Idiopathic hypoparathyroidism presenting as chorea

Abstract

The case of an 8-year old girl with idiopathic hypoparathyroidism, presenting with chorea and oculogyric crises, is described. The literature was reviewed for patients with hypoparathyroidism and abnormal movements of the basal ganglion type and a total of 17 such cases was uncovered. Chorea, paralysis agitans, oculogyric crises, torticollis, athetosis, and dystonia were among the movements described. Basal ganglion calcification did not correlate with the presence of abnormal movements. The case records of patients diagnosed as having hypoparathyroidism, hypocalcemia, tetany, and chorea at the Columbia-Presbyterian Medical Center from the years 1950 to 1960 inclusive were reviewed. Out of a total of 442 cases, there were 2 cases of hypoparathyroidism in which the histories were suggestive of oculogyric crises. There was another case of a patient who had had episodes of chorea thirteen years previously. The mechanisms whereby hypoparathyroidism induces basal ganglion dysfunction are hypothetically considered. It is concluded that, among the 2 explanations offered, the one postulating functional hyperirritability of the nerve fiber fits the clinical situation better than does the possibility of selective areas of ischemia.