Long-term mortality of patients with West syndrome

Abstract

<p>Objective: To study long-term survival and mortality among patients with West<br />syndrome.<br />Methods: The study population included all children born in 1960–1976 and treated for<br />West syndrome in three tertiary care hospitals in Helsinki, Finland. The participants<br />were prospectively followed for five decades for survival. Death data were derived<br />from the National Causes of Death Register of the Population Register Center of<br />Statistics Finland.<br />Results: During follow-up, 102 (49%) of 207 patients had died at the mean age of<br />19 years. The mean overall annual mortality rate was 15.3 per 1,000 patient-years.<br />The rates ranged from 18.2 per 1,000 after 10 years to 17.2 per 1,000 after 20 years<br />and 15.4 per 1,000 patient-years after 40 years of follow-up. One fourth (25%) had died<br />by 17.2 (95% CI 11.8–22.7) years and 50% by 48.6 (95% CI 38.5–NA) years of follow-up.<br />Etiology at onset was symptomatic in 87% patients and cryptogenic in 13%; 6 of the latter<br />26 patients later turned out to be symptomatic. The mean annual mortality rate<br />was 3.7 per 1,000 for 4 patients with cryptogenic etiology and 17.6 per 1,000 for those<br />with symptomatic etiology. The hazard of death was fivefold in patients with symptomatic<br />etiology versus cryptogenic etiology. The overall autopsy rate was 73%. Pneumonia<br />was the most frequent cause of death (46%). All patients who died of<br />pneumonia had symptomatic etiology. SUDEP occurred in 10 patients and was the<br />most common epilepsy-related cause of death (10%).<br />Significance: Risk of excess death of participants with West syndrome is not limited to<br />early age but continues into adulthood, particularly in those with symptomatic etiology,<br />and leads to death in half the cases at around 50 years of age. Measures should be<br />directed to prevent pneumonia, the most common overall cause, and SUDEP, the<br />most frequent seizure-related cause, of death.<br /></p>