DIFFERENT NEUROLOGIC OUTCOMES IN 2 PATIENTS WITH NEONATAL HYPERINSULINEMIC HYPOGLYCEMIA

The authors report the cases of two patients with neonatal onset of hy poketotic hypoglycemia with hyperinsulinism and a poor response to dia zoxide. Pancreatic venous sampling showed a diffuse pancreatic hyperpl asia in one patient and a focal lesion in the other. The second patien t, diagnosed after a significant delay, suffered severe longterm neuro logical sequelae, despite having more limited hyperplasia; the brain M RI also showed severe pathological changes in cortex and white matter, predominantly in the parietooccipital region. Early and accurate diag nosis is critical in these patients in whom hypoglycemia is compounded by a lack of the ketone bodies which represent a vital alternative so urce of energy for the central nervous system.