title: Hemispherectomy for Hemimegalencephaly Due to Tuberous Sclerosis and a Review of the Literature.
abstract: BACKGROUND
  Hemimegalencephaly with tuberous sclerosis complex is an uncommon association, usually associated with intractable seizures that begin in the neonatal period or early infancy. Typically, the seizures are managed with medications until the patient is older when surgical treatment is considered safe.
  
  
  METHODS
  We describe a 7-week-old infant with tuberous sclerosis (TSC1 mutation) and hemimegalencephaly who underwent a functional hemispherectomy for status epilepticus. No clinical seizures have occurred since surgery nearly 5 years ago and subsequent weaning of antiepileptic drugs 3 years ago. This is one of the youngest patients with tuberous sclerosis complex treated with a hemispherectomy and one of seven patients described in the literature.
  
  
  CONCLUSIONS
  Our patient, along with previously reported cases, suggests that a hemispherectomy is a viable option in the very young. With evolution of this surgical process since its inception nearly 6 decades ago, it may now be performed safely in early infancy, engendering the possibility of seizure freedom in most and thus optimizing neurodevelopmental outcome.
authors.affiliation: University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: Vishnu Anand
authors.initials: VA
authors.lastname: Cuddapah
authors.affiliation: Children's Hospital of Alabama, Birmingham, Alabama.
authors.forename: Matthew
authors.initials: M
authors.lastname: Thompson
authors.affiliation: University of Alabama at Birmingham, Birmingham, Alabama; Children's Hospital of Alabama, Birmingham, Alabama.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Blount
authors.affiliation: Children's Hospital of Alabama, Birmingham, Alabama.
authors.forename: Rong
authors.initials: R
authors.lastname: Li
authors.affiliation: University of Alabama at Birmingham, Birmingham, Alabama; Children's Hospital of Alabama, Birmingham, Alabama.
authors.forename: Saurabh
authors.initials: S
authors.lastname: Guleria
authors.affiliation: University of Alabama at Birmingham, Birmingham, Alabama; Children's Hospital of Alabama, Birmingham, Alabama. Electronic address: mgoyal@peds.uab.edu.
authors.forename: Monisha
authors.initials: M
authors.lastname: Goyal
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D065705:Hemimegalencephaly / Q000209:etiology* / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D038421:Hemispherectomy / Q000379:methods*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D049268:Positron-Emission Tomography; D013226:Status Epilepticus / Q000209:etiology / Q000235:genetics / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000150:complications* / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D000077004:Tuberous Sclerosis Complex 1 Protein; D025521:Tumor Suppressor Proteins / Q000235:genetics
chemical_list: C000624650:TSC1 protein, human; D000077004:Tuberous Sclerosis Complex 1 Protein; D025521:Tumor Suppressor Proteins
keywords: Neurocutaneous syndrome; epilepsy surgery; functional hemispherectomy; hemispherectomy; intractable epilepsy; tuberous sclerosis