Periventricular leukomalacia and West syndrome

The authors studied the clinical course and electroencephalograms (EEGs) of 27 patients with periventricular leukomalacia (PVL), to investigate the relation between PVL and West syndrome. Seven of the 27 patients with PVL developed WS; in all seven the PVL was severe. Bilateral parieto-occipital dominant irregular polyspike-and-wave (PO-polySpW) bursts were seen in eight of the 21 patients whose EEGs were recorded within the age of one year (corrected for preterm birth). All eight had severe PVL, and in six, bilateral PO-polySpW bursts were followed by hypsarrhythmia and spasms. None of the 12 patients without polyspike-and-wave bursts developed West syndrome, the study shows that West syndrome is a common complication of severe PVL and that it correlates strongly with the occurrence of bilateral PO-polySpW bursts on follow-up EEGs.