Exp Clin Endocrinol Diabetes 2002; 110(5): 245-247
DOI: 10.1055/s-2002-33074
Case Reports

© Johann Ambrosius Barth

Pseudohypoparathyroidism and Graves'disease: a rare combination of two endocrinological diseases

A. Gerhardt, K. Hackenberg
  • Department of Medicine, Evangelisches Krankenhaus Herne, Academic Teaching hospital of the University of Bochum, Germany
Further Information

Publication History

received 22 March 2001 first decision 22 June 2001

accepted 15 January 2002

Publication Date:
30 July 2002 (online)

Summary

We report a 26-year-old woman presented at the day of admission in the I.C.U. with increased perspiration, plethora and distinct tetany of both legs. Particularly unusual was an exophthalmus on both sides, a rectal temperature of 38.3 ° Celsius and a blood pressure of high level (180/110 mmHg).

Laboratory findings were a low serum calcium concentration of 2.86 mval/l, a hyperphosphataemia (5.0 mg/dl), free thyroxine of 31.7 pmol/l, TSH basal of < 0.01 U/ml and positive MAK and TRAK.

Serum parathormone concentration was excessively high: 766 ng/l (12-72).

Ultrasound of the thyroid gland revealed a normal size with a volume of 10.4 ml; the echosonic state was not typical for Graves' disease.

The initial treatment consisted of high dose thiamazole and hydrocortisone intravenous, calciumcarbonate and propranolol per os.

After acute situation the treatment continued with thyreostatics, calcitriol and calciumcarbonate.

The symptoms at the day of admission (tetany) disappeared within 2 days; only local paraesthesia of fingers persisted longer.

Normalization of thyroid parameters was reached after 11 days; the serum calcium concentration persisted on an increasing but still lower level than standard (3.8 mval/l).

During substitution parathormone decreased to 443 ng/l.

What is unusual about this case is the combined appearance of autoimmunethyreoiditis (Graves' disease) and pseudohypoparathyroidism.

References

  • 1 Albright F, Burnett C H, Smith P H. Pseudohypoparathyroidism: an example of “Seabright-Bantam-Syndrome”.  Endocrinology. 1942;  30 922-932
  • 2 Bastepe M, Juppner H. Pseudohypoparathyroidism. New insights into an old disease.  Endocrinol Metabo Clin North Am. 29(3) 569-589 2000; 
  • 3 Bastepe M, Lane A H, Juppner H. Paternal uniparental isodisomy of chromosome 20 q - and the resulting changes in GNAS1 methylation - as a plausible cause of pseudohypoparathyroidism.  Am J Hum Genet. 68(5) 1283-9 2001; 
  • 4 Blecher M. Receptors, antibodies and disease.  Clin Chem. 30(7) 1137-56 1984; 
  • 5 Coutant R, Care J C, Mathivon L, Boisson Lesage C. et al . Primary hypothyroidism revealing pseudohypoparathyroidism without hypocalcaemia and hypophosphataemia.  Arch Pediatr. 4(5) 433-7 1997; 
  • 6 Drezner M, Neelon F A, Lebovitz H E. Pseudohypoparathyroidism Type II: a possible defect in the reception of the cyclic AMP signal.  N Engl J Med. 1973;  289 1056-1060
  • 7 Eubanks P J, Stabile B E. Osteitis fibrosa cystica with renal Parathyroid hormone resistance.  Arch Surg. 1998;  133 673-676
  • 8 Gerok. Endokrinologie (2Bd.) editor, Urban & Schwarzenberg 1992
  • 9 Kanatani M, Sugimoto T, Kaji H, Ikeda K, Chiara K. Skeletal responsiveness to parathyroid hormone in pseudohypoparathyroidism.  Eur J Endocrinol. 144 (3) 263-9 2001; 
  • 10 Kruse K. Myopathien bei endokrinologischen Störungen.  Monatschr Kinderhilkd. 132 (8) 581-6 1984; 
  • 11 Liu J, Litman D, Rosenberg M J, Yu S, Biesecker L G, Weinstein L S. A GNAS1 imprinting defect in pseudohypoparathyroidism type Ib.  J Clin Invest. 106 (9) 1167-74 2000; 
  • 12 Spiegel A M. The molecular basis of disorders caused by defects in G proteins.  Horm Res. 47 (3) 89-96 1997; 
  • 13 Tollin S R, Perlmutter S, Aloia J F. Serial changes in bone mineral density and bone turnover after correction of secondary hyperparathyroidism in a patient with pseudohypoparathyroidism type Ib.  J Bone Miner Res. 15 (7) 1412-6 2000; 
  • 14 Uchimura H, Ariyama T, Wada K. et al . A case of pseudohypoparathyroidism with hyperthyroidism.  Calcified tissue research. 15 (2) 161 1974; 
  • 15 Vlaeminck-Guillem V, Wemeau J L. Pseudohypoparathyroidism and the concept of the hormonal resistance. Types Ib and II.  Presse Med 1999. 28 (26) 1442-7 1999; 
  • 16 Wu W J, Schwindinger W F, Apacio L F, Levine M A. Selective resistance to parathyroid hormone caused by a novel uncoupling mutation in the carboxyl terminus of Galpha(s). A cause of pseudohypoparathyroidism type Ib.  J Biol Chem. 276 (1) 165-171 2001; 
  • 17 Yokoro S, Matsou M, Ohtsuka T, Ohzeki T. Hyperthyrotropinemia in a neonate with normal thyroid levels: the earliest diagnostic clue for pseudohypoparathyroidism.  Biol Neonate. 58 (2) 69-72 1990; 

Dr. med. A. Gerhardt

Department of medicine (Prof. Dr. med. K. Hackenberg)

Evangelisches Krankenhaus Herne

Academic teaching hospital of the University of Bochum

Wiescherstr. 24

44623 Herne, Germany

Phone: 02323/498-0

Fax: 02323/498-2485

Email: a.gerhardt@evk-herne.de