Prognostic value of EEG in West syndrome

Dimitrije M. Nikolić; Ivan D. Milovanović; Biljana P. Medjo; Marina Atanasković; Petar I. Ivanovski; Ana V. Nikolić

doi:10.2298/VSP160214384N

Dimitrije M. Nikolić University Children's Hospital, Belgrade, Serbia
Ivan D. Milovanović University Children's Hospital, Belgrade, Serbia
Biljana P. Medjo University Children's Hospital, Belgrade, Serbia
Marina Atanasković University Children's Hospital, Belgrade, Serbia
Petar I. Ivanovski University Children's Hospital, Belgrade, Serbia
Ana V. Nikolić University of Belgrade, Faculty of Medicine, Belgrade, Serbia; Clinical Center of Serbia, Neurology Clinic, Belgrade, Serbia

DOI: https://doi.org/10.2298/VSP160214384N

Keywords: electroencephalography;, prognosis;, treatment outcome;, spasm;, infant, newborn;, epilepsy.

Abstract

Background/Aim. West syndrome (WS) is an epileptic encephalopathy which is characterized by the trias: infantile spasms, psychomotor delay and specific electroencephalography (EEG) pattern. The aim of this study was to determine the prognostic value of EEG in the therapy of West syndrome. Methods. This study group comprised 68 patients (40 boys and 28 girls) with the diagnosis of WS. Criteria for inclusion of patients in this study were the disease onset in the first or the second year of life, specific seizure type and a characteristic EEG pattern. All patients were divided into 2 groups: symptomatic (37 patients) and cryptogenic (31 patients) WS. The outcome was assessed through the response to the therapy (seizure control and EEG findings). Follow-up was at 3, 6, 12 and 24 months after the diagnosis was established. Results. Three months after starting the treatment 80.6% of patients with improved EEG were seizure free (p < 0.01); 85.7% of patients with EEG improvement at 3 months check-up were seizure free after 6 months (p < 0.01); 82.8% of patients with better EEG findings after 3 months had no seizures after 12 months (p < 0.05). Also, the majority of patients with improvement in EEG at 6 month follow-up (95.8%) had no seizures at one year follow-up (p < 0.01). The presence of seizures during this period did not depend on EEG after 6 months of treatment (p > 0.05). Most of the patients with improved (89.7%) and unchanged (70.6%) EEG after 12 months had no seizures after two years, whereas the patients with worsened EEG were with seizures. Conclusion. Seizure control after 6, 12 and 24 months depended on EEG finding at 3 months follow up. Seizure control after 12 months correlated with EEG after 6 months. The correlation between EEG after 12 months and seizure control after 24 months was not clear. EEG at 6 months follow-up did not affect seizure control after 2 years.

References

Johnston M. Generalized seizures. In: Behrman RE, Klieg¬man R, Jenson J, editors. Nelson textbook of pediatrics. Philadelphia: W. B. Saunders; 2007. p. 2462‒4.

Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross HJ, Emde BW, et al. Revised terminology and concepts for or¬ganization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia 2010; 51(4): 676‒85.

Arzimanoglou A, Guerrini R, Aicardi J. Aicardi's epilepsy in children. 3rd ed. USA: Philadephia: Lippincot Wiliams & Wil-kins; 2004.

Panayiotopoulos CP. The Epilepsies. Seizures, Syndromes and Management. Oxfordshire (UK): Bladon Medical Publishing; 2005.

Engel J Jr. ILAE classification of epilepsy syndromes. Epi¬lepsy Res 2006; 70 Suppl 1: S5‒10.

Dulac O, Plouin P, Jambaque I. Predicting Favorable Out¬come in Idiopathic West Syndrome. Epilepsia 1993; 34(4): 747‒56.

Cvitanović-Sojat L, Gjergja R, Sabol Z, Hajnzić TF, Sojat T. Treatment of West syndrome. Acta Med Croatica 2005; 59(1): 19‒29. (Croatian)

Shields WD. Infantile Spasms: Little Seizures, BIG Conse-quences. Epilepsy Curr 2006; 6(3): 63‒9.

Cohen-Sadan S, Kramer U, Ben-Zeev B, Lahat E, Sahar E, Nevo Y, et al. Multicenter long-term follow-up of children with idi-opathic West syndrome: ACTH versus vigabatrin. Eur J Neu-rol 2009; 16(4): 482‒7.

Fois A. Infantile spasms: Review of the literature and per¬sonal experience. Italian J Pediatr 2010; 36: 1‒10.

Go CY, Mackay MT, Weiss SK, Stephens D, Adams-Webber T, Ashwal S, et al. Evidence-based guideline update: Medi¬cal treatment of infantile spasms: Report of the Guideline Devel-opment Subcommittee of the American Academy of Neurolo-gy and the Practice Committee of the Child Neu¬rology Socie-ty. Neurology 2012; 78(24): 1974‒80.

Hrachovy RA, Frost JD. Severe Encephalopathic Epilepsy in In-fants: Infantile Spasms (West Syndrome). In: Pellock JM, Bour-geois B, Dodson WE, editors. Pediatric Epilepsy - Diagnosis and treatment. 3rd ed. New York: Demos; 2008. p. 249‒69.

Kotagal P. Multifocal independent Spike syndrome: Rela-tionship to hypsarrhythmia and the slow spike-wave (Lennox-Gastaut) syndrome. Clin Electroencephalogr 1995; 26(1): 23‒9.

Riikonen R. Infantile spasms: Modern practical aspects. Acta Paediatr Scand 1984; 73(1): 1‒12.

Vigevano F, Cilio MR. Vigabatrin versus ACTH as first-line treatment for infantile spasms: A randomized, pro¬spective study. Epilepsia 1997; 38(12): 1270‒4.

Jeavons PM. West syndrome: Infantile spasms. In: Roger J, Dra-vet C, Bureau M, Dreifuss FE, Wolf P, editors. Epileptic syn-dromes in infancy, Childhood and Adolescence. Lon¬don: John Libbey; 1985. p. 42‒50.

Riikonen R. Infantile spasms: Therapy and outcome. J Child Neurol 2004; 19(6): 401‒4.

Mackay MT, Weiss SK, Adams-Webber T, Ashwal S, Stephens D, Ballaban-Gill K, et al. Practice parameter: medical treatment of infantile spasms: report of the American Academy of Neurol-ogy and the Child Neurology Society. Neurology 2004; 62(10): 1668‒81.