Syndrome de Susac et atteinte ophtalmologique chez l’enfant

Saliba, Marcos Tadeu Adas; Pelosse, B; Momtchilova, M.; Laroche, L.

doi:10.1016/s0181-5512(07)79280-x

Cited by 18 publications

(1 citation statement)

References 13 publications

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“…[34] Saliba M et al , reporting a 14-year-old girl with diagnosis of Susac's syndrome state that it usually affects young women in young adulthood and is extremely rare during childhood. [8] The important differentials are multiple sclerosis, aseptic meningitis, systemic lupus erythematosus, Behcet's and complicated migraine, sarcoidosis, tuberculosis, syphilis and lymphomas. A most recent study says that, the commonest cause of retinal arterial occlusion in young adults of Indian origin was found to be hyperhomocysteinemia.…”

Section: Discussionmentioning

confidence: 99%

Susac′s syndrome: First from India and youngest in the world

Prakash

Jain

Gupta

et al. 2013

Indian J Ophthalmol

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A two and half year old female was admitted at the emergency room suffering from gradually worsening headache followed by nausea. The child presented with reduced level of consciousness and bilateral hypoacusis. The patient was lethargic. Ophthalmic examination showed branch retinal artery occlusion (BRAO). This finding was crucial to the diagnosis of Susac's syndrome (SS), a rare autoimmune disease characterized by, endotheliopathy of retina, encephalic tissues and cochlea. Magnetic resonance imaging of the brain also showed typical features. Thorough blood investigations did not reveal any other abnormality. Patient was treated with immunosuppressive to prevent her from developing severe sequelae of this disease. The child showed dramatic improvement in her systemic condition within 48 h of starting the treatment. This is the youngest ever and first case report from India.

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Section: Discussionmentioning

confidence: 99%

Susac′s syndrome: First from India and youngest in the world

Prakash

Jain

Gupta

et al. 2013

Indian J Ophthalmol

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Susac-Syndrom

et al. 2011

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Susac syndrome, named after John Susac, the first to describe this condition, is characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Although certainly a rare disease, Susac syndrome needs to be considered in the differential diagnosis of a broad variety of diseases. The pathogenesis is not yet clear. Autoimmune processes leading to damage and inflammation-related occlusion of the microvessels in brain, retina, and inner ear are thought to play a causal role. The diagnosis is based primarily on the clinical presentation, the documentation of branch retinal artery occlusion by fluorescence angiography, and characteristic findings on cerebral MRI. Usually, immunosuppressive therapy is required, though controlled therapy trials are missing so far. The intention of this review article is to raise awareness of this disease among neurologists, psychiatrists, ophthalmologists, and ENT specialists as a high number of unreported cases probably exists. Accordingly, the focus is on the clinical presentation and the diagnostic approach.

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Cyclophosphamide and methotrexate in Susac’s Syndrome: a successful sequential therapy in a case with involvement of the cerebellum

Catarsi

Pelliccia²,

Pizzanelli

et al. 2014

Clin Rheumatol

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Susac's Syndrome (SS) is a rare disease with unknown aetiology due to a microangiopathy affecting the precapillary arterioles of the brain, retina, cochlea and semicircular canals. Neurological manifestations, visual dysfunction and hearing loss represent the classical clinical triad of SS. Diagnosis is confirmed by laboratory investigations, neuroimaging findings, fluoroangiography and inner-ear studies. An early treatment with steroids and immunosuppressors limits the sequelae of disease. We report a case of SS in which the clinical triad occurred in a very short period of time. Brain MRI showed the involvement of cerebellum, this representing a rare neuroradiological finding in SS. A full remission of disease was obtained by using corticosteroids and cyclophosphamide in the acute-subacute phase and methotrexate as maintenance therapy. This latter has never been used before in SS.

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Syndrome de Susac et atteinte ophtalmologique chez l’enfant

Cited by 18 publications

References 13 publications

Susac′s syndrome: First from India and youngest in the world

Susac′s syndrome: First from India and youngest in the world

Susac-Syndrom

Cyclophosphamide and methotrexate in Susac’s Syndrome: a successful sequential therapy in a case with involvement of the cerebellum

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