Summary
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.
References
Dresner M, Neelon TA, Lebovitz HE (1973) Pseudohypoparatyroidism type II: a possible defect in the reception of the cyclic AMP signal. N Engl J Med 289:1056
Editorial (1982) Hyperventilation syndromes. Lancet II:1498–1499
Herman S, Stickier J, Lucas A (1981) Hyperventilation syndrome in children and adolescents: long-term follow up. Pediatrics 67:183–187
Hoppe A, Metler M, Berndt T, Knox F, Angielski S (1982) Effect of respiratory alkalosis on renal phosphate excretion. Am J Physiol 243:F471-F475
Hoppe A, Tulko T, Rybczyńska A, Angielski S (1985) β-Adrenoreceptors and respiratory alkalosis induced resistance to phosphaturic effects of PTH and cAMP. Miner Electrolyte Metab 11:13
McKusick V (1986) Mendelian inheritance in man. Johns Hopkins University Press Baltimore
Nyland H, Skre H (1977) Cerebral calcinosis with late onset encephalopathy: an unusual type of pseudo-pseudohypoparathyroidism. Acta Neurol Scand 56:309
Potts YT (1983) Pseudohypoparathyroidism. In: Stanbury JB, Wyngaarden JB, Fredrickson DS (eds) Metabolic basis of inherited disease. McGraw-Hill, New York
Rodriques HJ, Villareal, Klahr S, Slatopolsky E (1974) Pseudohypoparathyroidism type II: restoration of normal renal responsiveness to parathyroid hormone by calcium administration. J Clin Endocrinol Metab 39:693
Woodcock E, Johnston C (1985) Renal proximal tabular α-adrenergic receptors oppose urinary 3,5′-cyclic adenosine monophosphate response to parathyroid hormone in vivo. Endocrinology 116:1085–1089
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Pronicka, E., Kulczycki, J., Rowińska, E. et al. Abolished phosphaturic response to parathormone in adult patients with Fahr disease and its restoration after propranolol adminstration. J Neurol 235, 185–187 (1988). https://doi.org/10.1007/BF00314315
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DOI: https://doi.org/10.1007/BF00314315