Pseudohypoparathyroidism

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Abstract
A fifty-two year old man with progressive mental deficiency is described. Neurologic evaluation of an isolated right Babinski sign led to the discovery of dense calcification of the basal ganglia in his skull roentgenograms. Laboratory data revealed a serum Ca of 6.4 - 7.1 mg/100 ml and serum phosphate of 4.8 - 5.8 mg/100 ml. Short metacarpals were present in hand X-rays. There was no evidence of renal disease, malabsorption, or bone disease. The patient denied previous thyroid surgery and all manifestations of tetany. Three Ellsworth-Howard tests were performed, none of which could differentiate the patient from the controls. Administration of 2000 USP units of parathyroid extract in over 3 days produced no rise in serum Ca while the same dose elicited increments of 1.5 and 1.7 mg/100 ml in 2 normal controls. The authors discuss the inadequacies of the Ellsworth-Howard test in evaluating parathyroid sensitivity.

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