Neurobehavioral profile of children with persistent hyperinsulinemic hypoglycemia of infancy

Abstract

We describe the neurocognitive profile of 6 school‐age children with persistent hyperinsulemic hypoglycemia of infancy (PHHI). PHHI causes severe intractable nonketotic hypoglycemia during the neonatal period and, unless aggressively treated, results in significant neurological morbidity. Early diagnosis and treatment have changed the natural history of PHHI. We have examined 7 children ages 6 to 12 years treated aggressively since birth. All 7 children were assessed by standard psychological tests and their IQs ranged from 91 to 123, with the exception of 1 child who had subnormal intelligence (IQ 65). These 6 children and 6 matched controls underwent a series of neuropsychological tests. The study children manifested impaired short‐term verbal and visual memory, dysgraphia and dyscalculia, and variable degrees of dysnomia and dyslexia. Their cognitive profile suggests bilateral temporoparietal dysfunction and is consistent with the known vulnerability of the posterior cortical regions of the neonate to hypoglycemia.