Susac's syndrome: An update

Garcı́a-Carrasco, Mario; Jiménez-Hernández, César; Jiménez-Hernández, Mario; Voorduin-Ramos, S.; Ramos-Álvarez, Gloria; Montiel-Jarquín, Álvaro José; Rojas-Rodriguez, Jorge; Cervera, Ricard

doi:10.1016/j.autrev.2011.04.006

Cited by 27 publications

(20 citation statements)

References 35 publications

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“…SS affects women aged 20–40 years and has been reported in patients aged 7–70 years. [ 3 ] The triad of criteria for SS develops over time, starting with headache in most cases. Psychiatric problems and the other symptoms of the triad take months to develop.…”

Section: Discussionmentioning

confidence: 99%

Susac’s syndrome (retinocochleocerebral vasculopathy): Follow-up of a pediatric patient

et al. 2017

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Susac’s syndrome (SS) is a triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensorineural hearing loss as a result of microvascular occlusions of the brain, retina, and inner ear. It is also a disorder of autoimmune endotheliopathy. SS usually affects young women between the age of 20 and 40 years. SS can be misdiagnosed as multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM) because of similar findings. A 15-year-old girl presented in June 2015 with vomiting and severe headache. Cerebral magnetic resonance imaging revealed multiple lesions in the corpus callosum. Cerebrospinal fluid findings gave normal results. The initial diagnosis was MS and steroid (1000 mg/day) was given. She started to describe hallucinations and became paraplegic. She then underwent plasmapheresis five times without response. Her electroencephalogram was diffusely slow with 2–3 Hz delta rhythm at the frontal regions. Audiological examination showed that she had sensorineural hearing loss in her left ear. Ophthalmologic evaluation revealed BRAO in both eyes. On the basis of these findings, she was diagnosed with SS and treated with intravenous immunoglobulin (IVIG) and aspirin. After monthly treatment with IVIG for 6 months, the patient has almost fully recovered. SS should be kept in mind in the differential diagnosis of MS and ADEM.

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Section: Discussionmentioning

confidence: 99%

Susac’s syndrome (retinocochleocerebral vasculopathy): Follow-up of a pediatric patient

et al. 2017

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“…(3) Initial MRI could be normal in patients with SS. However, repeated scans will probably disclose white matter lesions, not necessarily the typical changes described in the central part of the corpus callosum [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

“…The pathogenesis of SS is unknown; however, recent findings suggest that it is underlain by a distinct autoimmune endotheliopathy syndrome associated with anti-endothelial antibodies [ 1 ]. During the last three decades, more than 300 cases have been published [ 2 ]. Women are more commonly affected than men with a ratio of 3.5/1; the mean age of onset is 31.…”

Section: Introductionmentioning

confidence: 99%

“…Brain MRI may show a distinctive white matter changes that affect the supratentorial white matter particularly the corpus callosum, infratentorial white matter, cortical deep gray, and leptomeningeal involvement. SS requires treatment with immunosuppressants: steroids, cyclophosphamide, and intravenous immunoglobulin, usually in combination [ 2 – 4 ]. We describe a case of SS with several unusual features: cardiac involvement with persistent bradycardia, central vestibular involvement with upbeat nystagmus, and the late appearance of MRI white matter change.…”

Section: Introductionmentioning

confidence: 99%

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Susac’s Syndrome: A Case with Unusual Cardiac Vestibular and Imaging Manifestations

River

Shupak

Tiosano

et al. 2015

Case Reports in Neurological Medicine

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“…Some patients with severe cases may need intravenous immunoglobulins. Antithrombotic measures and antiplatelet agents should be strongly considered (12,13). Although there is agreement that high-dose corticosteroids should be the first line therapy and early, aggressive, sustained immunosuppressive treatment may markedly improve outcomes, it remains unclear as to how much immunosuppressive medication is required and for how long.…”

Section: Case Reportmentioning

confidence: 99%

Headache in a young woman: do not forget Susac’s syndrome

et al. 2017

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SUMMARYSusac syndrome is a rare disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. This underdiagnosed condition needs to be considered in the differential diagnosis of a broad variety of disorders. An early diagnosis is important as treatment can halt disease progression and prevent permanent disability. Herein, we report a case of Susac syndrome in a 31-year-old woman and we highlight how challenging an early diagnosis was and the importance of an aggressive therapeutic approach, including the combination of steroids and other cytotoxic drugs.Key words: Susac's syndrome; Headache; Young woman. Reumatismo, 2017; 69 (3): 122-125 n INTRODUCTION S usac's syndrome (SS), also known as retinocochleocerebral vasculopathy, is a rare condition that was first reported in 1973 (1). Although SS pathophysiology remains mysterious, it is now thought that it is most probably an immune-mediated endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear (2). Although the clinical triad of encephalopathy, branch retinal artery occlusions (BRAO), and sensorineural hearing loss, representing the main feature of SS, is increasingly recognized, SS remains often underdiagnosed particularly at the early stage (3). Herein, we report a case of a 30-year-old woman with SS and we highlight the difficulties of an early diagnosis and adequate treatment. n CASE REPORTA 31-year-old woman was admitted to our department with a history of two months of severe headache, blurry vision and sudden painless vision deterioration in the left eye (LE). A review of the remaining medical history was unremarkable. Particularly, there were no hearing difficulties, no cognitive impairment, no memory loss, no vertigo, no psychiatric disorders and no weakness. The patient denied any infectious exposure or seizure. On physical examination, she was afebrile, and conscious. Blood pressure was normal and all peripheral pulses were palpable and symmetrical. There were no pertinent findings especially from examination of cardiovascular, pulmonary and neurological systems. Ophthalmic examination in the LE revealed BRAO with retinal cloudy swelling at the upper part of the macula. The rest of the ophthalmic examination showed no abnormalities. The right eye (RE) was completely normal with vision of 10/10. Laboratory tests showed no relevant alterations in blood cell count, hemostasis tests, renal and liver function, electrolytes, or lipid panel. There was no biological inflammatory syndrome. Tests for antinuclear antibodies (ANA), anti double stranded DNA (dsDNA), extractable nuclear antigen (ENA) panel, anti-neutrophil cytoplasmic antibodies (ANCA), anti-prothrombin antibodies and antiphospholipid antibodies were negative. Electrocardiogram, and echography of the supra-aortic trunks showed no ab- o n l y vent permanent disability. Herein, we report a case of Susac syndrome in a 31-year-old woman and we highlight how challenging an early diagnosis was and the...

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Susac's syndrome: An update

Cited by 27 publications

References 35 publications

Susac’s syndrome (retinocochleocerebral vasculopathy): Follow-up of a pediatric patient

Susac’s syndrome (retinocochleocerebral vasculopathy): Follow-up of a pediatric patient

Susac’s Syndrome: A Case with Unusual Cardiac Vestibular and Imaging Manifestations

Headache in a young woman: do not forget Susac’s syndrome

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