Case 10
History:
55 year old male progressive deterioration of mental function
Findings:
Unenhanced CT reveals dense calcifications within the basal ganglia, subcortical white matter of the posterior parietal lobes, and the dentate nuclei of the cerebellum.
Diagnosis:
Fahr's Disease
Discussion:
Fahr’s Disease is a rare degenerative condition characterized by calcium deposition and associated cell loss in certain areas of the brain such as basal ganglia, subcortical white matter and cerebellum. The condition is often referred to as idiopathic basal ganglia calcification or IBGC because there is no apparent explanation for such calcification in these brain regions (idiopathic). Associated symptoms include progressive deterioration of cognitive abilities (dementia) and loss of acquired motor skills. As the condition progresses, paralysis may develop that is associated with increased muscle stiffness. Additional abnormalities athetosis or chorea.
Fahr’s Disease is often familial with variable inheritance patterns. In other instances, the condition appears to be sporadic. There are no know cures and therapy is mostly supportive.
References:
- Osborn A. Diagnostic Neuroradiology. Mosby, St. Louis; 1994:745-746.
- Haaga JR. Computed Tomography and Magnetic Resonance Imaging of the Whole Body. Mosby, St. Louis; 1994
