Symmetrical Calcification of the Cerebral Basal Ganglia

Abstract

In 1939 Eaton, Love and I (1) reported I six cases in which symmetric calcification of the cerebral basal ganglia was observed roentgenographically. In two of these cases there was definite clinical evidence of spontaneous parathyroid insufficiency and tetany. In retrospect, our original case, which was reported (2) in 1938 would also seem to be an example of this condition, although the diagnosis was not established before death. The basic pathologic changes responsible for symmetric calcification of the cerebral basal ganglia have been noted by many writers since Bamberger's (3) and Virchow's (4) original observations in 1855. It was not until 1935, however, that the first report concerning the roentgenographic appearance in vivo was published. In that year Fritzsche (5) described the changes observed in roentgenograms of three siblings and Kasanin and Crank (6) presented the roentgenographic and postmortem findings in one case. None of these authors mentioned the presence of parathyroid insufficiency. The older literature contains reports of seven cases of parathyroid insufficiency (tetany) in which calcification of the basal ganglia was observed by pathologists (7–11). In most cases the process was marked and, if roentgenograms had been made, these would probably have presented the characteristics of symmetric cerebral calcification. To date, at the Mayo Clinic, we have observed 12 cases of symmetric calcification of the cerebral basal ganglia in which there was definite clinical evidence of parathyroid insufficiency and tetany. In one, the calcification followed a thyroidectomy at the age of nineteen years; in the other 11, the disease was of the spontaneous type. The pathologic basis for the roentgenologic changes is a colloid deposition in and around the finer cerebral blood vessels, with subsequent calcification of the deposits, which coalesce and form vascular sheaths and concretions (Fig. 1). There is general agreement as to the involvement of the media and adventitia of the smaller arteries and the infrequency with which the veins are affected. When the process is extensive, the capillaries may be occluded but the lumens of the arteries are rarely narrowed (Fig. 1 b). Ostertag (8) concluded that the deposited colloidal material occurs so frequently in the anterior half of the globus pallidus and in the dentate nucleus of the cerebellum that its presence to a slight degree, if not normal, is at least not unexpected. A marked increase in the degree and extent of the vascular process is likely to occur at any age, in either sex, and in response to many diseases, not all of which need produce neurologic symptoms. Calcium is always present microscopically to a considerable degree before it can be revealed by present roentgenologic methods. In 5 of the 12 cases presented herewith, deposits of calcium were observed roentgenographically in the deeper layers of the cerebral cortex as well as in the basal ganglia and cerebellum.