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Evidence of late-onset infantile spasms
N Bednarek1, J Motte, C Soufflet
1Unité de Neurologie Pédiatrique, American Memorial Hospital, Reims, France.
Insights
Late-onset epileptic spasms (ES) in children over one year old are uncommon but possible. These cases, distinct from early Lennox-Gastaut syndrome, share similar causes, outcomes, and treatments with typical infantile spasms.
Area of Science:
- Pediatric Neurology
- Epileptology
Background:
- Epileptic spasms (ES) typically manifest in infancy.
- Late-onset ES, occurring after 1 year of age, is an unusual presentation.
Purpose of the Study:
- To highlight the occurrence and characteristics of epileptic spasms with onset after one year of age.
- To differentiate late-onset ES from early Lennox-Gastaut syndrome.
Main Methods:
- Retrospective review of patient records for ES.
- Inclusion criteria: spasm onset after 12 months of age.
- Data collected from 1974-1994 at two French hospitals.
Main Results:
- Eighteen cases of late-onset ES identified (onset 12-38 months).
- Diagnosis delayed in 2/3 of cases; neurobehavioral regression in 2/3.
- EEG showed modified hypsarrhythmia in 11 patients; steroids effective in 6/13.
Conclusions:
- Late-onset ES is a distinct entity from early Lennox-Gastaut syndrome.
- Etiology, prognosis, and treatment of late-onset ES resemble classical infantile spasms.
- Favorable seizure control achieved in some patients, but cognitive outcomes were poor.
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.