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The Journals of Gerontology Series A: Biological Sciences and Medical Sciences Advance Access published online on February 4, 2009
The Journals of Gerontology Series A: Biological Sciences and Medical Sciences, doi:10.1093/gerona/gln028
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© The Author 2009. Published by Oxford University Press on behalf of The Gerontological Society of America. All rights reserved. For permissions, please e-mail: journals.permissions@oxfordjournals.org.

Superficial Siderosis Revealed by Isolated Cognitive Impairment

Julie Le Scanff1, Alain Vighetto2, Claire Gédéon1, Marc Bonnefoy1 and Pierre Krolak-Salmon1,2,3

1 Geriatric Department
2 Neurology Department, University of Lyon, Centre for Memory Resources and Research, Hospices Civils de Lyon, Hôpital Neurologique Pierre Wertheimer, France
3 INSERM Unit 821, Brain Dynamics and Cognition, Hôpital Le Vinatier, Lyon, France

Address correspondence to Dr. Pierre Krolak-Salmon, Service de gériatrie, Centre hospitalier Lyon Sud, Saint Genis Laval, France. Email: pierre.krolak-salmon{at}chu-lyon.fr


  Abstract

Superficial siderosis (SS) is a rare disorder due to chronic bleeding into the subarachnoid or intraventricular space. The most common clinical presentation is progressive ataxia and hearing loss. The authors report two patients who presented with dementia as the primary manifestation of SS. The cognitive impairment marked by cortical frontotemporoparietal dysfunction was consistent with the pattern of signal abnormalities seen on brain magnetic resonance imaging (MRI). Diagnosis of SS must be considered when T2*-weighted MRI shows typical signal hypointensity outlining the brain and spinal cord surfaces. Performing such MRI sequences appears to be of particular interest in the context of dementia etiological diagnosis.

Keywords Dementia; Diagnosis; Superficial siderosis

Received: April 28, 2008; Accepted: July 24, 2008

Decision Editor: Luigi Ferrucci, MD, PhD


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