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• Conditions (26): Genetic and Rare Conditions, Achalasia, Agenesis of Corpus Callosum, and 23 others Genetic and Rare Conditions, Achalasia, Agenesis of Corpus Callosum, Hirschsprung's Disease, Trimethylaminuria, Addison's Disease, Anencephaly, Dercum's Disease, Fragile X Syndrome, Dandy Walker Syndrome, 1p36 Deletion Syndrome, Aarskog Syndrome, Pachygyria, Legg-Calve-Perthes Disease, Behcet's Syndrome, Cri-du-Chat Syndrome, Ledderhose Disease, PANDA Syndrome, Cleidocranial Dysplasia, Empty Sella Syndrome, Darier's Disease, Fructose Intolerance, Kartagener Syndrome, Adie Syndrome, Androgen Insensitivity Syndrome, Menkes Kinky Hair Syndrome [hide]
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• Treatments (2): Hearing Tests, Auscultation Hearing Tests, Auscultation [hide]

About Susac syndrome

Susac's syndrome (Retinocochleocerebral Vasculopathy) is a microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss. It is caused by the immune system attacking healthy tissue, and can lead to mental disorders. Description Susac's Syndrome is named after Dr... more

Susac's syndrome (Retinocochleocerebral Vasculopathy) is a microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss. It is caused by the immune system attacking healthy tissue, and can lead to mental disorders.

Description

Susac's Syndrome is named after Dr John Susac, who first spotted the problem in 1975. Sufferers often experience a personality change and develop bizarre and paranoid behaviour. Their speech can be affected, and many experience unrelenting and intense headaches and migraines, some form of hearing loss, and impaired vision. The problem usually corrects itself, but this can take up to five years. In some cases, subjects can become confused, and believe they are living in a time from their memory once again. According to Michael Hahn, a US Expert on the subject, thinking you live in a foreign country happens in a "fair percentage" of cases.
Only 200 people in the world have Susac's Syndrome.

Treatment

There are a few case reports of the retinopathy due to Susac's disease responding to hyperbaric oxygen treatment with significant improvement of acuity. There is another report of the syndrome recurring after 18 years.(Pub Med reference). Treatment regiments proposed empirically include IVIG, high dose IV steroids. There are characteristic MRI saggital FLAIR appearances described in the radiologic literature.

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