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Neuropediatrics 2020; 51(01): 030-036
DOI: 10.1055/s-0039-1698450
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Does Co-occurred Cerebral Palsy Change the Prognosis of West Syndrome?

Eszter Nagy 
1   Department of Pediatrics, Clinical Center, University of Pécs, Medical School, Pécs, Hungary
,
Nelli Farkas
2   Institute of Bioanalysis, University of Pécs, Medical School, Pécs, Hungary
,
Katalin Hollódy
1   Department of Pediatrics, Clinical Center, University of Pécs, Medical School, Pécs, Hungary
› Author Affiliations
Further Information

Publication History

17 April 2019

26 August 2019

Publication Date:
22 October 2019 (online)

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Abstract

Aim We aimed to examine the occurrence of cerebral palsy (CP) in children with West syndrome (WS), to estimate the possible causative factors by analyzing the neuroimaging examinations of patients, to evaluate their cognitive/motor function and epileptic status and to compare the prognosis of children with double pathology of WS and CP and of those without CP.

Methods The clinical and magnetic resonance imaging (MRI) data of 62 patients with West syndrome were evaluated. A total of 39 of 62 patients (63%) suffered from CP (CP group). The non-CP group included 23 patients.

Results Abnormal MRI was found in 55/62 (89%) patients. Main anomalies were: brain malformation (21), hypoxic-ischemic encephalopathy (13), cerebrovascular insult (8), infection (7), and other anomalies (6). In the CP group, the most common MRI abnormalities included pre/perinatal hypoxia/ischemia, brain malformation, cerebrovascular insult, and infection. In the non-CP group, brain malformations were the most frequent. Significantly more negative MRIs were found in the non-CP group. More than 60% of the patients were severely cognitively impaired, almost 90% of them had CP. Not only the occurrence of intellectual disability was lower in the non-CP group, but its severity was milder as well. A total of 78% of the children with CP had a very severe motor disability. Fifty-four percent in the CP and 67% in the non-CP group had therapy-resistant epilepsy.

Conclusion WS has an especially unfavorable prognosis: cerebral anomaly was confirmed in 89% of our patients. CP was present in almost two-thirds of the children with WS, most of them had severe cognitive and motor deficits.

Keywords

West syndrome - infantile spasm - cerebral palsy - etiology - MRI - walking ability
 

  • References

  • 1 D'Alonzo R, Rigante D, Mencaroni E, Esposito S. West syndrome: a review and guide for paediatricians. Clin Drug Investig 2018; 38 (02) 113-124
    CrossrefPubMedGoogle Scholar
  • 2 Pavone P, Striano P, Falsaperla R, Pavone L, Ruggieri M. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013. Brain Dev 2014; 36 (09) 739-751
    CrossrefPubMedGoogle Scholar
  • 3 West WJ. On a peculiar form of infantile convulsions. Lancet 1841; 35: 724-725
    CrossrefPubMedGoogle Scholar
  • 4 Cans C, Dolk H, Platt MJ, Colver A, Prasauskiene A, Krägeloh-Mann I. ; SCPE Collaborative Group. Recommendations from the SCPE collaborative group for defining and classifying cerebral palsy. Dev Med Child Neurol Suppl 2007; 109: 35-38
    PubMedGoogle Scholar
  • 5 Himmelmann K, Uvebrant P. The panorama of cerebral palsy in Sweden. XI. Changing patterns in the birth-year period 2003-2006. Acta Paediatr 2014; 103 (06) 618-624
    CrossrefPubMedGoogle Scholar
  • 6 Himmelmann K, Uvebrant P. The panorama of cerebral palsy in Sweden part XII shows that patterns changed in the birth years 2007-2010. Acta Paediatr 2018; 107 (03) 462-468
    CrossrefPubMedGoogle Scholar
  • 7 Pellock JM, Hrachovy R, Shinnar S. , et al. Infantile spasms: a U.S. consensus report. Epilepsia 2010; 51 (10) 2175-2189
    CrossrefPubMedGoogle Scholar
  • 8 Jeavons PM, Harper JR, Bower BD. Long-term prognosis in infantile spasms: a follow-up report on 112 cases. Dev Med Child Neurol 1970; 12 (04) 413-421
    PubMedGoogle Scholar
  • 9 Lombroso CT. A prospective study of infantile spasms: clinical and therapeutic correlations. Epilepsia 1983; 24 (02) 135-158
    CrossrefPubMedGoogle Scholar
  • 10 Watanabe K, Takeuchi T, Hakamada S, Hayakawa F. Neurophysiological and neuroradiological features preceding infantile spasms. Brain Dev 1987; 9 (04) 391-398
    CrossrefPubMedGoogle Scholar
  • 11 LúôAvígsson P, Ólafsson E, Sigurôardóttir S, Hauser WA. Epidemiologic features of infantile spasms in Iceland. Epilepsia 1994; 35 (04) 802-805
    CrossrefPubMedGoogle Scholar
  • 12 Trevathan E, Murphy CC, Yeargin-Allsopp M. The descriptive epidemiology of infantile spasms among Atlanta children. Epilepsia 1999; 40 (06) 748-751
    CrossrefPubMedGoogle Scholar
  • 13 Wong V. West syndrome—the University of Hong Kong experience (1970-2000). Brain Dev 2001; 23 (07) 609-615
    CrossrefPubMedGoogle Scholar
  • 14 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
  • 15 Harini C, Sharda S, Bergin AM. , et al. Detailed magnetic resonance imaging (MRI) analysis in infantile spasms. J Child Neurol 2018; 33 (06) 405-412
    CrossrefPubMedGoogle Scholar
  • 16 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
  • 17 Riikonen R. Long-term outcome of West syndrome: a study of adults with a history of infantile spasms. Epilepsia 1996; 37 (04) 367-372
    CrossrefPubMedGoogle Scholar
  • 18 Riikonen RS. Favourable prognostic factors with infantile spasms. Eur J Paediatr Neurol 2010; 14 (01) 13-18
    CrossrefPubMedGoogle Scholar
  • 19 Poulat AL, Lesca G, Sanlaville D. , et al. A proposed diagnostic approach for infantile spasms based on a spectrum of variable aetiology. Eur J Paediatr Neurol 2014; 18 (02) 176-182
    CrossrefPubMedGoogle Scholar
  • 20 Wirrell EC, Shellhaas RA, Joshi C, Keator C, Kumar S, Mitchell WG. ; Pediatric Epilepsy Research Consortium. How should children with West syndrome be efficiently and accurately investigated? Results from the National Infantile Spasms Consortium. Epilepsia 2015; 56 (04) 617-625
    CrossrefPubMedGoogle Scholar
  • 21 Sellier E, Uldall P, Calado E. , et al. Epilepsy and cerebral palsy: characteristics and trends in children born in 1976-1998. Eur J Paediatr Neurol 2012; 16 (01) 48-55
    CrossrefPubMedGoogle Scholar
  • 22 Reid SM, Meehan EM, Arnup SJ, Reddihough DS. Intellectual disability in cerebral palsy: a population-based retrospective study. Dev Med Child Neurol 2018; 60 (07) 687-694
    CrossrefPubMedGoogle Scholar
  • 23 Hollung SJ, Vik T, Lydersen S, Bakken IJ, Andersen GL. Decreasing prevalence and severity of cerebral palsy in Norway among children born 1999 to 2010 concomitant with improvements in perinatal health. Eur J Paediatr Neurol 2018; 22 (05) 814-821
    CrossrefPubMedGoogle Scholar
  • 24 Palisano R, Rosenbaum P, Walter S, Russell D, Wood E, Galuppi B. Development and reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol 1997; 39 (04) 214-223
    PubMedGoogle Scholar