Persistence of Infantile Spasms into Mid-Childhood and Adult Life: A Nasty Addition to West Syndrome?
Abstract number :
3.052
Submission category :
Year :
2000
Submission ID :
2690
Source :
www.aesnet.org
Presentation date :
12/2/2000 12:00:00 AM
Published date :
Dec 1, 2000, 06:00 AM
Authors :
Peter R Camfield, Carol S Camfield, Husam Darwish, Annie Lortie, IWK Grace Health Ctr, Dalhousie Univ, Halifax, NS, Canada; IWK Grace Health Ctr, Halifax, NS, Canada; Univ of Alberta, Calgary, AB, Canada; Hosp Ste Justine, Montreal, PQ, Canada.
RATIONALE: West syndrome is defined as infantile spasms with hypsarrhythmia and is perceived as a disorder of infants only. METHODS: At 2 tertiary care pediatric centres, we have recognized and followed 10 patients with persistent spasms for an average of 15 years (range 8-25 years). RESULTS: The cause of infantile spasms was cryptogenic in 6 and symptomatic in 4. All were mentally handicapped. Eight developed typical West syndrome at age <1 year and two at >1 year. Of 8 treated with ACTH or corticosteroids, all had an excellent initial response. Spasms stopped completely in 8/10 for 5 months-6 years but then returned and were intractable with daily strings of spasms throughout follow up. When spasms reappeared, they were typical with brief arm extension, eye elevation, and head drop without falling. Individual spasms would last about 2-6 seconds and recur in rhythmic strings over 20-60 minutes. Persistent spasms were particularly troublesome because of incontinence in 1 and post-ictal confusion in several. During the string of spasms, most patients refused to interact with caretakers and often would wander off. Up to 15 AEDs did not render any patient spasm-free, although several medications decreased the frequency and severity of attacks (vigabatrin, nitrazepam, clonazepam, felbamate, topiramate). Only 2 had persistent spasms as the only seizure type; 8 also had intractable complex partial seizures beginning by the 2nd year of life and 3 had occasional convulsions persisting through follow up. When spasms persisted or started at >2 yrs of age, the interictal EEG showed multifocal spikes, rather than hypsarrhythmia. Ictal recordings in 5 cases showed electro-decremental events. CONCLUSIONS: Persistent spasms may be uncommon, but they represent an extremely resistant, distressing form of epilepsy that is not captured in the current restrictive definition of West syndrome. The onset of West syndrome may be age related with intial remission, but it does not reliably vanish - the spasms may return as an intractable epilepsy.