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Fahr's disease; striopallidodentate calcinosis; idiopathic basal ganglia calcification
idiopathic basal ganglia calcification
Epidemiology:
- rare
Pathology:
- basal ganglia calcification
- Calcified deposits consist of calcium carbonate & calcium phosphate
- calcification may also occur in
- thalamus, hippocampus, cerebral cortex, cerebellar subcortical white matter dentate nucleus [5]
Genetics:
- dominantly inherited
Clinical manifestations:
- patients typically present in the 5th decade of lif
- case reports {3,4] of
- cognitive impairment
- behavioral disorder
- depression
- movement disorders [3]
- dystonia, ataxia, & parkinsonism
General
genetic disease of the central nervous system
neurodegenerative disease
Database Correlations
OMIM 213600
References
- National Institute of Neurological Disorders and Stroke (NINDS)
Fahr's Syndrome Information Page
https://www.ninds.nih.gov/disorders/all-disorders/fahrs-syndrome-information-page
- OMIM 213600
- Randhawa J, Onyshchenko M, Mbuvah F, Bagai R.
A Rare Association of Fahr's Disease With an Autoimmune Tria
J Neuropsychiatry Clin Neurosci. 2014 Fall;26(4):E16-8.
PMID: 26037872
https://doi.org/10.1176/appi.neuropsych.13100243
- Samuels C R, Khanni J L, Barkley K, et al.
Case Report: An Incidental Finding of Fahr's Disease in a Patient
with Hypochondria.
Cureus. 2018 Dec 1;10(12):e3668.
PMID: 30761221 Free PMC article.
https://www.cureus.com/articles/15165-case-report-an-incidental-finding-of-fahrs-disease-in-a-patient-with-hypochondria
- Saleem, S., Aslam, H.M., Anwar, M. et al.
Fahr's syndrome: literature review of current evidence.
Orphanet J Rare Dis 8, 156 (2013)
PMID: 24098952 Free PMC article
https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-8-156