A case with congenital hydrocephaly and West syndrome who recovered from hypsarrhythmia after the resolution of shunt trouble

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.