Last modified: 2014-04-03
Abstract
Introduction:
The West Delphi group proposed a classification of infantile spasms (IS): IS in cluster with Hypsarhythmia (West syndrome), IS in cluster without hypsarrhythmia; IS single-spasms variant (ISSV) with hypsarrhytmia and ISSV without hypsarrhytmia.
Purpose:
To classify the IS registered in the video EEGs according to the Delphi West group proposal and analyze outcome.
Methods:
Observational retrospective study including 33 video-EEGs, between January 2010 and September 2013.
Results:
median age:6.1 months, 19 (57.5%) were female. According to the classification, we observed: 14 (42.4%) had West syndrome (WS), 10 (30.3%) IS in cluster without hypsarrhythmia, 7 (21.2%) ISSV with hypsarrhythmia, and 2 (6%)ISSV without hypsarrhythmia.
24 patients had symptomatic epilepsy and 7 cryptogenic.
EEG findings: 18 (63.6%) presented hypsarrhythmia, 24 (81.8%) multifocal paroxysms and 6 unifocal paroxysms. Fourteen of the 18 (77.7%) patients with hypsarrhythmia, had symptomatic epilepsy.
We analyzed 31 patients. 8 (25.8%), were preterm. 23 (74,2%) had a normal neurological exam before seizures started, 15 (49.5%) worsened developmental delay. One patient died.The median duration of IS was 4 months (IQR 1,5-9), 4 months in patients with hypsarrhythmia and 6.5 months, without hypsarrhythmia.
Outcome: 13 developed refractory epilepsy, 10/13 had symptomatic epilepsy and 10/13 had hypsarrhythmia.
12 out of 17 (70,5%) patients with hypsarrhythmia and 3/12 (25%) patients without hypsarrhythmia lost developmental skills. (p: 0.01).
Conclusions:
We observed a high frequency of patients with West syndrome and ISSV without hypsarrhythmia. Patients with or without hypsarrhythmia developed refractory epilepsy associated to developmental delay, being more frequent in patients with hypsarrhythmia.