COGNITIVE AND PSYCHOSOCIAL FUNCTIONING OF PATIENTS WITH CONGENITAL NEPHROGENIC DIABETES-INSIPIDUS

Mental retardation (MR) is generally considered one of the main compli cations of congenital nephrogenic diabetes insipidus (NDI), However, p sychometric studies of NDI patients are scarce and outdated, In the pr esent study, 17 male NDI patients underwent psychological evaluation, Total intelligence quotient of 14 patients was within (n = 13) or abov e (n = 1) the normal range, 1 patient had an intelligence score betwee n -1 and -2 standard deviations (S.D.) and 2 young patients had a gene ral cognitive index more than 2 S.D. below the norm, Attention deficit hyperactivity disorder criteria were met by 8 out of 17 patients and scores on short-term memory were low in 7 out of 10, No relation betwe en test performances and age at diagnosis or hypernatremia could be fo und, with the exception of a negative correlation between age at start of therapy and verbal IQ in one age group, Although several explanati ons for an association between MR and NDI can be postulated, it seems that the current prevalence of MR among patients with this disease is considerably lower than suggested in literature. (C) 1996 Wiley-Liss, Inc.