts with the diagnosis of Susac syndrome were found (0.15%). The whole collective, including the additional case, comprised three women aged 28, 32 and 63 at presentation and one man, aged 42. None of the 3 cases that were referred were diagnosed beforehand. The characteristic item found in all 4 cases was the abrupt arterial stop or segmental interruption of arteries and increased staining of arterial wall on angiography more clearly shown on indocyanine green angiography that can potentially be proposed as a crucial diagnostic element. All 4 cases responded to dual steroidal and non-steroidal immunosuppression. Under treatment, all four patients did not show any further evolution. Conclusion Susac syndrome is a multilocation arteritis of the head that can involve the eye, ear and brain often first diagnosed by the ophthalmologist. The diagnosis is rapidly reached in uveitis referral centres but seems to be missed otherwise, A helpful angiographic sign to be searched is an abrupt or segmental arterial stop and increased staining of the arterial wall more clearly seen on indocyanine green angiography. Patients often present first to the ophthalmologist who should be acting as a whistleblower to avoid severe involvement of the brain.

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Susac’s syndrome: an update

Cited by 17 publications

References 66 publications

Susac Syndrome With Livedo Reticularis: Pathogenesis and Literature Review

Susac Syndrome With Livedo Reticularis: Pathogenesis and Literature Review

Multimodal Imaging in Susac Syndrome: A Case Report and Literature Review

Susac syndrome (Retino-cochleo-cerebral vasculitis), the ophthalmologist in the role of the whistleblower

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