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Seizure and developmental outcomes after hemispherectomy in children and adolescents with intractable epilepsy

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Abstract

Purpose

The aim of this study is to describe a series of pediatric hemispherectomies, reviewing pathologic substrate, epilepsy characteristics and seizure outcome as well as developmental profiles, before and after surgery, in different domains.

Methods

Seventeen patients with full pre-surgical work-up, minimum follow-up of 12 months, and at least one post-surgical neuropsychological evaluation were selected. Three had Rasmussen encephalitis (RE), five hemispheric malformations of cortical development (MCD), and nine hemispheric vascular lesions.

Results

At latest follow-up, all patients with RE and 66.7 % of those with vascular lesions are in Engel’s class I; in the latter group, pre-surgical independent contralateral EEG discharges statistically correlated with a worse seizure outcome. Patients with MCD showed the worst seizure outcome. Pre-surgical language transfer to the right hemisphere was confirmed in a boy with left RE, operated on at 6 years of age. Patients with MCD and vascular lesions already showed severe global developmental delay before surgery, which persists afterwards. A linear correlation was found between earlier age at surgery and better outcome in personal–social, gross motor, and adaptive domains, in the vascular lesions group. The case with highest cognitive improvement had continuous spike and wave during sleep on pre-surgical EEG.

Conclusions

Pathologic substrate was the main factor related with seizure outcome. In children with MCD and vascular lesions, although developmental progression is apparent, significant post-surgical improvements are restricted by the severity of pre-surgical neuropsychological disturbances and a slow maturation. Early surgery assessment is recommended to enhance the possibilities for a better quality of life in terms of seizure control, as well as better autonomy and socialization.

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References

  1. Rasmussen T (1983) Hemispherectomy for seizures revisited. Can J Neurol Sci 10:71–78

    PubMed  CAS  Google Scholar 

  2. Delalande O, Pinard JM, Basdevant C, Gauthe M, Plouin P, Dulac O (1992) Hemispherotomy: a new procedure for central disconnection. Epilepsia 33(Suppl 3):99–100

    Google Scholar 

  3. Villemure JG, Mascott CR (1995) Peri-insular hemispherotomy: surgical principles and anatomy. Neurosurgery 37:975–981

    Article  PubMed  CAS  Google Scholar 

  4. Smith JR, Fountas KN, Lee MR (2005) Hemispherotomy: description of surgical technique. Childs Nerv Syst 21(6):466–472

    Article  PubMed  Google Scholar 

  5. Delalande O, Bulteau C, Dellatolas G, Fohlen M, Jalin C, Buret V, Viguier D, Dorfmüller G, Jambaqué I (2007) Vertical parasagittal hemispherotomy: surgical procedures and clinical long term outcomes in a population of 83 children. Oper Neurosur 60:19–32

    Google Scholar 

  6. Kossoff EH, Vining EP, Pyzik PL, Kriegler S, Min KS, Carson BS, Avellino AM, Freeman JM (2002) The postoperative course and management of 106 hemidecortications. Pediatr Neurosurg 37:298–303

    Article  PubMed  CAS  Google Scholar 

  7. Harvey S, Cross JH, Shinnar S, Mathern GW, the ILAE Pediatric Epilepsy Surgery Taskforce (2008) Defining the spectrum of international practice in pediatric epilepsy surgery patients. Epilepsia 49(1):146–155

    Article  PubMed  Google Scholar 

  8. Carreño M, Wyllie E, Bingaman W, Kotagal P, Comair Y, Ruggieri P (2001) Seizure outcome after functional hemispherectomy for malformations of cortical development. Neurology 57:331–333

    Article  PubMed  Google Scholar 

  9. Devlin AM, Cross JH, Harkness W, Chong WK, Harding B, Vargha-Khadem F, Neville BGR (2003) Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence. Brain 126:556–566

    Article  PubMed  CAS  Google Scholar 

  10. Jonas R, Nguyen S, Asarnow RF, LoPresti C, Curtiss S, de Bode S, Yudovin S, Shields WD, Vinters HV, Mathern GW (2004) Cerebral hemispherectomy. Hospital course, seizure, developmental, language and motor outcomes. Neurology 62:1712–1721

    Article  PubMed  CAS  Google Scholar 

  11. Kossoff EH, Vining EPG, Pillas DJ, Pyzik PL, Avellino AM, Carson BS, Freeman JM (2003) Hemispherectomy for intractable unihemispheric epilepsy. Neurology 61:887–890

    Article  PubMed  CAS  Google Scholar 

  12. Basheer SN, Connolly MB, Lautzenhiser A, Sherman El MS, Hendson G, Steinbok P (2007) Hemispheric surgery in children with refractory epilepsy: seizure outcome, complications, and adaptive function. Epilepsia 48(1):133–140

    Article  PubMed  Google Scholar 

  13. Van Schooneveld MM, Jennekens-Schinkel A, van Rijen PC, Braun KP, van Nieuwenhuizen O (2011) Hemispherectomy: a basis for mental development in children with epilepsy. Epileptic Disord 13(1):47–55

    PubMed  Google Scholar 

  14. Pulsifer MB, Brandt J, Salorio CF, Vining EPG, Carson BS, Freeman JM (2004) The cognitive outcome of hemispherectomy in 71 children. Epilepsia 3:243–254

    Article  Google Scholar 

  15. Carreño M, Kotagal P, Perez-Jimenez A, Mesa T, Bingaman W, Wyllie E (2002) Intractable epilepsy in vascular congenital hemiparesis: clinical features and surgical options. Neurology 59:129–131

    Article  PubMed  Google Scholar 

  16. Terra-Bustamante VC, Machado HR, dos Santos OR, Serafini LN, Souza-Oliveira C, Escorsi-Rosset S, Yacubian EMT, Naffah-Mazzacoratti MG, Scorza CA, Cavalheiro EA, Scorza FA, Sakamoto AC (2009) Rasmussen encephalitis: long-term outcome after surgery. Childs Nerv Syst 25:583–589

    Article  PubMed  Google Scholar 

  17. Lettori D, Battaglia D, Sacco A, Veredice C, Chieffo D, Massimi L, Tartaglione T, Chiricozzi F, Staccioli S, Mittica A, Di Rocco C, Guzzeta F (2008) Early hemispherectomy in catastrophic epilepsy. A neuro-cognitive and epileptic long-term follow-up. Seizure 17:49–63

    Article  PubMed  CAS  Google Scholar 

  18. Thomas SG, Daniel RT, Chacko AG, Thomas M, Russell PS (2010) Cognitive changes following surgery in intractable hemispheric and sub-hemispheric pediatric epilepsy. Childs Nerv Syst 26(8):1067–1073

    Article  PubMed  Google Scholar 

  19. Dunkley C, Kung J, Scott RC, Nicolaides P, Neville B, Aylett SE, Harkness W, Cross JH (2011) Epilepsy surgery in children under 3 years. Epilepsy Res 93:96–106

    Article  PubMed  CAS  Google Scholar 

  20. Schramm J, Delev D, Wagner J, Elger CE, von Lehe M (2012) Seizure outcome, functional outcome, and quality of life after hemispherectomy in adults. Acta Neurochir (Wien) 154(9):1603–1612

    Article  CAS  Google Scholar 

  21. De Francisco J, Fernández D, Carreño M, Rumiá J, Donaire A, Maestro I, Bargalló N, Candela S, Pintor L, Boget T, Setoain W (2009) Successful functional hemispherectomy in adult patients with refractory epilepsy. Neurologia 24(1):9–14

    PubMed  Google Scholar 

  22. Engel J Jr, Van Ness PC, Rasmussen TB, Ojemann LM (1993) Outcome with respect to epileptic seizures. In: Engel J Jr (ed) Surgical treatment of the epilepsies. Raven, New York, pp 609–621

    Google Scholar 

  23. Newborg J, Stock JR, Wnek L (1998) Inventario de desarrollo Batelle (Spanish adaptation). TEA Ediciones, Madrid

    Google Scholar 

  24. Fournier-Del Castillo C, Pérez-Jiménez MA, García-Fernández M, García-Peñas JJ, Villarejo-Ortega F (2010) Presurgery neuropsychological evaluation in pediatric focal epilepsies. Rev Neurol 50(Supl. 3):S49–S57

    PubMed  Google Scholar 

  25. Comair Y, Villarejo F (1998) Hemisferectomía funcional. In: Villarejo-Ortega F (ed) Tratamiento de la Epilepsia. Díaz de Santos, Madrid, pp 353–358

    Google Scholar 

  26. Spencer DD, Spencer SS, Mattson RH, Williamson PD, Novelly RA (1984) Access to the posterior medial temporal lobe structures in the surgical treatment of temporal lobe epilepsy. Neurosurgery 15(5):667–671

    Article  PubMed  CAS  Google Scholar 

  27. Blümcke I, Thom M, Aronica E, Armstrong DD, Vinters HV, Palmini A, Jacques TS, Avanzini G, Barkovich AJ, Battaglia G, Becker A, Cepeda C, Cendes F, Colombo N, Crino P, Cross JH, Delalande O, Dubeau F, Duncan J, Guerrini R, Kahane P, Mathern G, Najm I, Ozkara C, Raybaud C, Represa A, Roper SN, Salamon N, Schulze-Bonhage A, Tassi L, Vezzani A, Spreafico R (2011) The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission. Epilepsia 52(1):158–174

    Article  PubMed  Google Scholar 

  28. González-Martínez JA, Gupta A, Kotagal P, Lachhwani D, Wyllie E, Lüders HO, Bingaman WE (2005) Hemispherectomy for catastrophic epilepsy in infants. Epilepsia 46(9):1518–1525

    Article  PubMed  Google Scholar 

  29. Koh S, Nguyen S, Asarnow RF, LoPresti C, Yudovin S, Shields WD, Vinters HV, Mathern GW (2004) Five or more acute postoperative seizures predict hospital course and long-term seizure control after hemispherectomy. Epilepsia 45(5):527–533

    Article  PubMed  Google Scholar 

  30. Mani J, Gupta A, Mascha E, Lachhwani D, Prakash K, Bingaman W, Wyllie E (2006) Postoperative seizures after extratemporal resections and hemispherectomy in pediatric epilepsy. Neurology 66:1038–1043

    Article  PubMed  CAS  Google Scholar 

  31. Krsek P, Pieper T, Karlmeier A, Hildebrandt M, Kolodziejczyk D, Winkler P, Pauli E, Blümcke I, Holthausen H (2009) Different presurgical characteristics and seizure outcomes in children with focal cortical dysplasia type I or II. Epilepsia 50(1):125–137

    Article  PubMed  Google Scholar 

  32. Döring S, Cross H, Boyd S, Harkness W, Neville B (1999) The significance of bilateral EEG abnormalities before and alter hemispherectomy in children with unilateral major hemisphere lesions. Epilepsy Res 34:65–73

    Article  PubMed  Google Scholar 

  33. Chugani HT, Shewmon DA, Shields WD, Sankar R, Comair Y, Vinters HV, Peacock WJ (1993) Surgery for intractable infantile spasms: neuroimaging perspectives. Epilepsia 34:764–771

    Article  PubMed  CAS  Google Scholar 

  34. Kramer U, Sue WC, Mikati MA (1997) Focal features in West syndrome indicating candidacy for surgery. Pediatr Neurol 16(3):213–217

    Article  PubMed  CAS  Google Scholar 

  35. Wyllie E (1998) Surgical treatment of epilepsy in children. Pediatr Neurol 19(3):179–188

    Article  PubMed  CAS  Google Scholar 

  36. Angelini L, Broggi G, Riva D, Solero CL (1979) A case of Lennox–Gastaut syndrome successfully treated by removal of a parietotemporal astrocytoma. Epilepsia 20:665–669

    Article  PubMed  CAS  Google Scholar 

  37. Quarato PP, Di Gennaro G, Manfredi M, Esposito V (2002) Atypical Lennox–Gastaut syndrome successfully treated with removal of a parietal dysembryoplastic tumour. Seizure 11:325–329

    Article  PubMed  Google Scholar 

  38. Wyllie E, Lachhwani DK, Gupta A, Chirla A, Cosmo G, Worlet S, Kotagal P, Ruggieri P, Bingaman WE (2007) Successful surgery for epilepsy due to early brain lesions despite generalized EEG findings. Neurology 69:389–397

    Article  PubMed  CAS  Google Scholar 

  39. Gupta A, Chirla A, Wyllie E, Lachhwani DK, Kotagal P, Bingaman WE (2007) Pediatric epilepsy surgery in focal lesions and generalized electroencephalogram abnormalities. Pediatr Neurol 37:8–15

    Article  PubMed  Google Scholar 

  40. Loddenkemper T, Cosmo G, Kotagal P, Haut J, Klaas P, Gupta A, Lachhwani DK, Bingaman W, Wyllie E (2009) Epilepsy surgery in children with electrical status epilepticus in sleep. Neurosurgery 64:328–337

    Article  PubMed  Google Scholar 

  41. Peltola ME, Liukkonen E, Granström ML, Paetau R, Kantola-Sorsa EK, Valanne L, Falck B, Blomstedt G, Gaily E (2011) The effect of surgery in encephalopathy with electrical status epilepticus during sleep. Epilepsia 52(3):602–609

    Article  PubMed  Google Scholar 

  42. Garzon E, Gupta A, Bingaman W, Sakamoto AC, Lüders H (2009) Paradoxical ictal EEG lateralization in children with unilateral encephaloclastic lesion. Epileptic Disord 11(3):215–221

    PubMed  Google Scholar 

  43. Boshuisen K, van Schooneveld MMJ, Leijten FSS, de Kort GAP, van Rijen PC, Gosselaar PH, van Nieuwenhuizen O, Braun KPJ (2010) Contralateral MRI abnormalities affect seizure and cognitive outcome after hemispherectomy. Neurology 75:1623–1630

    Article  PubMed  CAS  Google Scholar 

  44. Hallbook T, Ruggieri P, Adina C, Lachhwani DK, Gupta A, Kotagal P, Bingaman WE, Wyllie E (2010) Contralateral MRI abnormalities in candidates for hemispherectomy for refractory epilepsy. Epilepsia 51(4):556–563

    Article  PubMed  Google Scholar 

  45. Telfeian AE, Berqvist C, Danielak C, Simon SL, Duhaime AC (2002) Recovery of language after left hemispherectomy in a sixteen-year-old girl with late-onset seizures. Pediatr Neurosurg 37(1):19–21

    Article  PubMed  Google Scholar 

  46. Loddenkemper T, Wyllie E, Lardizabal D, Stanford LD, Bingaman W (2003) Late language transfer in patients with Rasmussen encephalitis. Epilepsia 44(6):870–871

    Article  PubMed  Google Scholar 

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Acknowledgment

We thank Arturo Ugalde-Canitrot for reviewing the English language and contents of this manuscript.

Disclosure of conflicts of interest

We declare that we have no conflicts of interest.

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Authors and Affiliations

  1. Neurosurgery Service, Hospital Infantil Universitario Niño Jesús, Madrid, Spain

    Francisco Villarejo-Ortega & Marcelo Budke

  2. Epilepsy Monitoring Unit, Clinical Neurophysiology Section, Hospital Infantil Universitario Niño Jesús, Madrid, 28009, Spain

    Marta García-Fernández & María-Ángeles Pérez-Jiménez

  3. Neuropsychology Unit, Psychiatry and Psychology Section, Hospital Infantil Universitario Niño Jesús, Madrid, Spain

    Concepción Fournier-Del Castillo

  4. Foundation for Biomedical Investigation, Hospital Universitario Ramón y Cajal, Madrid, Spain

    Martín Fabregate-Fuente

  5. Neuroradiology Section, Diagnostic Imaging Service, Hospital Ruber Internacional, Madrid, Spain

    Juan Álvarez-Linera

  6. Pathology Service, Hospital Infantil Universitario Niño Jesús, Madrid, Spain

    Inmaculada De Prada-Vicente

  7. Neurology Section, Hospital Infantil Universitario Niño Jesús, Madrid, Spain

    María-Luz Ruiz-Falcó

Authors
  1. Francisco Villarejo-Ortega
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  2. Marta García-Fernández
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  3. Concepción Fournier-Del Castillo
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  9. María-Ángeles Pérez-Jiménez
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Corresponding author

Correspondence to Marta García-Fernández.

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Villarejo-Ortega, F., García-Fernández, M., Fournier-Del Castillo, C. et al. Seizure and developmental outcomes after hemispherectomy in children and adolescents with intractable epilepsy. Childs Nerv Syst 29, 475–488 (2013). https://doi.org/10.1007/s00381-012-1949-8

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  • DOI: https://doi.org/10.1007/s00381-012-1949-8

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