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Brain Advance Access published online on March 23, 2005
Brain, doi:10.1093/brain/awh487
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© The Author (2005). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org
Received September 6, 2004
Revised January 10, 2005
Accepted January 18, 2005

Article

Cognitive and social impairments in patients with superficial siderosis

N. J. van Harskamp 1, P. Rudge 1, and L. Cipolotti 1*

1 Department of Neuropsychology, National Hospital for Neurology and Neurosurgery, London, UK

* To whom correspondence should be addressed.
L. Cipolotti, E-mail: l.cipolotti{at}ion.ucl.ac.uk


  Abstract

Summary 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 subarachnoid or intraventricular haemorrhage. Typically, the hindbrain structures, especially the cerebellum, are most affected. There is a surprising lack of studies investigating in detail the behavioural functioning of patients with such a condition. In this study, we document for the first time the cognitive, social and emotional processing of six patients with a confirmed clinical diagnosis of superficial 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 cognitive ability and social cognitive tasks. A review of MRI was also undertaken. The findings indicate selective cognitive impairments affecting speech production, visual recall memory and executive functions. In addition, a selective pattern of social dysfunction, affecting the ability to represent other people's mental states, was found. These behavioural dysfunctions are reported in the context of MRI-documented lesions maximally involving the cerebellum, in particular the superior vermis, as well as the medial and inferior frontal cortex. These results 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 functions.

Keywords: superficial siderosis; theory of mind; cerebellum; cognitive functioning; social cognition.
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