Three young women (aged 18 years, 19 years and 19 years) who developed progressive neuropsychic and neurologic disturbances with hearing loss and multifocal retinal artery branch occlusions are reported. This retinocochleocerebral syndrome has been reported previously only in 12 young North American women. Its pathogenesis is unknown, but an atypical viral infection of the vessel walls has been...

A 50 year old woman presented with a subacute onset of vertigo and diplopia followed by an encephalopathy with confusion, spasticity, ataxia, myoclonus, and multiple branch retinal arteriolar occlusions and unilateral sensorineural deafness. Brain biopsy confirmed multiple microinfarcts with no vasculitis. After the procedure she had a right iliofemoral deep vein thrombosis and was found to be ...

We describe a patient with Susac's syndrome presenting with the triad of encephalopathy, branch retinal artery occlusion and hearing loss. MRI has some characteristic features and can be helpful in establishing the diagnosis, particularly in an early stage before the classic triad is complete. Besides MRI often allows to differentiate this rare disorder from more common neurologic diseases like...

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 di...

<b>Hintergrund:</b> Das Susac-Syndrom (SS) ist gekennzeichnet durch die Trias aus Enzephalopathie, Arterienastverschluss der Netzhaut und Innenohrschwerhörigkeit. Die Diagnose von SS jedoch nach wie vor schwierig, da klinische nur selten zu Beginn Erkrankung auftritt Schwere Symptome variiert. SS-Symptome deuten oft auf andere Krankheiten hin, insbesondere Multiple Sklerose (MS), hä...