Susac's syndrome: the first case report in a Nordic country, with an 8‐year follow‐up

Gruhn, Nicolai; Pedersen, Lene; Nielsen, Niels Vesti

doi:10.1111/j.1600-0420.2005.00558.x

Cited by 7 publications

(1 citation statement)

References 4 publications

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“…Cyclosporin A (CSA) and tacrolimus (TAC) are used in NMOSD, MG and SLE, and sometimes in SuS. 51,61,[70][71][72] TAC and CSA should not be started, but can be continued relatively safe in pregnancy. However, strict drug level monitoring is required to limit toxicities.…”

Section: Intravenous Immunoglobulinsmentioning

confidence: 99%

Susac syndrome and pregnancy: a review of published cases and considerations for patient management

Willekens

Kleffner

2021

Ther Adv Neurol Disord

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Susac syndrome (SuS) is a rare autoimmune endotheliopathy leading to hearing loss, branch retinal artery occlusions and encephalopathy. Young females are more frequently affected than males, making counselling for family planning an important issue. We reviewed published cases on SuS during pregnancy or in the postpartum period, and selected 27 reports describing the details of 33 patients with SuS. Treatment options and implications for pregnancy and breastfeeding are discussed. We propose new areas for research and suggest a management strategy.

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Section: Intravenous Immunoglobulinsmentioning

confidence: 99%

Susac syndrome and pregnancy: a review of published cases and considerations for patient management

Willekens

Kleffner

2021

Ther Adv Neurol Disord

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Vasculopathy and Vasculitis

Bathla

Moritani

Kirby

et al. 2021

Diffusion-Weighted MR Imaging of the Brain, Head and Neck, and Spine

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Treatment of Susac’s syndrome

Rennebohm

Egan²,

Susac³

2008

Curr Treat Options Neurol

131

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Susac's syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusions (BRAO), and hearing loss. It usually affects women aged 20 to 40, but men are also affected, and the age range extends from 9 to 72 years. It tends to be unrecognized, even in major academic centers. The complete triad may not be present at the onset, which makes diagnosis more difficult. However, since this disorder is treatable, early diagnosis is important. The encephalopathy is usually associated with headaches, multifocal neurologic manifestations, and psychiatric features (particularly paranoia). MRI shows a white matter disturbance that is frequently confused with multiple sclerosis and acute disseminated encephalomyelitis. During the encephalopathy, the corpus callosum is always affected and shows central involvement--small to large "snowballs" and linear defects, "spokes." As the acute changes (microinfarcts) resolve, central callosal "holes" develop, a pathognomonic finding. The deep gray matter (70%) and leptomeninges (33%) also may be involved. Dilated fundus examination will reveal branch retinal artery occlusions. Fluorescein angiography may disclose pathognomonic staining of the arterioles proximal to the occlusions and of nonoccluded arterioles. The cochlear hearing loss, sometimes associated with vertigo, is usually bilateral, and deafness becomes a major disabling problem. Brain biopsies, anatomic observations, and responses to immunosuppressive therapy suggest that SS represents an autoimmune endotheliopathy in the microvasculature of the brain, retina, and cochlea. Treatment requires immunosuppression. High-dose corticosteroid therapy is the mainstay, but additional therapies such as intravenous immunoglobulin, mycophenolate mofetil, and cyclophosphamide are often necessary. Rituximab is the newest therapy to consider. Treatment should be prompt, aggressive, and sustained to avoid the dreaded residuals of dementia, deafness, and blindness.

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Susac's syndrome: the first case report in a Nordic country, with an 8‐year follow‐up

Cited by 7 publications

References 4 publications

Susac syndrome and pregnancy: a review of published cases and considerations for patient management

Susac syndrome and pregnancy: a review of published cases and considerations for patient management

Vasculopathy and Vasculitis

Treatment of Susac’s syndrome

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