December 20, 1985

Intracranial Calcification in Nephrogenic Diabetes Insipidus

Author Affiliations

From the Departments of Pediatrics (Drs Kanzaki, Omura, Miyake, and Enomoto) and Neurosurgery (Drs Miyata and Ishimitsu), Iwakuni (Japan) National Hospital.

JAMA. 1985;254(23):3349-3350. doi:10.1001/jama.1985.03360230081028

NEPHROGENIC diabetes insipidus (NDI) is a congenital hereditary disorder characterized by insensitivity of the renal tubule to antidiuretic hormone. This congenital hereditary disorder has been associated with a number of central nervous system complications.1,2 However, intracranial calcification is an extremely rare complication of this disorder.3 We describe a 2-year-old boy with NDI who presented with a unique pattern of intracranial calcification. Our case shows that NDI may result in organic brain damage if treatment is delayed.

Report of a Case  A boy 2 years 11 months old was admitted to our hospital because of a seizure. He was the product of a normal pregnancy, labor, and delivery of a 30-year-old, gravida 3, para 1, abortus 2 woman. The parents are not related and are of Japanese extraction. His birth weight was 2,900 g. He was given an Apgar score of 9 at both one and five minutes after