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Journal of Pediatric Epilepsy 2020; 09(01): 001-006
DOI: 10.1055/s-0040-1708809
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Outcome of West Syndrome: A Critical Review

Sudhir Adhikari
1   Department of Paediatrics, Chitwan Medical College, Bharatpur, Nepal
,
Deepa Gautam
2   Department of Radiation Oncology, BPKMCH, Chitwan, Nepal
› Author Affiliations
Further Information

Publication History

26 December 2019

20 February 2020

Publication Date:
04 April 2020 (online)

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Abstract

West syndrome is a genetically heterogeneous electro-clinical syndrome starting in early infancy. Short-term goal of therapy is spasm control and with standard hormonal or vigabatrin treatment, spasms can be controlled in 60 to 80% of patients in 2 weeks to 3 months period. Hormonal treatment with oral steroid is an alternative therapy to injectable adrenocorticotropin hormone, especially in low resource areas. Vigabatrin is preferred in tuberous sclerosis patients. Long-term aim of treatment is sustained remission of seizures and better neurodevelopmental outcome. About 50 to 70% of children are spasm free for prolonged duration, but epilepsy with multiple seizure types including Lennox–Gastaut syndrome is evident in 20 to 40% of children in long-term follow-up. Though hypsarrhythmia is helpful for the diagnosis, prognostic role of the resolution of electroencephalographic abnormalities is still uncertain. Seizures can be controlled in 40 to 60% of the patients, but only 12 to 40% children have normal neurodevelopmental outcome and a third of children are left with severe disabilities. Children with unknown etiology and normal development at spasm onset have better clinical outcome. Young age at onset, nonstandard therapy have less favorable outcome. Surgery is helpful for spasm control in patients with structural lesions and refractory spasms even in the absence of structural lesions.

Keywords

epileptic spasm - infantile seizures - infantile spasm

Note

Authors confirm that they have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.


 

  • References

  • 1 Lúthvígsson P, Olafsson E, Sigurthardóttir S, Hauser WA. Epidemiologic features of infantile spasms in Iceland. Epilepsia 1994; 35 (04) 802-805
    CrossrefPubMedGoogle Scholar
  • 2 Hrachovy RA, Frost Jr JD. Infantile spasms. Handb Clin Neurol 2013; 111: 611-618
    PubMedGoogle Scholar
  • 3 Wilmshurst JM, Gaillard WD, Vinayan KP. , et al. Summary of recommendations for the management of infantile seizures: task force report for the ILAE commission of pediatrics. Epilepsia 2015; 56 (08) 1185-1197
    CrossrefPubMedGoogle Scholar
  • 4 Fisher RS, Cross JH, D'Souza C. , et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia 2017; 58 (04) 531-542
    CrossrefPubMedGoogle Scholar
  • 5 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 (11) 1416-1428
    CrossrefPubMedGoogle Scholar
  • 6 Güveli BT, Çokar Ö, Dörtcan N, Benbir G, Demirbilek V, Dervent A. Long-term outcomes in patients with West syndrome: an outpatient clinical study. Seizure 2015; 25: 68-71
    CrossrefPubMedGoogle Scholar
  • 7 Djuric M, Kravljanac R, Tadic B, Mrlješ-Popovic N, Appleton RE. Long-term outcome in children with infantile spasms treated with vigabatrin: a cohort of 180 patients. Epilepsia 2014; 55 (12) 1918-1925
    CrossrefPubMedGoogle Scholar
  • 8 Fujii A, Oguni H, Hirano Y, Shioda M, Osawa M. A long-term, clinical study on symptomatic infantile spasms with focal features. Brain Dev 2013; 35 (05) 379-385
    CrossrefPubMedGoogle Scholar
  • 9 Lagae L, Verhelst H, Ceulemans B. , et al. Treatment and long term outcome in West syndrome: the clinical reality. A multicentre follow up study. Seizure 2010; 19 (03) 159-164
    CrossrefPubMedGoogle Scholar
  • 10 Perret EV, von Elm E, Lienert C, Steinlin M. Infantile spasms: does season influence onset and long-term outcome?. Pediatr Neurol 2010; 43 (02) 92-96
    CrossrefPubMedGoogle Scholar
  • 11 Riikonen R. Epidemiological data of West syndrome in Finland. Brain Dev 2001; 23 (07) 539-541
    CrossrefPubMedGoogle Scholar
  • 12 Gulati S, Jain P, Kannan L, Sehgal R, Chakrabarty B. The clinical characteristics and treatment response in children with West syndrome in a developing country: a retrospective case record analysis. J Child Neurol 2015; 30 (11) 1440-1447
    CrossrefPubMedGoogle Scholar
  • 13 Riikonen R. A long-term follow-up study of 214 children with the syndrome of infantile spasms. Neuropediatrics 1982; 13 (01) 14-23
    Article in Thieme ConnectPubMedGoogle Scholar
  • 14 King DW, Dyken PR, Spinks Jr IL, Murvin AJ. Infantile spasms: ictal phenomena. Pediatr Neurol 1985; 1 (04) 213-218
    CrossrefPubMedGoogle Scholar
  • 15 Alrifai MT, AlShaya MA, Abulaban A, Alfadhel M. Hereditary neurometabolic causes of infantile spasms in 80 children presenting to a tertiary care center. Pediatr Neurol 2014; 51 (03) 390-397
    CrossrefPubMedGoogle Scholar
  • 16 Muir AM, Myers CT, Nguyen NT. , et al; EuroEPINOMICS-RES NLES working group, Sarah Weckhuysen. Genetic heterogeneity in infantile spasms. Epilepsy Res 2019; 156: 106181
    CrossrefPubMedGoogle Scholar
  • 17 Michaud JL, Lachance M, Hamdan FF. , et al. The genetic landscape of infantile spasms. Hum Mol Genet 2014; 23 (18) 4846-4858
    CrossrefPubMedGoogle Scholar
  • 18 Berg AT, Chakravorty S, Koh S. , et al. Why West? Comparisons of clinical, genetic and molecular features of infants with and without spasms. PLoS One 2018; 13 (03) e0193599
    CrossrefPubMedGoogle Scholar
  • 19 Mohamed BP, Scott RC, Desai N, Gutta P, Patil S. Seizure outcome in infantile spasms--a retrospective study. Epilepsia 2011; 52 (04) 746-752
    CrossrefPubMedGoogle Scholar
  • 20 Karvelas G, Lortie A, Scantlebury MH, Duy PT, Cossette P, Carmant L. A retrospective study on aetiology based outcome of infantile spasms. Seizure 2009; 18 (03) 197-201
    CrossrefPubMedGoogle Scholar
  • 21 Demarest ST, Shellhaas RA, Gaillard WD. , et al; Pediatric Epilepsy Research Consortium. The impact of hypsarrhythmia on infantile spasms treatment response: observational cohort study from the National Infantile Spasms Consortium. Epilepsia 2017; 58 (12) 2098-2103
    CrossrefPubMedGoogle Scholar
  • 22 Yamada K, Toribe Y, Kimizu T. , et al. Predictive value of EEG findings at control of epileptic spasms for seizure relapse in patients with West syndrome. Seizure 2014; 23 (09) 703-707
    CrossrefPubMedGoogle Scholar
  • 23 Werner K, Fosi T, Boyd SG, Baldeweg T, Scott RC, Neville BG. Temporal lobe impairment in West syndrome: event-related potential evidence. Ann Neurol 2015; 77 (01) 47-57
    CrossrefPubMedGoogle Scholar
  • 24 Davis PE, Kapur K, Filip-Dhima R. , et al; Tuberous Sclerosis Autism Centers of Excellence Research Network. Increased electroencephalography connectivity precedes epileptic spasm onset in infants with tuberous sclerosis complex. Epilepsia 2019; 60 (08) 1721-1732
    CrossrefPubMedGoogle Scholar
  • 25 Spenner B, Krois-Neudenberger J, Kurlemann G, Althaus J, Schwartz O, Fiedler B. The prognostic value of sleep spindles in long-term outcome of West Syndrome. Eur J Paediatr Neurol 2019; 23 (06) 827-831
    CrossrefPubMedGoogle Scholar
  • 26 Kobayashi K, Akiyama T, Oka M, Endoh F, Yoshinaga H. A storm of fast (40-150Hz) oscillations during hypsarrhythmia in West syndrome. Ann Neurol 2015; 77 (01) 58-67
    CrossrefPubMedGoogle Scholar
  • 27 Hancock EC, Osborne JP, Edwards SW. Treatment of infantile spasms. Cochrane Database Syst Rev 2013; 6 (06) CD001770
    Google Scholar
  • 28 Lux AL, Edwards SW, Hancock E. , et al. The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomised controlled trial. Lancet 2004; 364 (9447): 1773-1778
    CrossrefPubMedGoogle Scholar
  • 29 O'Callaghan FJ, Edwards SW, Alber FD. , et al; participating investigators. Safety and effectiveness of hormonal treatment versus hormonal treatment with vigabatrin for infantile spasms (ICISS): a randomised, multicentre, open-label trial. Lancet Neurol 2017; 16 (01) 33-42
    CrossrefPubMedGoogle Scholar
  • 30 Wanigasinghe J, Arambepola C, Sri Ranganathan S, Sumanasena S, Attanapola G. Randomized, single-blind, parallel clinical trial on efficacy of oral prednisolone versus intramuscular corticotropin on immediate and continued spasm control in west syndrome. Pediatr Neurol 2015; 53 (03) 193-199
    CrossrefPubMedGoogle Scholar
  • 31 Raga SV, Wilmshurst JM. Epileptic spasms: evidence for oral corticosteroids and implications for low and middle income countries. Seizure 2018; 59: 90-98
    CrossrefPubMedGoogle Scholar
  • 32 Chang YH, Chen C, Chen SH, Shen YC, Kuo YT. Effectiveness of corticosteroids versus adrenocorticotropic hormone for infantile spasms: a systematic review and meta-analysis. Ann Clin Transl Neurol 2019; 6 (11) 2270-2281
    CrossrefPubMedGoogle Scholar
  • 33 O'Callaghan FJ, Lux AL, Darke K. , et al. The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study. Epilepsia 2011; 52 (07) 1359-1364
    CrossrefPubMedGoogle Scholar
  • 34 Yuskaitis CJ, Ruzhnikov MRZ, Howell KB. , et al. Infantile spasms of unknown cause: predictors of outcome and genotype-phenotype correlation. Pediatr Neurol 2018; 87 (87) 48-56
    CrossrefPubMedGoogle Scholar
  • 35 Wanigasinghe J, Arambepola C, Ranganathan SS, Sumanasena S. Randomized, single-blind, parallel clinical trial on efficacy of oral prednisolone versus intramuscular corticotropin: a 12-month assessment of spasm control in West syndrome. Pediatr Neurol 2017; 76: 14-19
    CrossrefPubMedGoogle Scholar
  • 36 Lux AL, Edwards SW, Hancock E. , et al. United Kingdom Infantile Spasms Study, The United Kingdom Infantile Spasms Study (UKISS) comparing hormone treatment with vigabatrin on developmental and epilepsy out-comes to age 14 months: a multicentre randomised trial. Lancet Neurol 2005; 4: 712-717
    CrossrefPubMedGoogle Scholar
  • 37 Yum MS, Lee EH, Ko TS. Vigabatrin and mental retardation in tuberous sclerosis: infantile spasms versus focal seizures. J Child Neurol 2013; 28 (03) 308-313
    CrossrefPubMedGoogle Scholar
  • 38 Yi Z, Wu H, Yu X. , et al. High-dose prednisone therapy for infantile spasms and late-onset epileptic spasms in China: The addition of topiramate provides no benefit. Seizure 2019; 71: 174-178
    CrossrefPubMedGoogle Scholar
  • 39 Hussain SA, Zhou R, Jacobson C. , et al. Perceived efficacy of cannabidiol-enriched cannabis extracts for treatment of pediatric epilepsy: a potential role for infantile spasms and Lennox-Gastaut syndrome. Epilepsy Behav 2015; 47: 138-141
    CrossrefPubMedGoogle Scholar
  • 40 Tang-Wai R, Mailo J, Rosenblatt B. Breaking the cycle: a comparison between intravenous immunoglobulins and high dosage prednisone in the treatment of medically intractable epilepsy in children. Seizure 2017; 47: 34-41
    CrossrefPubMedGoogle Scholar
  • 41 Kossoff EH, Hedderick EF, Turner Z, Freeman JM. A case-control evaluation of the ketogenic diet versus ACTH for new-onset infantile spasms. Epilepsia 2008; 49 (09) 1504-1509
    CrossrefPubMedGoogle Scholar
  • 42 Hussain SA, Shin JH, Shih EJ. , et al. Limited efficacy of the ketogenic diet in the treatment of highly refractory epileptic spasms. Seizure 2016; 35: 59-64
    CrossrefPubMedGoogle Scholar
  • 43 Iwatani Y, Kagitani-Shimono K, Tominaga K. , et al. Long-term developmental outcome in patients with West syndrome after epilepsy surgery. Brain Dev 2012; 34 (09) 731-738
    CrossrefPubMedGoogle Scholar
  • 44 Ostrowsky-Coste K, Neal A, Guenot M. , et al. Resective surgery in tuberous Sclerosis complex, from Penfield to 2018: a critical review. Rev Neurol (Paris) 2019; 175 (03) 163-182
    CrossrefPubMedGoogle Scholar
  • 45 Baba H, Toda K, Ono T, Honda R, Baba S. Surgical and developmental outcomes of corpus callosotomy for West syndrome in patients without MRI lesions. Epilepsia 2018; 59 (12) 2231-2239
    CrossrefPubMedGoogle Scholar
  • 46 Rantala H, Putkonen T. Occurrence, outcome, and prognostic factors of infantile spasms and Lennox-Gastaut syndrome. Epilepsia 1999; 40 (03) 286-289
    CrossrefPubMedGoogle Scholar
  • 47 Maguire MJ, Hemming K, Wild JM, Hutton JL, Marson AG. Prevalence of visual field loss following exposure to vigabatrin therapy: a systematic review. Epilepsia 2010; 51 (12) 2423-2431
    CrossrefPubMedGoogle Scholar
  • 48 Riikonen R, Rener-Primec Z, Carmant L. , et al. Does vigabatrin treatment for infantile spasms cause visual field defects? An international multicentre study. Dev Med Child Neurol 2015; 57 (01) 60-67
    PubMedGoogle Scholar
  • 49 Nagy E, Farkas N, Hollódy K. Does co-occurred cerebral palsy change the prognosis of west syndrome?. Neuropediatrics 2020; 51 (01) 30-36
    Article in Thieme ConnectPubMedGoogle Scholar
  • 50 Darke K, Edwards SW, Hancock E. , et al; trial steering committee on behalf of participating investigators. Developmental and epilepsy outcomes at age 4 years in the UKISS trial comparing hormonal treatments to vigabatrin for infantile spasms: a multi-centre randomised trial. Arch Dis Child 2010; 95 (05) 382-386
    CrossrefPubMedGoogle Scholar
  • 51 Autry AR, Trevathan E, Van Naarden Braun K, Yeargin-Allsopp M. Increased risk of death among children with Lennox-Gastaut syndrome and infantile spasms. J Child Neurol 2010; 25 (04) 441-447
    CrossrefPubMedGoogle Scholar
  • 52 Sillanpää M, Riikonen R, Saarinen MM, Schmidt D. Long-term mortality of patients with West syndrome. Epilepsia Open 2016; 1 (1-2): 61-66
    CrossrefPubMedGoogle Scholar
  • 53 Kaushik JS, Patra B, Sharma S, Yadav D, Aneja S. Clinical spectrum and treatment outcome of West Syndrome in children from Northern India. Seizure 2013; 22 (08) 617-621
    CrossrefPubMedGoogle Scholar
  • 54 Jóźwiak S, Kotulska K, Domańska-Pakieła D. , et al. Antiepileptic treatment before the onset of seizures reduces epilepsy severity and risk of mental retardation in infants with tuberous sclerosis complex. Eur J Paediatr Neurol 2011; 15 (05) 424-431
    CrossrefPubMedGoogle Scholar