title: Abolished phosphaturic response to parathormone in adult patients with Fahr disease and its restoration after propranolol administration.
abstract: The similar localization of intracranial calcification in hypoparathyroidism and in Fahr disease without parathyroid gland disorder suggests that in these two disorders the pathomechanism of calcium phosphate deposition in the brain may be similar. It may be that in Fahr disease some factors, such as chronic respiratory alkalosis, could lead to hypoparathyroidism-like changes in the brain tissue. Abolition of the phosphaturic response to parathormone (PTH) was recently demonstrated in acute experimental hypocapnia. In three adult patients with Fahr disease, a tendency towards compensatory respiratory alkalosis and arterial hypocapnia was found. The parathormone test revealed a marked decrease in phosphaturia response to PTH, but normal cAMP response. In one patient, the parathormone test was repeated during propranolol administration and showed a considerable improvement in the phosphaturic response to parathormone. It is postulated that chronic hyperventilation and hypocapnia as well as phosphaturic resistance to PTH, intracellular increase of phosphate concentration and development of hypoparathyroidism-like intracranial calcification in patients with Fahr disease could all be caused by disturbance of adrenergic receptors and their relationship to PTH receptors.
authors.affiliation: Department of Metabolic Diseases, Monument-Hospital Child Health Centre, Warsaw, Poland.
authors.forename: E
authors.initials: E
authors.lastname: Pronicka
authors.forename: J
authors.initials: J
authors.lastname: Kulczycki
authors.forename: E
authors.initials: E
authors.lastname: Rowińska
authors.forename: W
authors.initials: W
authors.lastname: Kuran
mesh_terms: D000328:Adult; D001927:Brain Diseases / Q000097:blood / Q000652:urine*; D002114:Calcinosis / Q000097:blood / Q000652:urine*; D002118:Calcium / Q000097:blood; D000242:Cyclic AMP / Q000652:urine; D005260:Female; D006801:Humans; D008297:Male; D010281:Parathyroid Hormone; D010710:Phosphates / Q000097:blood / Q000652:urine*; D011433:Propranolol / Q000494:pharmacology*
chemical_list: D010281:Parathyroid Hormone; D010710:Phosphates; D011433:Propranolol; D000242:Cyclic AMP; D002118:Calcium