The most commonly affected region of the<unk>  is the lenticular nucleus and in particular the internal globus pallidus.<unk> s in the caudate, dentate nuclei, putamen and thalami are also common. Occasionally<unk> s begin or predominate in regions outside the basal ganglia.<unk>  seems to be progressive, since<unk> s are generally more extensive in older individuals and an increase in<unk>  can sometimes be documented on follow up of affected subjects. As well as the usual sites the cerebellar gyri,<unk>  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<unk> s. Histologically concentric calcium deposits within the walls of small and medium-sized arteries are present. Less frequently the veins may also be affected. Droplet<unk> s 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. The<unk> s seen in this condition are indistinguishable from those secondary to hypoparathyroidism or other causes.
Primary familial brain calcification
Generalized arterial calcification of infancy
Cerebral arteriovenous malformation
Normolipoproteinemic xanthomatosis
Osteopathia striata
Vertebral hemangioma
Intravascular lymphomas
Gerodermia osteodysplastica