Advertisement

Catastrophic Epilepsy and Hemispherotomy

  • Brian J. Dlouhy
  • Matthew D. SmythEmail author
Living reference work entry

Abstract

The catastrophic epilepsies encompass a number of severe epilepsies that are intractable, result in intellectual and behavioral disability, and have the highest incidence of sudden unexpected death in epilepsy (SUDEP). For the catastrophic hemispheric epilepsies such as Sturge-Weber syndrome, hemimegalencephaly, Rasmussen’s encephalitis, extensive unilateral cortical dysplasia, perinatal hemispheric infarct, and other unilateral refractory epilepsy, hemispheric disconnection has long been used to treat these types of epilepsies. Krynauw reported on the first major series of anatomical hemispherectomy (AH) for intractable seizures in 1950. Complications encouraged modifications to the original technique. Rasmussen introduced the functional hemispherectomy (FH) in 1974, which allowed much less brain excision and introduced the concept of hemispheric disconnection. Further modifications continued, all based on the concept of maximal disconnection and minimal excision. Therefore, future modifications became known as variants of the “hemispherotomy.” In 1995, Villemure introduced the peri-insular hemispherotomy (PIH). Here, we will discuss the catastrophic hemispheric epilepsies and surgical indications, preoperative planning, operative steps, and postoperative management of patients undergoing PIH – our modification of the approach described by Villemure and Mascott and Shimizu and Maehara.

Keywords

Epilepsy Seizures Intractable Hemispherotomy Sturge-Weber syndrome Hemimegalencephaly Rasmussen’s encephalitis Cortical dysplasia 

References

  1. Bachur CD, Comi AM (2013) Sturge-weber syndrome. Curr Treat Options Neurol 15(5):607–617CrossRefGoogle Scholar
  2. Bien CG, Tiemeier H, Sassen R et al (2013) Rasmussen encephalitis: incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins. Epilepsia 54(3):543–550CrossRefGoogle Scholar
  3. Ciliberto MA, Limbrick D, Powers A, Titus JB, Munro R, Smyth MD (2012) Palliative hemispherotomy in children with bilateral seizure onset. J Neurosurg Pediatr 9(4):381–388CrossRefGoogle Scholar
  4. Daniel RT, Villemure JG (2003) Peri-insular hemispherotomy: potential pitfalls and avoidance of complications. Stereotact Funct Neurosurg 80(1–4):22–27CrossRefGoogle Scholar
  5. Danielpour M, von Koch CS, Ojemann SG, Peacock WJ (2001) Disconnective hemispherectomy. Pediatr Neurosurg 35(4):169–172CrossRefGoogle Scholar
  6. De Almeida AN, Marino R Jr, Aguiar PH, Jacobsen Teixeira M (2006) Hemispherectomy: a schematic review of the current techniques. Neurosurg Rev 29(2):97–102; discussion 102CrossRefGoogle Scholar
  7. De Ribaupierre S, Delalande O (2008) Hemispherotomy and other disconnective techniques. Neurosurg Focus 25(3):E14CrossRefGoogle Scholar
  8. Delalande O, Pinard JM, Basevant C, Gauthe M, Plouin P, Dulac O (1992) Hemispherotomy: a new procedure for central disconnection. Epilepsia 33(Suppl 3):99–100Google Scholar
  9. Delalande O, Bulteau C, Dellatolas G et al (2007) Vertical parasagittal hemispherotomy: surgical procedures and clinical long-term outcomes in a population of 83 children. Neurosurgery 60(2 Suppl 1):ONS19-32; discussion ONS32Google Scholar
  10. Dlouhy BJ, Miller B, Jeong A, Bertrand ME, Limbrick DD Jr, Smyth MD (2016) Palliative epilepsy surgery in Dravet syndrome-case series and review of the literature. Childs Nerv Syst 32(9):1703–1708CrossRefGoogle Scholar
  11. Dymerska M, Kirkorian AY, Offermann EA, Lin DD, Comi AM, Cohen BA (2017) Size of facial port-wine birthmark may predict neurologic outcome in Sturge-Weber syndrome. J Pediatr 188:205–209. e201CrossRefGoogle Scholar
  12. Ghatan S, McGoldrick P, Palmese C et al (2014) Surgical management of medically refractory epilepsy due to early childhood stroke. J Neurosurg Pediatr 14(1):58–67CrossRefGoogle Scholar
  13. Howard MA, Baraban SC (2017) Catastrophic epilepsies of childhood. Annu Rev Neurosci 40:149–166CrossRefGoogle Scholar
  14. Kamath AA, Limbrick DL, Smyth MD (2015) Characterization of postoperative fevers after hemispherotomy. Childs Nerv Syst 31(2):291–296CrossRefGoogle Scholar
  15. Krynauw RA (1950) Infantile hemiplegia treated by removing one cerebral hemisphere. J Neurol Neurosurg Psychiatry 13(4):243–267CrossRefGoogle Scholar
  16. Limbrick DD, Narayan P, Powers AK et al (2009) Hemispherotomy: efficacy and analysis of seizure recurrence. J Neurosurg Pediatr 4(4):323–332CrossRefGoogle Scholar
  17. McKenzie KG (1938) The present status of a patient who had the right cerebral hemisphere removed. JAMA 111:168–183Google Scholar
  18. Morino M, Shimizu H, Ohata K, Tanaka K, Hara M (2002) Anatomical analysis of different hemispherotomy procedures based on dissection of cadaveric brains. J Neurosurg 97(2):423–431CrossRefGoogle Scholar
  19. Nagahama Y, Joshi C, Dlouhy B et al (2017) Functional hemispherotomy in Rasmussen syndrome in the absence of classic MRI findings. Epilepsy Behav Case Rep 7:24–27CrossRefGoogle Scholar
  20. O’Rourke DJ, Bergin A, Rotenberg A et al (2014) Rasmussen’s encephalitis presenting as focal cortical dysplasia. Epilepsy Behav Case Rep 2:86–89CrossRefGoogle Scholar
  21. Rasmussen T (1973) Postoperative superficial hemosiderosis of the brain, its diagnosis, treatment and prevention. Trans Am Neurol Assoc 98:133–137PubMedGoogle Scholar
  22. Rasmussen T (1983) Hemispherectomy for seizures revisited. Can J Neurol Sci 10(2):71–78CrossRefGoogle Scholar
  23. Schramm J, Behrens E, Entzian W (1995) Hemispherical deafferentation: an alternative to functional hemispherectomy. Neurosurgery 36(3):509–515; discussion 515–506PubMedGoogle Scholar
  24. Schramm J, Kral T, Clusmann H (2001) Transsylvian keyhole functional hemispherectomy. Neurosurgery 49(4):891–900; discussion 900–891PubMedGoogle Scholar
  25. Shimizu H, Maehara T (2000) Modification of peri-insular hemispherotomy and surgical results. Neurosurgery 47(2):367–372; discussion 372–363CrossRefGoogle Scholar
  26. Shirley MD, Tang H, Gallione CJ et al (2013) Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med 368(21):1971–1979CrossRefGoogle Scholar
  27. Sillanpaa M, Shinnar S (2010) Long-term mortality in childhood-onset epilepsy. N Engl J Med 363(26):2522–2529CrossRefGoogle Scholar
  28. Smith SJ, Andermann F, Villemure JG, Rasmussen TB, Quesney LF (1991) Functional hemispherectomy: EEG findings, spiking from isolated brain postoperatively, and prediction of outcome. Neurology 41(11):1790–1794CrossRefGoogle Scholar
  29. Sood S, Asano E, Chugani HT (2008) Role of external ventriculostomy in the management of fever after hemispherectomy. J Neurosurg Pediatr 2(6):427–429CrossRefGoogle Scholar
  30. Vadera S, Moosa AN, Jehi L et al (2012) Reoperative hemispherectomy for intractable epilepsy: a report of 36 patients. Neurosurgery 71(2):388–392; discussion 392–383CrossRefGoogle Scholar
  31. Varadkar S, Bien CG, Kruse CA et al (2014) Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol 13(2):195–205CrossRefGoogle Scholar
  32. Villemure JG, Daniel RT (2006) Peri-insular hemispherotomy in paediatric epilepsy. Childs Nerv Syst 22(8):967–981CrossRefGoogle Scholar
  33. Villemure JG, Mascott CR (1995) Peri-insular hemispherotomy: surgical principles and anatomy. Neurosurgery 37(5):975–981CrossRefGoogle Scholar
  34. Wang DD, Benkli B, Auguste KI et al (2014) Unilateral holohemispheric central nervous system lesions associated with medically refractory epilepsy in the pediatric population: a retrospective series of hemimegalencephaly and Rasmussen’s encephalitis. J Neurosurg Pediatr 14(6):573–584CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Pappajohn Biomedical InstituteUniversity of Iowa Carver College of MedicineIowa CityUSA
  2. 2.Department of NeurosurgeryUniversity of Iowa Hospitals and ClinicsIowa CityUSA
  3. 3.Washington UniversitySt. LouisUSA

Section editors and affiliations

  • James T. Rutka
    • 1
  1. 1.Division of Neurosurgery, The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick ChildrenUniversity of TorontoTorontoCanada

Personalised recommendations