Gass plaques and fluorescein leakage in Susac Syndrome

Egan, Robert A.; Hills, William L.; Susac, John O.

doi:10.1016/j.jns.2010.08.043

Cited by 75 publications

(43 citation statements)

References 11 publications

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“…We agree that fluorescein angiography is important not only to show arteriolar occlusions but also to show segmental arteriolar staining (8) , as observed in our current case (Figure 1 F). This finding, supports our initial suggestion that SS was an autoimmune disease against some unknown antigen present in the endothelium which has a blood-tissue barrier mechanism, as observed in the retina, cochlea and brain (3,4) .…”

Section: Reply: Fibrin-like Materials In the Retinal Arterioles And Flsupporting

confidence: 91%

Reply: Fibrin-like material in the retinal arterioles and fluorescein angiography findings in Susac syndrome

Monteiro¹

2013

Arq. Bras. Oftalmol.

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Section: Reply: Fibrin-like Materials In the Retinal Arterioles And Flsupporting

confidence: 91%

Reply: Fibrin-like material in the retinal arterioles and fluorescein angiography findings in Susac syndrome

Monteiro¹

2013

Arq. Bras. Oftalmol.

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“…Ophthalmological involvement includes many types of disturbances such as photopsia, black spots, scintillating scotomas and even severe visual loss, which was reported in our patient (9). These symptoms are often caused by BRAO (6).…”

Section: Case Reportsupporting

confidence: 65%

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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“…To meet the ‘retinal involvement criterion’, at least one of the following findings should be documented in the ophthalmological examination: (1) at least one acute BRAO in FLA or funduscopy, (2) focal segmental staining of the arterial wall (arterial wall hyperfluorescence, AWH) near the site of obstruction (figure 2) in FLA,10 or (3) sectorial damage of the inner retinal layers from the retinal nerve fibre layer through to the outer plexiform layer in optical coherence tomography (OCT) 11. AWH in patients with SuS indicates the impaired integrity of the retinal vessel wall, has to be differentiated from fluorescein leakage, can be considered as a marker for acute disease activity and has not been demonstrated in other retinal vasculitides 10.…”

Section: Resultsmentioning

confidence: 99%

“…AWH in patients with SuS indicates the impaired integrity of the retinal vessel wall, has to be differentiated from fluorescein leakage, can be considered as a marker for acute disease activity and has not been demonstrated in other retinal vasculitides 10. Visual field deficits or scintillating scotoma may support the diagnosis and help to initiate the necessary diagnostic procedures, but are not sufficient to fulfil the retinal involvement criterion.…”

Section: Resultsmentioning

confidence: 99%