Spinal Cord Compression in Pseudohypoparathyroidism.

The syndrome of pseudohypoparathyroidism describedbyAlbright, Burnett, Smith, andParson (1942) has been well reviewed by MacGregor andWhitehead (1954) and by Mann, Alterman, and Gorman Hills (1962). The clinical features consist of mental retardation, epilepsy and attacks of tetany, as well as ectodermal defects such as alopecia, defective dentition, brittlenails, andcataracts. There are also certaincharacteristic somatic abnormalities. The patient has a moon face, a short stocky stature, and shortening of some of the metacarpals and metatarsals due to premature epiphyseal closure. The bone structure is normal but ectopic calcification can usuallybe seen in the basal ganglia and in the subcutaneous tissues. The diagnosis is confirmed by finding low serum calcium and high serum phosphorus levels. Dickson, Morita, Cowsert, Graves, and Meyer (1960) have recorded the occurrence of E.E.G. abnormalities in pseudohypoparathyroidism. The syndrome has also been associated with diabetes mellitus, diabetes insipidus, and hypothyroidism (Mann et al., 1962). There has recently been controversy regarding the nosological status of the two conditions, which have been called pseudohypoparathyroidism and pseudopseudohypoparathyroidism. In the latter condition (Albright, Forbes, and Henneman, 1952) similar clinical and radiological abnormalities are found but the values of serum calcium and phosphorus are normal. Most workers now regard the latter condition as being an incompletely expressed form of the syndrome of pseudohypoparathyroidism (Dickson et al., 1960; Mann et al., 1962) and there seems to be little point in attempting to identify the two disorders as distinctive clinical entities. The following case report concerns a young woman with pseudohypoparathyroidism who developed a progressive spastic paraplegia due to spinal cord compression caused by abnormal bone formation.