[Familial diabetes insipidus of central origin (author's transl)].

In a 10-year-old girl admitted to hospital for polyuria and polydipsia, the central nervous system origin of the symptoms was demonstrated by low antidiuretic hormone levels (inferior to 1 pg/ml) and reduction of diuresis after administration of 1-deamino-8D-arginine-vasopressin (DDAVP). A study of the girl's family history showed that the disease was hereditary and autosomal dominant. Intranasal instillations of DDAVP twice daily constitute the best known treatment of the condition.