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Behavioural Neurology
Volume 2014, Article ID 750975, 3 pages
Case Report

Fahr’s Disease Presenting with Dementia at Onset: A Case Report and Literature Review

IRCCS Centro Neurolesi “Bonino-Pulejo”, 98124 Messina, Italy

Received 8 June 2013; Accepted 21 July 2013; Published 12 March 2014

Academic Editor: Stefano F. Cappa

Copyright © 2014 Rocco Salvatore Calabrò et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Fahr’s disease (FD) is characterized by sporadic or familiar idiopathic calcification of the basal ganglia, dentate nuclei of the cerebellum, and centrum semiovale, mainly presenting with movement disorder, dementia, and behavioral abnormalities. We described a rare case of Fahr’s disease presenting at onset only with behavioral and neuropsychological alterations, whose diagnosis was supposed only after a brain CT, which showed extensive bilateral calcifications in the dentate nuclei of the cerebellum and basal ganglia. Since the onset of Fahr’s disease may be a dysexecutive syndrome with behavioral abnormalities, the clinical and radiological features are really important to do the appropriate diagnosis.