Susac's syndrome

Susac syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusions (BRAO), and hearing loss. The encephalopathy manifests with headache, confusion, memory loss, behavioral changes, dysarthria, and occasional mutism. The BRAO may be extensive or subtle; if the posterior pole of the retina is involved, patients may complain
of impaired vision. These BRAO are usually bilateral and may be the presenting features of the illness, or occur later in the clinical The MR scans in SS show a rather distinctive pattern of supratentorial white matter lesions that always involve the corpus callosum. There is often deep gray matter, posterior fossa involvement, and frequent parenchymal with occasional leptomeningeal enhancement. The central callosal lesions in Susac’s differ from multiple sclerosis as the ependymal undersurface of the corpus callosum is usually involved and callosal atrophy is usually seen in demyelinating disaese. Central involvement and an appropriate clinical picture should support the diagnosis of SS in patients with at least two of the three features of the clinical triad. NEUROLOGY 2003;61:1783–1787

See Abid's Susac DWI for another nice view