Infantile spasms in remission may reemerge as intractable epileptic spasms.
BACKGROUND
West syndrome consists of infantile spasms with hypsarrhythmia and is perceived as a disorder of infants.


METHODS
We describe 10 patients with West syndrome with spasms that remitted, started again, and persisted (followed up for 8-25 years).


RESULTS
In all, West syndrome developed at younger than 17 months (five cryptogenic, six symptomatic). With initial treatment, spasms completely stopped for 4.5 months to 6 years, when epileptic spasms returned. Recurrent spasms were typical with brief arm extension, eye elevation, and head drop without falling. Spasms lasted 2-6 s in rhythmic strings over 20- to 60-min periods and occurred daily throughout follow-up. Persistent spasms were particularly troublesome, because of incontinence in one and postictal confusion in several. During the string of spasms, most refused to interact, and several would wander off. Up to 15 antiepileptic drugs did not render any patient spasm free. Only two had persistent spasms as the only seizure type; six also had intractable complex partial seizures, and three had occasional grand mal convulsions. Interictal EEGs showed multifocal spikes. Ictal recordings in six showed electrodecremental events.


CONCLUSIONS
Recurrent spasms after remission of West syndrome represent an extremely resistant, distressing form of epilepsy. The onset of West syndrome is age related, but it does not reliably vanish.
Camfield
Peter
P
Dalhousie University and the IWK Grace Health Centre, PO Box 3070, Halifax, Nova Scotia, Canada B3J 3G9. camfield@dal.ca
Camfield
Carol
C
Lortie
Anne
A
Darwish
Husam
H
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use*; D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use*; D001925:Brain Damage, Chronic / Q000175:diagnosis / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004351:Drug Resistance; D004569:Electroencephalography; D017029:Epilepsy, Complex Partial / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D004830:Epilepsy, Tonic-Clonic / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D012008:Recurrence; D013036:Spasms, Infantile / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology
D000305:Adrenal Cortex Hormones; D000927:Anticonvulsants; D000324:Adrenocorticotropic Hormone