Familial calcification of the basal ganglia with response to parathormone.

Massive symmetrical calcification of the basal ganglia and dentate nuclei is now a well recognized radiological finding and great emphasis has been laid on its association with hypoparathyroidism (Lowenthal, 1948). While such an association is undeniable the connexion between the two phenomena is ill understood and cannot at present be accepted as invariable. It is the purpose of this paper to describe a family in which certain members were afflicted in middle life by a progressive neurological disorder accompanied by calcification of the basal ganglia. No clinical or chemical evidence of hypoparathyroidism was present but, in the member investigated in detail, an abnormal response to parathyroid hormone was observed.