Pathology
The most commonly affected region of the brain is the lenticular nucleus and in particular the internal globus pallidus. Calcifications in the caudate, dentate nuclei, putamen and thalami are also common. Occasionally calcifications begin or predominate in regions outside the basal ganglia.
Calcification seems to be progressive, since calcifications are generally more extensive in older individuals and an increase in calcification can sometimes be documented on follow up of affected subjects.
As well as the usual sites the cerebellar gyri, brain stem, centrum semiovale and subcortical white matter may also be affected.
Diffuse atrophic changes with dilatation of the subarachnoid space and/or ventricular system may coexist with the calcifications.
Histologically concentric calcium deposits within the walls of small and medium-sized arteries are present. Less frequently the veins may also be affected. Droplet calcifications can be observed along capillaries. These deposits may eventually lead to closure of the lumina of vessels.
The pallidal deposits stain positively for iron. Diffuse gliosis may surround the large deposits but significant loss of nerve cells is rare.
On electron microscopy the mineral deposits appear as amorphous or crystalline material surrounded by a basal membrane. Calcium granules are seen within the cytoplasm of neuronal and glial cells.
Read more about this topic: Fahr's Syndrome
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