Catastrophic Epilepsy and Hemispherotomy

Living reference work entry


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.


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


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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

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