Apneic Spells in a Patient with Myelomeningocele without Chiari Type II Malformation

A female neonate with myelomeningocele but without Chiari type II malformation suffered from apneic spells. Magnetic resonance imaging showed no obvious brainstem anomaly. Brainstem auditory evoked potentials were initially abnormal and subsequently deteriorated during the 6 months after birth. The brainstem deficits were not reversed by a ventriculoperitoneal shunt. She died of respiratory distress and cardiac failure at 2 years of age. This case indicates an intrinsic dysfunction in the brainstem of patients with myelomeningocele not complicated by Chiari type II malformation.