Superficial siderosis of the CNS is a rare condition, caused by deposition of haemosiderin in the superficial layers of the CNS due to repeated chronic subara chnoid or intraventricular haemorrhage. Typically, the hin...Superficial siderosis of the CNS is a rare condition, caused by deposition of haemosiderin in the superficial layers of the CNS due to repeated chronic subara chnoid or intraventricular haemorrhage. Typically, the hindbrain structures, esp ecially the cerebellum, are most affected. There is a surprising lack of studies investigating in detail the behavioural functioning of patients with such a con dition. In this study, we document for the first time the cognitive, social and emotional processing of six patients with a confirmed clinical diagnosis of supe rficial siderosis. They were aged between 40 and 62 years, with a mean age of 50 .2 years; four were male. We administered a comprehensive battery of general cog nitive ability and social cognitive tasks. A review of MRI was also undertaken. The findings indicate selective cognitive impairments affecting speech productio n, visual recall memory and executive functions. In addition, a selective patter n of social dysfunction, affecting the ability to represent other people’s ment al states, was found. These behavioural dysfunctions are reported in the context of MRI-documented lesions maximally involving the cerebellum, in particular th e superior vermis, as well as the medial and inferior frontal cortex. These resu lts suggest that superficial siderosis is associated with a distinct pattern of cognitive and social impairments. They are consistent with the recently proposed role of the cerebellum as a modulator of cognitive, social and emotional functi ons.展开更多
文摘Superficial siderosis of the CNS is a rare condition, caused by deposition of haemosiderin in the superficial layers of the CNS due to repeated chronic subara chnoid or intraventricular haemorrhage. Typically, the hindbrain structures, esp ecially the cerebellum, are most affected. There is a surprising lack of studies investigating in detail the behavioural functioning of patients with such a con dition. In this study, we document for the first time the cognitive, social and emotional processing of six patients with a confirmed clinical diagnosis of supe rficial siderosis. They were aged between 40 and 62 years, with a mean age of 50 .2 years; four were male. We administered a comprehensive battery of general cog nitive ability and social cognitive tasks. A review of MRI was also undertaken. The findings indicate selective cognitive impairments affecting speech productio n, visual recall memory and executive functions. In addition, a selective patter n of social dysfunction, affecting the ability to represent other people’s ment al states, was found. These behavioural dysfunctions are reported in the context of MRI-documented lesions maximally involving the cerebellum, in particular th e superior vermis, as well as the medial and inferior frontal cortex. These resu lts suggest that superficial siderosis is associated with a distinct pattern of cognitive and social impairments. They are consistent with the recently proposed role of the cerebellum as a modulator of cognitive, social and emotional functi ons.
