Calcification of the corpus stiatum and dentate nuclei occurring in a family.

Dense symmetrical calcification of the corpus striatum is rare, but of some general interest for two reasons. First, in a significant number of the reported cases this apparently degenerative condition is closely linked with a metabolic disorder, hypoparathyroidism, which may be idiopathic, parathyroprivic, or of the variety in which the parathyroid glands are histologically normal but the body is unable to respond to parathormone (Albright's pseudohypoparathyroidism). Secondly, in no other degenerative disease limited to a neurophysiological system can the process be visualized radiologically. In contrast with the rare gross calcification under discussion, microscopically demonstrable calcification occurring on a basis of hyaline degeneration in and around the vessels in these regions is very common, and can, indeed, be found to a certain extent in normal brains. Such calcification formerly attracted much attention in certain inflammatory conditions, chorea, encephalitis lethargica, polio-encephalitis, and cerebral malaria; in intoxications with carbon monoxide, nitrous oxide, and lead; in degenerative conditions such as amaurotic idiocy and the progressive pigmentary pallidonigral degeneration of Hallervorden and Spatz , and in certain metabolic and %-ndocrine disorders (other than hypoparathyroidism) such as Addison's disbase, cretinism, myxoedema, and severe pernicious anaemia.. Calcification in the basal ganglia gross enough to be visible radiologically occurs in two main groups of conditions: in association with parathyroid insufficiency, as is now well known, and in icerta rAre frnpilial instances. It is in the latter, of which the family reported below is the third in the literature, that the calcification attains its most florid form.