Susac’s Syndrome

Below you will find more information about Susac’s Syndrome from Medigest. If you believe that you are suffering from any of the symptoms of Susac’s Syndrome it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Susac’s Syndrome and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Susac’s Syndrome comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.

Definition

Susac's syndrome (Retinocochleocerebral Vasculopathy) is a microangiopathy marked by encephalopathy, branch retinal artery occlusions and hearing loss.

Diagnosis

In a recent analysis conducted in 2003, MRI images from 27 patients fulfilling the diagnostic criteria of Susac's syndrome were reviewed. Multifocal supratentorial lesions were found in all patients. Most lesions were small, though some were larger than 7 mm. All 27 patients possessed corpus callosum lesions. These all possessed a punched-out appearance on follow up MRI. Though most commonly involved white matter, many patients also had lesions in deep grey matter structures, as well as leptomeningeal enhancement.

Treatment

There are a few case reports of the retinopathy due to Susac's disease responding to hyperbaric oxygen treatment, with dramatic improvement of acuity. Treatment regiments that have been proposed empirically include IVIG, high dose IV steroids. There are certain MRI sagittal FLAIR appearances described in the radiologic literature.

Symptoms and Signs

Some symptoms include sensoneural deafness, brain disease, retinal blood vessel damage, headache, cognitive problems, ataxia, and psychiatric disorders.

Causes

It is caused by the immune system attacking healthy tissue, and can result to mental disorders.

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