Evidence of Late-Onset Infantile Spasms
Article first published online: 3 AUG 2005
Volume 39, Issue 1, pages 55–60, January 1998
How to Cite
Bednarek, N., Motte, J., Soufflet, C., Plouin, P. and Dulac, O. (1998), Evidence of Late-Onset Infantile Spasms. Epilepsia, 39: 55–60. doi: 10.1111/j.1528-1157.1998.tb01274.x
- Issue published online: 3 AUG 2005
- Article first published online: 3 AUG 2005
- Accepted May 21, 1997.
- Childhood epilepsy;
- Infantile spasms;
- West syndrome;
- Behavioral impairment
Summary: Purpose: To underline the unusual but possible Occurrence of epileptic spasms (ES) in children >1 year of age.
Methods: Cases in whom onset of spasms occurred after 1 year of age were identified through a retrospective review of the records of all patients referred for ES to the Saint-Vincent de Paul Hôpital (Paris) and American Memorial Hospital (Reims) between 1974 and 1994.
Results: Eighteen cases were identified among the 734 children referred for ES, 18 cases were identified where spasm onset time ranged from 12 to 38 months of age. In 1/3 of the cases, the diagnosis was suspected from the onset of clinical manifestations; in the remaining 2/3, diagnosis was delayed by a mean 6 months (range, 2–25 months). Neurobehavioral regression affected two-thirds of the patients. Modified hypsarrhythmia was present in 11 patients; all but one exhibited major and diffuse spike- and slow-wave activity. EEG abnormalities were detected in the frontal areas in 11 patients. Spasms were cryptogenic in 9 patients. Steroids were administered to 13 patients; these controlled the spasms in 6 patients. Outcome was favorable for both seizures; cognition favorable in only 2 of the 18 patients.
Conclusions: Beginning after the first year of life, ES, or late-onset infantile spasms, are distinct from early Lennox-Gastaut syndrome, although etiology, prognosis and treatment are similar to that for the classical infantile spasms.