Computed tomography and magnetic resonance imaging (MRI) were used to
examine three male siblings with nephrogenic diabetes insipidus (NDI).
The two elder brothers had verying degrees of unusual intracranial ca
lcification; the eldest also showed involvement of the cerebral white
matter on MRI. The severity of intracranial calcification was related
to the time before initiation of treatment and inversely to mental abi
lity. Brain damage and mental retardation in NDI may be caused by a de
lay in initiating treatment; early detection and treatment are importa
nt to prevent brain damage.