Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2013 Mar;7(3):532-3.
doi: 10.7860/JCDR/2013/4946.2814. Epub 2013 Mar 1.

Fahr's Syndrome- An Interesting Case Presentation

Athulya Geetha Asokan  1 , Sydney D'souza, Jayakumar Jeganathan, Shivananda Pai
Affiliations

Fahr's Syndrome- An Interesting Case Presentation

Athulya Geetha Asokan et al. J Clin Diagn Res. 2013 Mar.

Abstract

Fahr's syndrome refers to a rare syndrome which is characterized by symmetrical and bilateral intracranial calcification. We are presenting a 63 year old male, who complained of progressive dysarthria of 6 months, which was associated with slowness of movements. His neurological examination revealed Parkinsonian features. His CT scan revealed a symmetrical large area of calcification over the basal ganglia, the thalamus and the cerebellum. The secondary causes of the bilateral calcification were ruled out to make the clinical diagnosis of Idiopathic Bilateral Striopallidodentate Calcinosis, which is otherwise called as the Fahr's syndrome. Deposits of calcium and minerals cannot be linked to a single chromosomal locus and further genetic studies are in progress for identifying the chromosomal locus of the disease.

Keywords: Bilateral intracranial calcification; Fahr’s syndrome.

PubMed Disclaimer

Figures

[Table/Fig-1]:
[Table/Fig-1]:
CT Head showing bilateral symmetrical calcification in the cerebrum (fig-1a), cerebellum (fig-1b), basal ganglia (fig-1c)
See this image and copyright information in PMC

References

    1. Gülsün Murat, FuatBaykýz Ali, Kabata Serdar, Belli Hasan. Fahr’s Syndrome - Three cases presenting with psychiatric signs. Eur J Gen Med. 2006;3(1):35–40.
    1. Kotan D, Aygul R. Familial Fahr’s disease in a Turkish family. South Med J. 2009;102(1):85–86. - PubMed
    1. Billard C, Dulac O, Bouloche J, Echenne B, Lebon P, Motte J. Encephalopathy withcalcifications of the basal ganglia in children. A reappraisal of Fahr’s syndrome with respect to 14 new cases. Neuropediatrics. 1989;20(1):12–19. - PubMed
    1. Malik R, Pandya VK, Naik D. Fahr’s disease: A rare neurodegenerative disorder. Indian J Radiol Imaging. 2004;14:383–84.
    1. Manyam BV. Bilateral strio-pallido-dentate calcinosis: a proposed classification of genetic and secondary causes. Mov Disord. 1990;5(Suppl 1):94S.

LinkOut - more resources