Holoprosencephaly with neurogenic hypernatremia

Background. - Semi-lobar holoprosencephalies can be seldom complicated by n eurogenic hypernatremia which must be distinguished from other causes of hy pernatremia. Case report. - In two admitted children with semi-lobar holoprosencephaly, 7 months and 4 years old, biological data revealed chronic hypernatremia an d hyperosmolarity without clinical signs of dehydration, which were finally attributed to a neurogenic hypernatremia. Conclusion. - Neurogenic hypernatremia must be clearly differentiated from other causes of hypernatremia since it never causes specific complications. (C) 1999 Elsevier, Paris.