Pseudo-hypoparathyroidism: A Description of Three Cases and a Critical Appraisal of Earlier Accounts of the Disease

MacGregor, Malcolm E.; Whitehead, T. P.

doi:10.1136/adc.29.147.398

Cited by 68 publications

(21 citation statements)

References 57 publications

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“…The peripheral neurological complication, tetany, is commonly seen whatever the cause of hypocalcaemia. However, the central neurological complication of chorea has been reported only when the hypocalcaemia was a consequence of hypoparathyroidism (Simpson, 1952;Frame, 1965;MacGregor and Whitehead, 1954), or anti-convulsant therapy (Hosking et al, 1975). The parathyroid hormone estimation, during convalescence when the patient was normocalcaemic, was normal which does not exclude hypoparathyroidism but makes it unlikely.…”

Section: Discussionmentioning

confidence: 99%

“…In all but one of the previous reports of hypocalcaemia producing chorea, the hypocalcaemia has been a result of hypoparathyroidism, either idiopathic (Simpson, 1952), postoperative (Frame, 1965), or pseudo-hypoparathyroidism (MacGregor and Whitehead, 1954), the chorea being proportionately more common in the idiopathic variety (McKinney, 1962). In that one report, the chorea followed hypocalcaemia in a case of drug-induced osteomalacia during the treatment of epilepsy (Hosking et al, 1975).…”

Section: Introductionmentioning

confidence: 92%

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Hypocalcaemic chorea secondary to malabsorption

Howdle¹,

Bone²,

Losowsky³

1979

Postgraduate Medical Journal

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 92%

Hypocalcaemic chorea secondary to malabsorption

Howdle¹,

Bone²,

Losowsky³

1979

Postgraduate Medical Journal

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Section: Discussionmentioning

confidence: 99%

“…The Ellsworth-Howard test is unreliable as is shown by the inconsistency of the results obtained by different investigators (Dent, 1953;MacGregor and Whitehead, 1954;Surks and Levenson, 1962). The response of the serum calcium to large doses of parathormone is an improvement on this test, particularly if there is truth in the suggestion (MacGregor and Whitehead, 1954) that the parathyroid extract used in Britain has been affected by manufacturing processes, and while retaining its hypercalcaemic power, has lost its ability to increase urinary excretion of phosphorus.…”

Section: Discussionmentioning

confidence: 99%

“…The syndrome of pseudohypoparathyroidism de-scribedbyAlbright, Burnett, Smith, andParson (1942) has been well reviewed by MacGregor and Whitehead (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.…”

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Spinal cord compression in pseudohypoparathyroidism

Cullen¹,

Pearce²

1964

Journal of Neurology, Neurosurgery & Psychiatry

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The Syndrome of Pseudo-Pseudohypoparathyroidism

Wallach¹,

Englert²,

Brown³

1956

Arch Intern Med

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in 1942, described a group of patients with hypocalcemia, hyperphosphatemia, ectopic calcification and ossification, dyschondroplasia manifested by short metacarpal and metatarsal bones, and a characteristic short, round body and round face. Tetany, convulsive symptoms, lenticular opacities, and calcification of the basal ganglia were also present. Unlike persons with idiopathic hypoparathyroidism, these patients had normal parathyroid glands and there was little or no phosphate diuresis in response to the administration of parathyroid extract. Having named this syndrome pseudohypoparathyroidism, Albright and associates * postulated the presence of multiple, independent genetic defects to account for the stigmata noted and suggested that the hypocalcemia and hyperphosphatemia resulted from a failure of end-organ response to endogenous parathyroid hormone.Subsequently, there appeared case reports 3 of persons with some but not all of the stigmata described by Albright and associates.The term pseudopseudohypoparathyroidism has been applied to the condition in which hypocalcemia (and resultant tetany and convulsive symptoms) and hyperphosphatemia are absent but ectopie ossification, dyschondroplasia, and short stature and round face are present.Peculiarly, calcifi¬ cation of the basal ganglia has not been noted in this group despite the presence of ectopie calcification and ossification in other body areas.The present report concerns a patient with many characteristics of pseudopseudohypoparathyroidism and an un¬ usual type of dyschondroplasia. He also has gout, unexplained splenomegaly and lymphoid hyperplasia, and asymptomatic rheumatoid spondylitis. Report of CaseIn August, 1954, a 24-year-old white man, a machinist, developed mild aching in the right ankle two days after an inguinal herniorraphy. This progressed to severe inflammation, and several days later similar changes appeared in the right knee. Treatment by joint aspiration and with salicylates did not produce immediate relief, but the process gradually subsided after several days' rest. No further symptoms were noted until March, 1955, when inflammation of the metatarsophalangeal joint of the left hallux, left ankle, and left knee developed, resulting in admission to the Salt Lake Veterans Administration Hospital. The patient at¬ tributed the recurrence of this process to the trauma of ill-fitting shoes. Fever, chills, and pre¬ ceding infections were denied.Review of symptoms and past history were un¬ remarkable except for the presence of very short thumbs since birth. Two siblings, the patient's parents, and all other known relatives were free of recognized deformities. His mother and father were 63 in. and 69 in. tall, respectively. A 23-year-old sister measured 65 in. and was obese. A 17-yearold brother was 69 in. tall. Only the patient's sister could be considered to be round-faced. The hands of all members of the immediate family were normal except for the father, whose thumbs appeared short. A maternal uncle had gout. Physical examination revealed ...

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Pseudo-hypoparathyroidism: A Description of Three Cases and a Critical Appraisal of Earlier Accounts of the Disease

Cited by 68 publications

References 57 publications

Hypocalcaemic chorea secondary to malabsorption

Hypocalcaemic chorea secondary to malabsorption

Spinal cord compression in pseudohypoparathyroidism

The Syndrome of Pseudo-Pseudohypoparathyroidism

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