Long-term MRI findings of a case with persistent hyperinsulinemic hypoglycemia of infancy (nesidioblastosis)

Abstract Background and purpose To describe the sequential magnetic resonance imaging (MRI) findings in a neurologically handicapped newborn who had been suffered from neonatal hypoglycemia due to persistent hyperinsulinemic hypoglycemia of infancy (nesidioblastosis) and to evaluate the following changes in the long-term radiological follow-up. Case A case of newborn with severe hypoglycemia due to nesidioblastosis is reported. The patient was presented with poor feeding, irritability, and seizures. Nesidioblastosis was diagnosed on the basis of high intravenous glucose requirement, high insulin to glucose ratio, negative urinary ketones. Normoglycemia was maintained by a combined treatment including glucose infusions and steroid, and then somatostatin analoques. The patient was assessed neurologically and radiologically by sequential cerebral MRI within 2 years follow-up. Results The most striking findings were cystic lesions on corona radiata, parietooccipital deep white matter and diffuse subcortical involvement of the brain at the initial MRI. The lesions were recovered radiologically at the 5 months of age. Diffuse hyperintensity of the periventricular white matter and optic radiation suggests abnormal and delayed myelination at 1 year of age, and periventricular leukomalacia and ventricular irregularity at 2 years of age. More delayed neurologic sequelae included mental retardation and spasticity. Conclusion Neonatal hyperinsulinemic hypoglycemia must be suddenly and appropriately diagnosed and treated to prevent any further neurological dysfunction and damage. MRI studies are crucial in nesidioblastosis to define the characteristics and severity of cerebral lesions after hypoglycemia. Long-term radiologic follow-up should be further investigated to predict the neurologic outcome, although the radiologic recovery period was seen in acute or subacute phase of the disease.