Idiopathic infratentorial superficial siderosis of the central nervous system

The superficial siderosis (SS) of the central nervous system (CNS) is a rare condition characterized by a wide range of neurological manifestations directly linked to acquired iron-mediated neurodegeneration. First described more than 100 years ago, only recently SS
has been divided into two distinct entities, according to the distribution of iron deposition in
the CNS: cortical superficial siderosis (cSS) and infratentorial superficial siderosis (iSS). Here
we describe an adult case of iSS, with detailed clinical and radiological features. Moreover,
we extensively review the literature of SS, particularly focusing on the pathogenesis,
clinical-radiological classification, diagnostic algorithm and treatment options of this rare
condition.