title: Treatment of Focal Status Epilepticus plus Epileptic Spasms with Leukotomy in an Infant with TSC 1 Mutation: A Case Study.
abstract: BACKGROUND
  Focal status epilepticus and catastrophic epilepsy are not rare in infants. Epilepsy surgery can be safely done in selected infants to cure epilepsy.
  
  
  METHODS
  We report on an infant who began having drug-resistant status epilepticus at 2 weeks of age and developed epileptic spasms. We discuss in detail how the clinical and electroencephalographic data were used to reach a consensus for epilepsy surgery and why a particular surgical approach was preferred over other alternatives.
  
  
  METHODS
  Presurgical evaluation consisted of 32-channel scalp video EEG using the standard 10-20 system of electrodes, 3-Tesla brain magnetic resonance imaging, and 18 F-fluorodeoxyglucose positron emission tomography. The surgery consisted of resection of the extensive epileptogenic lesion, in addition to disconnection of the left frontal lobe anterior to the motor strip.
  
  
  RESULTS
  The infant underwent epilepsy surgery at three months of age. At two-year follow up, she remained seizure free, with no focal motor deficit and the epileptic encephalopathy resolved. The disconnected left frontal lobe shows epileptiform discharges restricted to the disconnected tissue.
  
  
  CONCLUSIONS
  We highlight the importance of epilepsy surgery in selected infants to achieve seizure freedom and to reverse epileptic encephalopathy. In the process, we demonstrate how epileptic spasms, although clinically and electrographically generalized, resolved after disconnecting the epileptogenic zone.
authors.forename: Jun T
authors.initials: JT
authors.lastname: Park
authors.forename: Asim M
authors.initials: AM
authors.lastname: Shahid
authors.forename: Jonathan P
authors.initials: JP
authors.lastname: Miller
authors.forename: Hans O
authors.initials: HO
authors.lastname: Lüders
mesh_terms: D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D007231:Infant, Newborn; D011612:Psychosurgery / Q000379:methods*; D013036:Spasms, Infantile / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery*; D013226:Status Epilepticus / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery*; D016896:Treatment Outcome; D000077004:Tuberous Sclerosis Complex 1 Protein; D025521:Tumor Suppressor Proteins / Q000235:genetics*
chemical_list: D000077004:Tuberous Sclerosis Complex 1 Protein; D025521:Tumor Suppressor Proteins