Hydrocephalus occasionally causes
West syndrome, but the mechanism is unknown. We experienced a case with
West syndrome and congenital
hydrocephaly, in which the EEG findings improved after the resolution of shunt complications. The course of this case implied the pathogenesis of
West syndrome associated with congenital
hydrocephaly, as well as the origin of the
seizures and that of the EEG findings in
West syndrome. A 7-month-old girl had congenital
hydrocephaly. A prenatal diagnosis was made by ultrasonography, and ventricle-peritoneal shunting was performed 7 days after birth. During the following 7 months several shunt replacements were done because of recurrent shunt complications. Her first series of
infantile spasms began at the age of 6 months, and treatment was started under the diagnosis of
West syndrome. One month later, her
seizures were controlled by
pyridoxal phosphate, while the EEG still showed hypsarrythmia. Her shunt was then removed again, because of
bacterial meningitis due to shunt
infection. The recovery from shunt complication resulted in marked improvement of the
hypsarrhythmia. Our experience and previous literature suggest the involvement of cerebral cortex in the occurrence of
West syndrome associated with congenital
hydrocephaly.