Infantile spasms: A prognostic evaluation

Mary Iype, Geetha Saradakutty, Puthuvathra Abdul Mohammed Kunju, Devi Mohan, Muttathu Krishnapanicker Chandrasekharan Nair, Babu George, Shahanaz M Ahamed, Mary Iype, Geetha Saradakutty, Puthuvathra Abdul Mohammed Kunju, Devi Mohan, Muttathu Krishnapanicker Chandrasekharan Nair, Babu George, Shahanaz M Ahamed

Abstract

Background: Few papers address the comprehensive prognosis in infantile spasms and look into the seizure profile and psychomotor outcome.

Objective: We aimed to follow up children with infantile spasms to study: a) the etiology, demographics, semiology, electroencephalogram (EEG), and radiological pattern; b) seizure control, psychomotor development, and EEG resolution with treatment; c) the effects of various factors on the control of spasms, resolution of EEG changes, and psychomotor development at 3-year follow-up.

Materials and methods: Fifty newly diagnosed cases with a 1-12 month age of onset and who had hypsarrhythmia in their EEG were recruited and 43 were followed up for 3 years.

Results: Of the children followed up, 51% were seizure-free and 37% had a normal EEG at the 3-year follow-up. Autistic features were seen in 74% of the children. Only 22.7% among the seizure-free (11.6% of the total) children had normal vision and hearing, speech with narration, writing skills, gross and fine motor development, and no autism or hyperactivity. On multivariate analysis, two factors could predict bad seizure outcome - the occurrence of other seizures in addition to infantile spasms and no response to 28 days of adrenocorticotropic hormone (ACTH). No predictor could be identified for abnormal psychomotor development.

Discussion and conclusion: In our study, we could demonstrate two factors that predict seizure freedom. The cognitive outcome and seizure control in this group of children are comparable to the existing literature. However, the cognitive outcome revealed by our study and the survey of the literature are discouraging.

Keywords: Development; West syndrome; cognitive outcome; infantile spasms; outcome; prognosis; psychomotor development; seizure freedom.

References

    1. Holmes GL. Effects of early seizures on later behavior and epileptogenicity. Ment Retard Dev Disabil Res Rev. 2004;10:101–5.
    1. Lux AL, Osborne JP. A proposal for case definitions and outcome measures in studies of infantile spasms and west syndrome: Consensus statement of the West Delphi Group. Epilepsia. 2004;45:1416–28.
    1. Riikonen R. A long-term follow-up study of 214 children with the syndrome of infantile spasms. Neuropediatrics. 1982;13:14–23.
    1. Glaze DG, Hrachovy RA, Frost JD, Jr, Kellaway P, Zion TE. Prospective study of outcome of infants with infantile spasms treated during controlled studies of ACTH and prednisone. J Pediatr. 1988;112:389–96.
    1. Lombroso CT. A prospective study of infantile spasms: Clinical and therapeutic correlates. Epilepsia. 1983;24:135–58.
    1. Matsumoto A, Watanabe K, Negoro T, Sugiura M, Iwase K, Hara K, et al. Long-term prognosis after infantile spasms: A statistical study of prognostic factors in 200 cases. Dev Med Child Neurol. 1981;23:51–65.
    1. Blume WT, Lüders HO, Mizrahi E, Tassinari C, van Emde Boas W, Engel J., Jr Glossary of descriptive terminology for ictal semiology: Report of the ILAE task force on classification and terminology. Epilepsia. 2001;42:1212–8.
    1. Panayiotopoulos CP. Epileptic Encephalopathies in Infancy and Early Childhood in Which the Epileptiform Abnormalities May Contribute to Progressive Dysfunction. Oxfordshire (UK): Bladon Medical Publishing; 2005. The Epilepsies: Seizures, Syndromes and Management. Chapter 7.
    1. Pellock JM, Hrachovy R, Shinnar S, Baram TZ, Bettis D, Dlugos DJ, et al. Infantile spasms: A U. S. consensus report. Epilepsia. 2010;51:2175–89.
    1. Nair MK, George B, Philip E, Lekshmi MA, Haran JC, Sathy N. Trivandrum developmental screening chart. Indian Pediatr. 1991;28:869–72.
    1. Kumar N, Shekhar C, Kumar P, Kundu AS. Kuppuswamy's socioeconomic status scale-updating for 2007. Indian J Pediatr. 2007;74:1131–2.
    1. Phatak P, Misra N. Developmental Assessment Scales for Indian Infants (DASII)1-30 months – Revision of Baroda norms with indigenous material. Psychol Stud. 1996;41:55–6.
    1. Schopler E, Reichler RJ, DeVellis RF, Daly K. Toward objective classification of childhood autism: Childhood Autism Rating Scale (CARS) J Autism Dev Disord. 1980;10:91–103.
    1. Kivity S, Lerman P, Ariel R, Danziger Y, Mimouni M, Shinnar S. Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone. Epilepsia. 2004;45:255–62.
    1. Trevathan E, Murphy CC, Yeargin-Allsopp M. The descriptive epidemiology of infantile spasms among Atlanta children. Epilepsia. 1999;40:748–51.
    1. Zhu X, Chen O, Zhang D, Jin R, Li F, Wang Y, et al. A prospective study on the treatment of infantile spasms with first-line topiramate followed by low-dose ACTH. Epilepsy Res. 2011;93:149–54.
    1. Ibrahim S, Gulab S, Ishaque S, Saleem T. Clinical profile and treatment of infantile spasms using vigabatrin and ACTH--a developing country perspective. BMC Pediatr. 2010;10:1.
    1. Singhi P, Ray M. Profile of west syndrome in North Indian children. Brain Dev. 2005;27:135–40.
    1. Mohamed BP, Scott RC, Desai N, Gutta P, Patil S. Seizure outcome in infantile spasms-a retrospective study. Epilepsia. 2011;52:746–52.
    1. Go CY, Mackay MT, Weiss SK, Stephens D, Adams-Webber T, Ashwal S, et al. Child Neurology Society; American Academy of Neurology. Evidence-based guideline update: Medical treatment of infantile spasms Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2012;78:1974–80.
    1. Hrachovy RA, Frost JD., Jr Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/West syndrome) J Clin Neurophysiol. 2003;20:408–25.
    1. Lagae L, Verhelst H, Ceulemans B, De Meirleir L, Nassogne MC, De Borchgrave V, et al. Treatment and long term outcome in West syndrome: The clinical reality. A multicentre follow up study. Seizure. 2010;19:159–64.
    1. Saemundsen E, Ludvigsson P, Rafnsson V. Autism spectrum disorders in children with a history of infantile spasms: A population-based study. J Child Neurol. 2007;22:1102–7.
    1. Yanagaki S, Oguni H, Hayashi K, Imai K, Funatuka M, Tanaka T, et al. A comparative study of high-dose and low-dose ACTH therapy for West syndrome. Brain Dev. 1999;21:461–7.
    1. Baram TZ, Mitchell WG, Tournay A, Snead OC, Hanson RA, Horton EJ. High-dose corticotropin (ACTH) versus prednisone for infantile spasms: A prospective, randomized, blinded study. Pediatrics. 1996;97:375–9.
    1. Hrachovy RA, Frost JD, Jr, Glaze DG. High-dose, long-duration versus low-dose, short-duration corticotropin therapy for infantile spasms. J Pediatr. 1994;124:803–6.
    1. Sharma NL, Viswanathan V. Outcome in west syndrome. Indian Pediatr. 2008;45:559–63.
    1. Snead OC, 3rd, Benton JW, Myers GJ. ACTH and prednisone in childhood seizure disorders. Neurology. 1983;33:966–70.
    1. Riikonen R, Donner M. ACTH therapy in infantile spasms: Side effects. Arch Dis Child. 1980;55:664–72.

Source: PubMed

Sponsorer og samarbeidspartnere

Medisinsk tilstand

Legemiddelintervensjoner

3
Abonnere