Long‐term Prognosis after Infantile Spasms: a Statistical Study of Prognostic Factors in 200 Cases

Abstract

SUMMARY A follow‐up study was made on 200 children (115 boys, 85 girls) who had had infantile spasms, in order to compare their present condition over the age of six years with various prognostic factors. 48 of the children (30 males and 18 females) had died, and all the rest were aged six years or older at the time of final follow‐up. 139 of the children had received ACTH therapy: at final follow‐up, spasms had ceased in 43.5 per cent, and about the same proportion showed normal physical development; 23 per cent had normal mental development and 15.4 per cent were attending ordinary schools. Complete recovery (normal mental and physical development and attending ordinary schools) was achieved in only 19 cases (9.5 per cent). Of the cryptogenic cases, 44.4 per cent had made a full recovery. The poor prognostic factors for continuing seizures were evolution into other types of fits, relapse of seizures after ACTH therapy, seizures concomitant with spasms, and convulsions before the onset of spasms. Poor prognostic factors for physical development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, symptomatic aetiology, neonatal convulsions, low birthweight, perinatal asphyxia and being female. Poor prognostic factors for mental development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, prenatal and perinatal aetiology, relapse after initial ACTH therapy, laughing attacks, and evolution into other types of fits. Only in the cryptogenic cases was there significant correlation between the delay in treatment and the long‐term prognosis for mental development. Poor prognostic factors for educability were very similar to those for mental development. In spite of conflicting views as to the long‐term effects of ACTH, prompt treatment seems to be mandatory, at least in cryptogenic cases of infantile spasms. RÉSUMÉ Pronostic á long terme des spasmes infantiles: étude stalistique des facteurs pronostiques dans 200 cas Une étude longitudinale a été entreprise chez 115 garçons et 85 filles ayant eu des spasmes infantiles dans le but de comparer leur condition présente à l̂âge de six ans ou plus avec des facteurs pronostiques variés. 48 des enfants (30 garçons et 18 filles) sont morts, tous les autres étaient âgés de six ans au moins à la fin de l̂étude longitudinale. 139 des enfants avaient reçu un traitement d̂ACTH. A la fin de l̂étude, les spasmes avaient cessé dans 43,5 pour cent des cas et une proportion identique présentait un développement physique normal; 23 pour cent avaient un développement mental normal et 15,4 pour cent fréquentaient une école ordinaire. La ‘guérison complète’ (développement mental et physique normaux, fréquentation d̂une école ordinaire) n'était observée que dans 19 cas (9,5 pour cent). Parmi les cas d̂origine inconnue, 44,4 pour cent presentaient une guerison complète. Les facteurs de mauvais pronostic pour la continuation des crises étaient l̂évolution vers d̂autres types...

A long-term, clinical study on symptomatic infantile spasms with focal features

“…Among patients with IS and epilepsy with ES, a patient with a localized PET lesion or a combination of PET and MRI lesions was shown to be the most ideal candidate for resective surgery [13,15,18,24,25]. However, the natural course of patients with these epilepsies who had not responded to medical treatment was not sufficiently investigated before considering [5,7]. Kramer et al [10] identified 22 (33%) of 67 cases of patients with WS and more than two focal features, which was similar to our result (23/69 cases).…”

“…Seizures and intellectual prognoses are generally poor. In 20-30% of the patients, IS evolves to Lennox-Gastaut syndrome [4][5][6]. In the other 20-30% of the patients, IS either transforms into focal or multifocal epilepsy although only a few detailed studies on patients whose epilepsy evolved into focal epilepsy following IS have been documented [7][8][9].…”

Clinical Profile of Infants with Hypsarrhythmia

“…In the early 1980s, most studies found identified symptomatic etiologies in approximately 45-60% of patients (18). As seen in our study more recent studies have consistently classified 70-80% of patients into the symptomatic group (8).…”

Infantile Spasms

“…10 Several other studies have also commented on the improvement in development with early treatment, 53-55 although some have differentiated this response to occur only in those with cryptogenic infantile spasms. 56 The 2004 American Academy of Neurology/ Child Neurology Society Practice Parameter concluded that the evidence for early treatment was "conflicting" and "insufficient." 33 Despite this, the majority of pediatric epileptologists feel that children with new-onset infantile spasms, regardless of the etiology, should be evaluated, diagnosed, and treated as promptly as possible.…”