Fahr’s syndrome and polyglandular syndrome type 4 in a patient with inadequate antidiuretic hormone secretion

Abstract

A 73-year-old woman with a history of epilepsy, cognitive impairment, stiffness and involuntary movements. She was admitted for emergency with disorientation and speech difficulty. Cerebral TEM showed calcifications in the basal ganglia and cerebellar hemispheres suggestive of Fahr’s syndrome. Primary hypoparathyroidism plus autoimmune thyroid disease was demonstrated with hypotonic euvolemic hyponatremia and no alteration of other pituitary axes. The diagnosis of autoimmune polyglandular syndrome type 4 was made; in addition, the dosage of ADH confirmed the diagnosis of inadequate secretion of antidiuretic hormone. Intravenous calcium, vitamin D supplements and calcitriol were administered, with significant clinical neurological improvement.