Pan African Medical Journal

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A three-year-old boy with hypodipsic hypernatremia syndrome

Bousayna Iraqi, Rachid Abilkassem, Nezha Dini, Aomar Agadr


We describe a three-year-old boy who had a growth and psychomotor retardation associated with inappropriate lack of thirst and vasopressin secretion in the presence of chronic plasma hyperosmolarity. Computed brain tomography revealed bilateral supratentorial sub-ependymal and cortical calcifications. Dissociation in the plasma vasopressin response to osmotic change was demonstrated in this patient. Treatment with a vasopressin analogue, desamino-D-arginine vasopressin (DDAVP) and forced intake of water restored plasma osmolality and serum sodium levels to normal.

Keywords: Brain calcifications, child, hypodipsic hypernatremia syndrome
AJOL African Journals Online