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Cortical Superficial Siderosis of the Central Nervous System – An Overview

Cortical superficial siderosis (cSS) is observed on gradient echo T2*-weighted magnetic resonance imaging (MRI) as a hypo-intensity outlining the convexity of the cerebral hemispheres. It is composed of haemosiderin deposits in the subpial space. It was initially thought to be a rare condition but with 7.0 Tesla (T) MRI it appears to be not uncommon. Its symptomatology depends on the underlying cortical pathology. Diffuse cSS is mainly related to the sequels of an underlying lobar haematoma, mainly due to cerebral amyloid angiopathy (CAA). Focal cSS is more due to cortical microinfarcts, that underwent haemorrhagic transformation, rather than due to cortical microbleeds. cSS is not only found in patients suffering from CAA but also in those suffering from several neurodegenerative dementia syndromes and after cerebral trauma.