Arch Dis Child: first published as 10.1136/adc.24.117.77 on 1 March 1949. Downloaded from IDIOPATHIC HYPOPARATHYROIDISM BY F. S. W. BRIMBLECOMBE, M.B., M.R.C.P. Late Children's Registrar, Harefield E.M.S. Hospital, Harefield, Middlesex Cases of spontaneous hypoparathyroidism, at rest and during exercise. During these spasms although rare, have been reported frequently in the her feet were held in the position of extreme talipes literature (as by Albright and Ellsworth (1929), equino-varus, and she often cried out from the pain Snapper (1934), Campbell (1935), Himsworth and in her legs. At this time, also, she began to com- Maizels (1940), Goerner and plain of frontal headaches. Samuelson (1934), and One afternoon in August, 1941, she complained Robertson (1941)). The disease is characterized by of frontal headache and went to bed. An hour increased neuromuscular irritability giving rise to later she was found in bed unconscious with marked tetany, and by a lowered blood calcium and raised pallor and rigidity of all her muscles; there was blood phosphorus., Cataracts may develop in some froth on her lips and her eyes were open and untreated cases. Generalized convulsions may staring. She recovered in five minutes and fell into occur. Although many cases arise in childhood, a deep sleep. During the next six weeks she had the disease may occur at any age, and one patient many of these seizures, the number varying between described by Snapper (1934) did not develop one and five a day. The attacks started without symptoms until the age of 76. The diagnosis is warning, and consisted first of a tonic phase lasting two to five minutes, during which time she was very established when other factors causing tetany such pale, followed by a clonic period of shorter duration as alkalosis, any form of rickets, osteomalacia, accompanied by opisthotonus, frothing at the coeliac disease, and steatorrhoea have been mouth, biting of the tongue, and sometimes incon- excluded. tinence of urine; the clonic phase was followed by copyright. Idiopathic hypoparathyroidism has been described a -period of unconsciousness lasting ten to twenty by Hunter (1931) in his classical review of calcium minutes, after which she would fall into a deep and phosphorus metabolism, as being a condition sleep. Between the seizures the spasms of rigidity analogous to myxoedema in relation to hypo- in her hands and feet continued to occur. thyroidism. Symptomatically it is found to be In October, 1941, she was given epanutin gr. i at identical with hypoparathyroidism following too night, and this appeared to reduce the severity of the seizures though it did not abolish them and had extensive thyroidectomy which has included removal no effect on the spasms in her limbs. She was of the parathyroid glands. admitted to Harefield Emergency Hospital under http://adc.bmj.com/ A case of spontaneous hypoparathyroidism, the care of Dr. Lightwood on Nov. 13, 1941. Her which has now been under observation for over parents and brother, aged 10, were alive and well, six years, and which has presented some unusual and there was no family history of epilepsy or other features is now reported and discussed. disease. On examination she was found to be afebrile but Case Report drowsy. She could, however, be roused to talk Sylvia A. developed normally until the age of intelligently for her age. Examination of her lungs, 7 years, having suffered only from acute intus- heart, and abdomen showed no abnormality. Her on October 1, 2021 by guest. Protected susception as a baby, and measles and whooping- pupils were equal and active to light, there was no cough in early childhood. At the age of 7, however, nystagmus, and her eye movements were normal. it was noticed that her fingers became rigid on first There was no papilloedema. waking up in the mornings, remaining immovable No cranial nerve abnormality was detected and usually for about fifteen minutes. During the spasms her limbs showed no paresis or anaesthesia, all her her fingers were flexed at the metacarpo-phalangeal reflexes were normal. Blood pressure was joints and extended at the interphalangeal joints. 105/70 mm. Hg. Her weight was 51 lb. (normal At first these spasms occurred only occasionally for age 55 to 60 lb.). but gradually they became more frequent and of Two spasms of her hands occurred on that day, longer duration. During them she remained quite and during each of them her handswereseentoadopt conscious and used to laugh at her hands which the typical 'main d'accoucheur ' position. After apparently gave her no pain. the spasms were over Trousseau's and Chvostek's In 1941, at the age of 8, she developed similar signs were found to be positive. Investigations at attacks of rigidity of her feet which occurred both this time showed the blood calcium to be 5 8 mg. 77 78 ARCHIVES OF DISEASE IN CHILDHOOD Arch Dis Child: first published as 10.1136/adc.24.117.77 on 1 March 1949. Downloaded from per 100 ml. of blood and 9 8 mg. blood phosphorus. symptoms, on a maintenance dose of vitamin D, Carbon dioxide combining power was 50-5 volumes 100,000 I.U. daily. per cent. The urine contained no albumin. Blood During the next two and a half years this dose of urea was 40 mg. per 100 ml. A chest radiograph vitamin D was continued, and she was observed at was clear, and one of the skeleton showed no the Hospital for Sick Children, Great Ormond decalcification (compared with a control) and no Street, as an out-patient. No untoward symptoms bony abnormalities. occurred until June, 1944, when the vitamin D was Analysis of the stools gave the following figures: discontinued. Two weeks after this she developed total fat 29 6 g. per cent.;- neutral fat 1 -7 g. per frank tetany with convulsions which were relieved cent.; free fatty acid 4 1 g. per cent.; fatty acid by the injection of intravenous calcium gluconate. (as soap) 23 8 g. per cent. Blood calcium at this time was 8 - 2 mg. per 100 ml. The cerebrospinal fluid pressure was 175 mm. of The vitamin D was immediately restarted, no further fluid. Queckenstedt's test was normal. There was tetany occurred, and her blood calcium returned to 20 mg. per 100 c.cm. of protein and 720 mg. per normal. 100 ml. of chlorides. There were 3 lymphocytes In August, 1944, she developed vomiting, per c.mm. generalized abdominal pain, and constipation, and Haemoglobin was 90 per cent. (Haldane), and the was admitted to another hospital. She was dis- blood sedimentation rate 9 mm. in one hour. charged home after a week, no diagnosis having In view of these investigations a diagnosis of been made. primary parathyroid tetany was made. After this she continued to have attacks of Treatment was started after a week in hospital vomiting, particularly in the mornings, associated and consisted first in the administration of calcium with increasing anorexia. She complained of right lactate, 1 drachm three times a day; but this frontal headaches and became increasingly listless produced no alteration in her tetany or serum and unco-operative. She was-readmitted to Hare- calcium. On Nov. 18, therefore, this treatment was field Emergency Hospital in November, 1944, discontinued and vitamin D therapy was started. having lost 17 lb. in weight in the previous five An initial daily dose of 18,000 I.U. of vitamin D2 months. (calciferol) was employed for one week but pro- Examination now revealed that she was a tall, duced no effect on her symptoms or blood chemistry, thin, lethargic girl with sunken eyes and somewhat nor did 36,000 I.U. daily for a similar period. The hirsute skin; she had slight bilateral conjunctivitis. dose was finally increased to 100,000 L.U. a day, Her lungs were clear. Her pulse was regular at copyright. and after two weeks on this treatment both the 80 to the minute, and the blood pressure was overt and latent tetany was abolished and her blood 115/70 mm. Hg. There was no cardiac enlargement calcium rose to 8-9 mg. per 100 ml., whilst the and no murmurs. Her abdomen showed no phosphorus fell to 8-2 mg. After a further week abnormality. Her central nervous system was her blood calcium had risen to 9 8 mg. per 100 ml. normal, and Trousseau's and Chvostek's signs were Daily estimations over the next month showed negative. A chest radiograph was clear. No little alteration in the blood calcium, which remained calcification was seen in a radiograph of the steady between 9-6 and 10-4 mg. per 100 ml., abdomen, and renal outlines appeared normal. A while the blood phosphorus fell to 5 0 mg. She radiograph of the skeleton showed normal calcifica-- http://adc.bmj.com/ gained 7 lb. in weight during this period and was tion (as compared with a control). No bony discharged home on Jan. 18, 1942, free from abnormality was seen. Serum calcium (thrice repeated) was 15 -6 mg. per 100 ml., blood phosphorus 5-4 mg., blood phosphatase 20 (King) units, serum sodium 316 mg., II~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~' serum potassium 18 mg., and blood chlorides vLO/A7 f0 sOs / 0000- /-x 590 mg. It .. ..*. There was a trace of albumin in the urine, no on October 1, 2021 by guest. Protected sugar, and a few granular casts and pus cells. The Addis count was 9 million red cells per twelve- hour specimen. Blood urea was 81 mg. per 100 c.cm. The urea clearance test (thrice repeated) showed 22 per cent.