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Cortical Dysplasia

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Textbook of Pediatric Neurosurgery

Abstract

The most common etiologic factor requiring epilepsy surgery in children is cortical dysplasia (CD). In recent years, the advent of modern imaging techniques and refined surgical approaches has allowed for a greater number of these patients to be properly diagnosed and treated, providing very good seizure outcomes. New pathological classifications of the different types of malformations of cortical development (MCD), CD among them, have included embryological, radiological, and genetic features. Moreover, it is now well recognized that the key to successful surgical treatment is to carry out a comprehensive presurgical workup, which consists of various noninvasive assessments, but that might also require invasive techniques such as the use of subdural electrodes or stereoelectroencephalography (SEEG). Thereby, completeness of resection of CD, which is certainly the most important prognostic factor for seizure freedom, can be achieved by means of different surgical strategies. These clinical, pathological, diagnostic, and surgical aspects, as well as outcome and other details regarding CD in children, will be discussed herein.

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References

  • Adelson PD (2001) Temporal lobectomy in children with intractable seizures. Pediatr Neurosurg 34(5):268–277

    Article  CAS  PubMed  Google Scholar 

  • Adelson PD, Black PM, Madsen JR, Kramer U, Rockoff MA, Riviello JJ, Helmers SL, Mikati M, Holmes GL (1995) Use of subdural grids and strip electrodes to identify a seizure focus in children. Pediatr Neurosurg 22:174–180

    Article  CAS  PubMed  Google Scholar 

  • Ajmone Marsan C (1980) Depth electrography and electrocorticography. In: Aminoff MJ (ed) Electrodiagnosis in clinical neurology. Churchill Livingstone, New York, pp 167–196

    Google Scholar 

  • Alexandre V Jr, Walz R, Bianchin MM, Velasco TR, Terra-Bustamante VC, Wichert-Ana L, Araújo D Jr, Machado HR, Assirati JA Jr, Carlotti CG Jr, Santos AC, Serafini LN, Sakamoto AC (2006) Seizure outcome after surgery for epilepsy due to focal cortical dysplastic lesions. Seizure 15(6):420–427

    Article  PubMed  Google Scholar 

  • Almeida AN, Martinez V, Feindel W (2005) The first case of invasive EEG monitoring for the surgical treatment of epilepsy: historical significance and context. Epilepsia 46(7):1082–1085

    Article  PubMed  Google Scholar 

  • Aronica E, Becker AJ, Spreafico R (2012) Malformations of cortical development. Brain Pathol 22(3):380–401

    Google Scholar 

  • Bancaud J, Angelergues R, Bernouilli C (1970) Functional stereotaxic exploration (SEEG) of epilepsy. Electroencephalogr Clin Neurophysiol 28(1):85–86

    Article  CAS  PubMed  Google Scholar 

  • Barkovich AJ, Kuzniecky RI, Dobyns WB, Jackson GD, Becker LE, Evrard P (1996) A classification scheme for malformations of cortical development. Neuropediatrics 27(2):59–63

    Article  CAS  PubMed  Google Scholar 

  • Barkovich AJ, Kuzniecky RI, Dobyns WB (2001) Radiologic classification of malformations of cortical development. Curr Opin Neurol 2:145–149

    Article  Google Scholar 

  • Barkovich AJ, Guerrini R, Kuzniecky RI, Jackson GD, Dobyns WB (2012) A developmental and genetic classification for malformations of cortical development: update 2012. Brain 135(Pt 5):1348–1369

    Article  PubMed  PubMed Central  Google Scholar 

  • Binder DK, Schramm J (2006) Transsylvian functional hemispherectomy. Childs Nerv Syst 22(8):960–966

    Article  PubMed  Google Scholar 

  • Blümcke I, Coras R (2013) The curse of in silico transformation from Palmini's into the ILAE classification system of focal cortical dysplasia: a critical comment. Epilepsia 54(8):1506–1507

    Article  PubMed  Google Scholar 

  • 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 

  • Bruce DA, Bizzi JWJ (2000) Surgical technique for the insertion of grids and strips for invasive monitoring in children with intractable epilepsy. Childs Nerv Syst 16:724–730

    Article  CAS  PubMed  Google Scholar 

  • Bulacio JC, Jehi L, Wong C et al (2012) Long-term seizure outcome after resective surgery in patients evaluated with intracranial elec- trodes. Epilepsia 53(10):1722–1730

    Article  PubMed  Google Scholar 

  • Cepeda C, André VM, Flores-Hernández J, Nguyen OK, Wu N, Klapstein GJ, Nguyen S, Koh S, Vinters HV, Levine MS, Mathern GW (2005) Pediatric cortical dysplasia: correlations between neuroimaging, electrophysiology and location of cytomegalic neurons and balloon cells and glutamate/GABA synaptic circuits. Dev Neurosci 27(1):59–76

    Article  CAS  PubMed  Google Scholar 

  • Chern JJ, Patel AJ, Jea A, Curry DJ, Comair YG (2010) Surgical outcome for focal cortical dysplasia: an analysis of recent surgical series. J Neurosurg Pediatr 6(5):452–458

    Article  PubMed  Google Scholar 

  • Colombo N, Salamon N, Raybaud C, Ozkara C, Barkovich AJ (2009) Imaging of malformations of cortical development. Epileptic Disord 11(3):194–205

    PubMed  Google Scholar 

  • Cossu M, Cardinale F, Castana L, Nobili L, Sartori I, Russo L (2006) Stereo-EEG in children. Childs Nerv Syst 22(8):766–778

    Article  CAS  PubMed  Google Scholar 

  • Cossu M, Fuschillo D, Bramerio M, Galli C, Gozzo F, Pelliccia V, Casaceli G, Tassi L, Lo Russo G (2013) Epilepsy surgery of focal cortical dysplasia-associated tumors. Epilepsia 54(Suppl 9):115–122

    Article  PubMed  Google Scholar 

  • Crino PB (2015) Focal cortical dysplasia. Semin Neurol 35(3):201–208

    Article  PubMed  Google Scholar 

  • Datta A, Sinclair DB, Wheatley M, Jurasek L, Snyder T, Quigley D, Ahmed SN, Gross D (2009) Selective amygdalohippocampectomy: surgical outcome in children versus adults. Can J Neurol Sci 36:187–191

    CAS  PubMed  Google Scholar 

  • De Ribaupierre S, Delalande O (2008) Hemispherotomy and other disconnective techniques. Neurosurg Focus 25(3):E14

    Article  PubMed  Google Scholar 

  • Delalande O, Dorfmüller G (2008) Parasagittal vertical hemispherotomy: surgical procedure. Neurochirurgie 54(3):353–357

    Article  CAS  PubMed  Google Scholar 

  • Dorfmüller G, Ferrand-Sorbets S, Fohlen M, Bulteau C, Archambaud F, Delalande O, Chipaux M, Taussig D (2014) Outcome of surgery in children with focal cortical dysplasia younger than 5 years explored by stereo-electroencephalography. Childs Nerv Syst 30(11):1875–1883

    Article  PubMed  Google Scholar 

  • Engel J Jr (1996) Surgery for seizures. N Engl J Med 334(10):647–652

    Article  PubMed  Google Scholar 

  • Englot DJ, Rolston JD, Wang DD, Sun PP, Chang EF, Auguste KI (2013) Seizure outcomes after temporal lobectomy in pediatric patients. J Neurosurg Pediatr 12(2):134–141

    Article  PubMed  Google Scholar 

  • Gallentine WB, Mikati MA (2009) Intraoperative electrocorticography and cortical stimulation in children. J Clin Neurophysiol 26(2):95–108

    Article  PubMed  Google Scholar 

  • Gonzalez-Martinez J, Lachhwani D (2014) Stereoelectroencephalography in children with cortical dysplasia: technique and results. Childs Nerv Syst 30(11):1853–1857

    Article  PubMed  Google Scholar 

  • Gonzalez-Martinez J, Najm IM (2014) Indications and selection criteria for invasive monitoring in children with cortical dysplasia. Childs Nerv Syst 30(11):1823–1829

    Article  PubMed  Google Scholar 

  • Gonzalez-Martinez J, Mullin J, Bulacio J, Gupta A, Enatsu R, Najm I, Bingaman W, Wyllie E, Lachhwani D (2014a) Stereoelectroencephalography in children and adolescents with difficult-to-localize refractory focal epilepsy. Neurosurgery 75(3):258–268

    Article  PubMed  Google Scholar 

  • Gonzalez-Martinez J, Mullin J, Vadera S, Bulacio J, Hughes G, Jones S, Enatsu R, Najm I (2014b) Stereotactic placement of depth electrodes in medically intractable epilepsy. J Neurosurg 120(3):639–644

    Article  PubMed  Google Scholar 

  • Guerrini R, Dobyns WB (2014) Malformations of cortical development: clinical features and genetic causes. Lancet Neurol 13(7):710–726

    Article  PubMed  PubMed Central  Google Scholar 

  • Guerrini R, Duchowny M, Jayakar P, Krsek P, Kahane P, Tassi L, Melani F, Polster T, Andre VM, Cepeda C, Krueger DA, Cross JH, Spreafico R, Cosottini M, Gotman J, Chassoux F, Ryvlin P, Bartolomei F, Bernasconi A, Stefan H, Miller I, Devaux B, Najm I, Giordano F, Vonck K, Barba C, Blumcke I (2015) Diagnostic methods and treatment options for focal cortical dysplasia. Epilepsia 56(11):1669–1686

    Article  CAS  PubMed  Google Scholar 

  • Gupta A, Chirla A, Wyllie E et al (2007) Pediatric epilepsy surgery in focal lesions and generalized electroencephalogram abnormalities. Pediatr Neurol 37(1):8–15

    Article  PubMed  Google Scholar 

  • Harvey AS, Cross JH, Shinnar S, Mathern GW, ILAE Pediatric Epilepsy Surgery Survey Taskforce (2008) Defining the spectrum of international practice in pediatric epilepsy surgery patients. Epilepsia 49(1):146–155. Erratum in: Epilepsia. 2013;54(6):1140

    Article  PubMed  Google Scholar 

  • Hirsch E, Arzimanoglou A (2004) Children with drug-resistant partial epilepsy: criteria for the identification of surgical candidates. Rev Neurol (Paris) 160:210–219

    Article  Google Scholar 

  • Jarrar RG, Buchhalter JR, Meyer FB, Sharbrough FW, Laws E (2002) Long-term follow-up of temporal lobectomy in children. Neurology 59(10):1635–1637

    Article  CAS  PubMed  Google Scholar 

  • Jayakar P, Gaillard WD, Tripathi M, Libenson MH, Mathern GW, Cross JH, Task Force for Paediatric Epilepsy Surgery, Commission for Paediatrics, and the Diagnostic Commission of the International League Against Epilepsy (2014) Diagnostic test utilization in evaluation for resective epilepsy surgery in children. Epilepsia 55(4):507–518

    Article  PubMed  Google Scholar 

  • Kang JW, Rhie SK, Yu R, Eom S, Hong W, Kim SH, Kang HC, Lee JS, Lee YM, Kim HD (2013) Seizure outcome of infantile spasms with focal cortical dysplasia. Brain and Development 35(8):816–820

    Article  PubMed  Google Scholar 

  • Kawai K (2015) Epilepsy surgery: current status and ongoing challenges. Neurol Med Chir (Tokyo) 55(5):357–366

    Article  Google Scholar 

  • Kerfoot C, Vinters HV, Mathern GW (1999) Cerebral cortical dysplasia: giant neurons show potential for increased excitation and axonal plasticity. Dev Neurosci 21(3–5):260–270

    Article  CAS  PubMed  Google Scholar 

  • Kim SK, Wang KC, Hwang YS, Kim KJ, Cho BK (2001) Intractable epilepsy associated with brain tumors in children: surgical modality and outcome. Childs Nerv Syst 17:445–452

    Article  CAS  PubMed  Google Scholar 

  • Krsek P, Maton B, Jayakar P, Dean P, Korman B, Rey G, Dunoyer C, Pacheco-Jacome E, Morrison G, Ragheb J, Vinters HV, Resnick T, Duchowny M (2009) Incomplete resection of focal cortical dysplasia is the main predictor of poor postsurgical outcome. Neurology 72(3):217–223

    Article  CAS  PubMed  Google Scholar 

  • Lee YJ, Lee JS (2013) Temporal lobe epilepsy surgery in children versus adults: from etiologies to outcomes. Korean J Pediatr 56(7):275–281

    Article  PubMed  PubMed Central  Google Scholar 

  • Miserocchi A, Cascardo B, Piroddi C, Fuschillo D, Cardinale F, Nobili L, Francione S, Russo GL, Cossu M (2013) Surgery for temporal lobe epilepsy in children: relevance of presurgical evaluation and analysis of outcome. J Neurosurg Pediatr 11(3):256–267

    Article  PubMed  Google Scholar 

  • Moosa AN, Gupta A (2014) Outcome after epilepsy surgery for cortical dysplasia in children. Childs Nerv Syst 30(11):1905–1911

    Article  PubMed  Google Scholar 

  • Mühlebner A, Gröppel G, Dressler A, Reiter-Fink E, Kasprian G, Prayer D, Dorfer C, Czech T, Hainfellner JA, Coras R, Blümcke I, Feucht M (2014) Epilepsy surgery in children and adolescents with malformations of cortical development – outcome and impact of the new ILAE classification on focal cortical dysplasia. Epilepsy Res 108(9):1652–1661

    Article  PubMed  Google Scholar 

  • Najm I, Ying Z, Babb T, Crino PB, Macdonald R, Mathern GW, Spreafico R (2004) Mechanisms of epileptogenicity in cortical dysplasias. Neurology 62(6 Suppl 3):S9–13

    Article  CAS  PubMed  Google Scholar 

  • Önal C, Otsubo H, Araki T, Chitoku S, Ochi A, Weiss S, Elliot I, Snead OC III, Rutka JT, Logan W (2003) Complications of invasive subdural grid monitoring in children with epilepsy. J Neurosurg 98:1017–1026

    Article  PubMed  Google Scholar 

  • Otsuki T, Honda R, Takahashi A, Kaido T, Kaneko Y, Nakai T, Saito Y, Itoh M, Nakagawa E, Sugai K, Sasaki M (2013) Surgical management of cortical dysplasia in infancy and early childhood. Brain and Development 35(8):802–809

    Article  PubMed  Google Scholar 

  • Palmini A, Najm I, Avanzini G, Babb T, Guerrini R, Foldvary-Schaefer N, Jackson G, Lüders HO, Prayson R, Spreafico R, Vinters HV (2004) Terminology and classification of the cortical dysplasias. Neurology 62(6 Suppl 3):S2–S8

    Article  CAS  PubMed  Google Scholar 

  • Pestana Knight EM, Gonzales-Martinez J, Gupta A (2015) Pre-operative evaluation in pediatric patients with cortical dysplasia. Childs Nerv Syst 31:2225–2233

    Article  PubMed  Google Scholar 

  • Qiu B, Ou S, Song T, Hu J, You L, Wang Y, Wang Y (2014) Intraoperative electrocorticography-guided microsurgical management for patients with onset of supratentorial neoplasms manifesting as epilepsy: a review of 65 cases. Epileptic Disord 16(2):175–184

    PubMed  Google Scholar 

  • Rowland NC, Englot DJ, Cage TA, Sughrue ME, Barbaro NM, Chang EF (2012) A meta-analysis of predictors of seizure freedom in the surgical management of focal cortical dysplasia. J Neurosurg 116(5):1035–1041

    Article  PubMed  Google Scholar 

  • Santos AC, Escorsi-Rosset S, Simao GN, Terra VC, Velasco T, Neder L, Sakamoto AC, Machado HR (2014) Hemispheric dysplasia and hemimegalencephaly: imaging definitions. Childs Nerv Syst 30(11):1813–1821

    Article  PubMed  Google Scholar 

  • Sarnat HB, Flores-Sarnat L (2015) Infantile tauopathies: Hemimegalencephaly; tuberous sclerosis complex; focal cortical dysplasia 2; ganglioglioma. Brain Dev 37(6):553–562

    Article  PubMed  Google Scholar 

  • Shimizu H, Maehara T (2000) Modification of peri-insular hemispherotomy and surgical results. Neurosurg 47(2):367–373

    Article  CAS  Google Scholar 

  • Simon SL, Telfeian A, Duhaime AC (2003) Complications of invasive monitoring used in intractable pediatric epilepsy. Pediatr Neurosurg 38:47–52

    Article  PubMed  Google Scholar 

  • Sisodiya SM (2004) Surgery for focal cortical dysplasia. Brain 127(Pt 11):2383–2384

    Article  CAS  PubMed  Google Scholar 

  • Skirrow C, Cross JH, Harrison S, Cormack F, Harkness W, Coleman R, Meierotto E, Gaiottino J, Vargha-Khadem F, Baldeweg T (2015) Temporal lobe surgery in childhood and neuroanatomical predictors of long-term declarative memory outcome. Brain 138(Pt 1):80–93

    Article  PubMed  Google Scholar 

  • Spreafico R, Blümcke I (2010) Focal cortical dysplasias: clinical implication of neuropathological classification systems. Acta Neuropathol 120(3):359–367

    Article  PubMed  Google Scholar 

  • Steinbok P, Gan PY, Connolly MB, Carmant L, Barry Sinclair D, Rutka J, Griebel R, Aronyk K, Hader W, Ventureyra E, Atkinson J (2009) Epilepsy surgery in the first 3 years of life: a Canadian survey. Epilepsia 50(6):1442–1449

    Article  PubMed  Google Scholar 

  • Sugano H, Shimizu H, Sunaga S (2007) Efficacy of intraoperative electrocorticography for assessing seizure outcomes in intractable epilepsy patients with temporal-lobe-mass lesions. Seizure 16(2):120–127

    Article  PubMed  Google Scholar 

  • Tassi L, Colombo N, Garbelli R, Francione S, Lo Russo G, Mai R, Cardinale F, Cossu M, Ferrario A, Galli C, Bramerio M, Citterio A, Spreafico R (2002) Focal cortical dysplasia: neuropathological subtypes, EEG, neuroimaging and surgical outcome. Brain 125(Pt 8):1719–1732

    Article  CAS  PubMed  Google Scholar 

  • Tassi L, Garbelli R, Colombo N, Bramerio M, Lo Russo G, Deleo F, Milesi G, Spreafico R (2010) Type I focal cortical dysplasia: surgical outcome is related to histopathology. Epileptic Disord 12(3):181–191

    PubMed  Google Scholar 

  • Taylor DC, Falconer MA, Bruton CJ, Corsellis JA (1971) Focal dysplasia of the cerebral cortex in epilepsy. J Neurol Neurosurg Psychiatry 34(4):369–387

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Terra VC, Thomé U, Rosset SS, Funayama SS, dos Santos AC, dos Santos MV, Sakamoto AC, Machado HR (2014) Surgery for focal cortical dysplasia in children using intraoperative mapping. Childs Nerv Syst 30(11):1839–1851

    Article  PubMed  Google Scholar 

  • Terra-Bustamante VC, Inuzuka LM, Fernandes RM, Escorsi-Rosset S, Wichert-Ana L, Alexandre V Jr, Bianchin MM, Araújo D, Santos AC, Oliveira dos Santos R, Machado HR, Sakamoto AC (2007) Outcome of hemispheric surgeries for refractory epilepsy in pediatric patients. Childs Nerv Syst 23(3):321–326

    Article  PubMed  Google Scholar 

  • Vadera S, Mullin J, Bulacio J, Najm I, Bingaman W, Gonzalez-Martinez J (2013) SEEG following subdural grid placement for difficult to localize epilepsy. Neurosurgery 72(5):723–729

    Article  PubMed  Google Scholar 

  • Villemure JG, Daniel RT (2006) Peri-insular hemispherotomy in paediatric epilepsy. Childs Nerv Syst 22(8):967–981

    Article  PubMed  Google Scholar 

  • Widdess-Walsh P, Jeha L, Nair D, Kotagal P, Bingaman W, Najm I (2007) Subdural electrode analysis in focal cortical dysplasia: predictors of surgical outcome. Neurology 69(7):660–667

    Article  CAS  PubMed  Google Scholar 

  • Wiebe S, Blume WT, Girvin JP, Eliasziw M, Effectiveness and Efficiency of Surgery for Temporal Lobe Epilepsy Study Group (2001) A randomized, controlled trial of surgery for temporal-lobe epilepsy. N Engl J Med 345:311–318

    Article  CAS  PubMed  Google Scholar 

  • Wong M, Crino PB (2012) mTOR and epileptogenesis in developmental brain malformations. In: Noebels JL, Avoli M, Rogawski MA, Olsen RW, Delgado-Escueta AV (eds) Jasper's basic mechanisms of the epilepsies, 4th edn. National Center for Biotechnology Information, Bethesda

    Google Scholar 

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Machado, H.R., Santos, M.V. (2017). Cortical Dysplasia. In: Di Rocco, C., Pang, D., Rutka, J. (eds) Textbook of Pediatric Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-319-31512-6_41-1

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