Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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April 20, 2009
Fahr's Disease
- Rare degenerative neurological disorder characterized radiologically by bilateral dense basal ganglia and cerebellar (dentate nucleus) calcifications without contrast enhancement
- Patients present with progressive mental deterioration along with extrapyramidal movement disorders.
- Symptoms usually seen between ages 30 and 60.
- CT is the best imaging modality to show the symmetric calcifications.
- PET imaging may show decreased uptake in the basal ganglia bilaterally.
- There is no specific treatment to limit progression of calcification.
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