Susac's Syndrome
Susac's Syndrome Causes
It is caused by the immune system attacking healthy tissue, and can result to mental disorders.
Susac's Syndrome Definition
Susac's syndrome (Retinocochleocerebral Vasculopathy) is a microangiopathy marked by encephalopathy, branch retinal artery occlusions and hearing loss.
Susac's Syndrome 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.
Susac's Syndrome Symptoms and Signs
Some symptoms include sensoneural deafness, brain disease, retinal blood vessel damage, headache, cognitive problems, ataxia, and psychiatric disorders.
Susac's Syndrome 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.