Seizure outcomes in children with Rasmussen’s encephalitis undergoing resective or hemispheric epilepsy surgery: an individual participant data meta-analysis

Restricted access

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00

OBJECTIVE

The objective of this study was to perform an individual participant data meta-analysis to identify preoperative factors associated with a good seizure outcome in children with Rasmussen’s encephalitis (RE) undergoing resective or hemispheric epilepsy surgery.

METHODS

Electronic databases (PubMed, Web of Science, CINAHL) were searched with no language or date restrictions to identify cohort studies of consecutive participants undergoing resective surgery that reported seizure outcomes. The authors recorded all preoperative factors that could plausibly be associated with seizure outcomes and used Cox regression analysis to identify which of these variables were associated with seizure freedom (i.e., Engel class I).

RESULTS

Of 720 citations, 19 articles reporting on 187 participants were eligible. Seizure freedom (Engel class I) was observed in 113 participants (60.4%). On univariate analyses, younger age at disease onset (hazard ratio [HR] 0.906, p = 0.001), younger age at surgery (HR 0.928, p < 0.001), shorter time to surgery (HR 0.921, p = 0.001), and hemispherectomy (HR 0.283, p < 0.001) were all associated with longer time to postoperative seizure recurrence. Additionally, multivariable analysis including the aforementioned variables showed that younger age at surgery (HR 0.946, p = 0.043) and hemispherectomy (HR 0.297, p < 0.001) were independently and significantly associated with a greater time to seizure recurrence and longer duration of seizure freedom.

CONCLUSIONS

The majority of pediatric patients undergoing resective or hemispheric surgery for RE achieve good seizure outcome. Although small retrospective cohort studies are inherently prone to bias, the best available evidence utilizing individual participant data suggests hemispheric surgery and younger age at surgery are associated with good seizure outcomes following epilepsy surgery. Large, multicenter observational studies with long-term follow-up are required to evaluate the risk factors identified in this review.

ABBREVIATIONS AED = antiepileptic drug; CI = confidence interval; HR = hazard ratio; IPD = individual participant data; IQR = interquartile range; RE = Rasmussen’s encephalitis.
Article Information

Contributor Notes

Correspondence Aria Fallah: University of California, Los Angeles, CA. afallah@mednet.ucla.edu.INCLUDE WHEN CITING Published online December 6, 2019; DOI: 10.3171/2019.9.PEDS19380.Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
Headings
References
  • 1

    Althausen AGleissner UHoppe CSassen RBuddewig Svon Lehe M: Long-term outcome of hemispheric surgery at different ages in 61 epilepsy patients. J Neurol Neurosurg Psychiatry 84:5295362013

    • Search Google Scholar
    • Export Citation
  • 2

    Bharti NBatra YKKaur H: Paediatric perioperative cardiac arrest and its mortality: database of a 60-month period from a tertiary care paediatric centre. Eur J Anaesthesiol 26:4904952009

    • Search Google Scholar
    • Export Citation
  • 3

    Bien CGTiemeier HSassen RKuczaty SUrbach Hvon Lehe M: Rasmussen encephalitis: incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins. Epilepsia 54:5435502013

    • Search Google Scholar
    • Export Citation
  • 4

    Bien CGWidman GUrbach HSassen RKuczaty SWiestler OD: The natural history of Rasmussen’s encephalitis. Brain 125:175117592002

    • Search Google Scholar
    • Export Citation
  • 5

    Bulteau CGrosmaitre CSave-Pédebos JLeunen DDelalande ODorfmüller G: Language recovery after left hemispherotomy for Rasmussen encephalitis. Epilepsy Behav 53:51572015

    • Search Google Scholar
    • Export Citation
  • 6

    Caraballo RHFortini SCersósimo RMonges SPasteris MCGomez M: Rasmussen syndrome: an Argentinean experience in 32 patients. Seizure 22:3603672013

    • Search Google Scholar
    • Export Citation
  • 7

    Chandra PSPadma VMShailesh GChandreshekar BSarkar CTripathi M: Hemispherotomy for intractable epilepsy. Neurol India 56:1271322008

    • Search Google Scholar
    • Export Citation
  • 8

    Cook SWNguyen STHu BYudovin SShields WDVinters HV: Cerebral hemispherectomy in pediatric patients with epilepsy: comparison of three techniques by pathological substrate in 115 patients. J Neurosurg 100 (2 Suppl Pediatrics):1251412004

    • Search Google Scholar
    • Export Citation
  • 9

    Delalande OBulteau CDellatolas GFohlen MJalin CBuret V: Vertical parasagittal hemispherotomy: surgical procedures and clinical long-term outcomes in a population of 83 children. Neurosurgery 60 (2 Suppl 1):ONS19ONS322007

    • Search Google Scholar
    • Export Citation
  • 10

    Devlin AMCross JHHarkness WChong WKHarding BVargha-Khadem F: Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence. Brain 126:5565662003

    • Search Google Scholar
    • Export Citation
  • 11

    Dorfer COchi ASnead OC IIIDonner EHolowka SWidjaja E: Functional hemispherectomy for catastrophic epilepsy in very young infants: technical considerations and complication avoidance. Childs Nerv Syst 31:210321092015

    • Search Google Scholar
    • Export Citation
  • 12

    Döring SCross HBoyd SHarkness WNeville B: The significance of bilateral EEG abnormalities before and after hemispherectomy in children with unilateral major hemisphere lesions. Epilepsy Res 34:65731999

    • Search Google Scholar
    • Export Citation
  • 13

    Fallah AGuyatt GHSnead OC IIIEbrahim SIbrahim GMMansouri A: Predictors of seizure outcomes in children with tuberous sclerosis complex and intractable epilepsy undergoing resective epilepsy surgery: an individual participant data meta-analysis. PLoS One 8:e535652013

    • Search Google Scholar
    • Export Citation
  • 14

    Fallah AWang ACWeil AGIbrahim GMMansouri ABhatia S: Predictors of outcome following cerebral aqueductoplasty: an individual participant data meta-analysis. Neurosurgery 78:2852962016

    • Search Google Scholar
    • Export Citation
  • 15

    Fuerst DShah JShah AWatson C: Hippocampal sclerosis is a progressive disorder: a longitudinal volumetric MRI study. Ann Neurol 53:4134162003

    • Search Google Scholar
    • Export Citation
  • 16

    Granata TMatricardi SRagona FFreri ECasazza MVillani F: Hemispherotomy in Rasmussen encephalitis: long-term outcome in an Italian series of 16 patients. Epilepsy Res 108:110611192014

    • Search Google Scholar
    • Export Citation
  • 17

    Griessenauer CJSalam SHendrix PPatel DMTubbs RSBlount JP: Hemispherectomy for treatment of refractory epilepsy in the pediatric age group: a systematic review. J Neurosurg Pediatr 15:34442015

    • Search Google Scholar
    • Export Citation
  • 18

    Guan YChen SLiu CDu XZhang YChen S: Timing and type of hemispherectomy for Rasmussen’s encephalitis: analysis of 45 patients. Epilepsy Res 132:1091152017

    • Search Google Scholar
    • Export Citation
  • 19

    Guan YZhou JLuan GLiu X: Surgical treatment of patients with Rasmussen encephalitis. Stereotact Funct Neurosurg 92:86932014

  • 20

    Hamad APCaboclo LOCenteno RCosta LVLadeia-Frota CJunior HC: Hemispheric surgery for refractory epilepsy in children and adolescents: outcome regarding seizures, motor skills and adaptive function. Seizure 22:7527562013

    • Search Google Scholar
    • Export Citation
  • 21

    Hertz-Pannier LChiron CJambaqué IRenaux-Kieffer VVan de Moortele PFDelalande O: Late plasticity for language in a child’s non-dominant hemisphere: a pre- and post-surgery fMRI study. Brain 125:3613722002

    • Search Google Scholar
    • Export Citation
  • 22

    Hoffman CEOchi ASnead OC IIIWidjaja EHawkins CTisdal M: Rasmussen’s encephalitis: advances in management and patient outcomes. Childs Nerv Syst 32:6296402016

    • Search Google Scholar
    • Export Citation
  • 23

    Honavar MJanota IPolkey CE: Rasmussen’s encephalitis in surgery for epilepsy. Dev Med Child Neurol 34:3141992

  • 24

    Honda RKaido TSugai KTakahashi AKaneko YNakagwa E: Long-term developmental outcome after early hemispherotomy for hemimegalencephaly in infants with epileptic encephalopathy. Epilepsy Behav 29:30352013

    • Search Google Scholar
    • Export Citation
  • 25

    Hu WHZhang CZhang KShao XQZhang JG: Hemispheric surgery for refractory epilepsy: a systematic review and meta-analysis with emphasis on seizure predictors and outcomes. J Neurosurg 124:9529612016

    • Search Google Scholar
    • Export Citation
  • 26

    Jonas RNguyen SHu BAsarnow RFLoPresti CCurtiss S: Cerebral hemispherectomy: hospital course, seizure, developmental, language, and motor outcomes. Neurology 62:171217212004

    • Search Google Scholar
    • Export Citation
  • 27

    Kadish NEBast TReuner GWagner KMayer HSchubert-Bast S: epilepsy surgery in the first 3 years of life: predictors of seizure freedom and cognitive development. Neurosurgery 84:E368E3772019

    • Search Google Scholar
    • Export Citation
  • 28

    Korkman MGranström MLKantola-Sorsa EGaily EPaetau RLiukkonen E: Two-year follow-up of intelligence after pediatric epilepsy surgery. Pediatr Neurol 33:1731782005

    • Search Google Scholar
    • Export Citation
  • 29

    Lamb KScott WMensah AVaradkar SRobinson RCross H: Incidence, prevalence and clinical outcome of Rasmussen encephalitis in children from the United Kingdom: 002. Epilepsia 54 (Suppl 3):42013 (Abstract)

    • Search Google Scholar
    • Export Citation
  • 30

    Liu SAn NYang HYang MHou ZLiu L: Pediatric intractable epilepsy syndromes: reason for early surgical intervention. Brain Dev 29:69782007

    • Search Google Scholar
    • Export Citation
  • 31

    Moosa ANVGupta AJehi LMarashly ACosmo GLachhwani D: Longitudinal seizure outcome and prognostic predictors after hemispherectomy in 170 children. Neurology 80:2532602013

    • Search Google Scholar
    • Export Citation
  • 32

    Moosa ANVJehi LMarashly ACosmo GLachhwani DWyllie E: Long-term functional outcomes and their predictors after hemispherectomy in 115 children. Epilepsia 54:177117792013

    • Search Google Scholar
    • Export Citation
  • 33

    Panigrahi MKrishnan SSVooturi SVadapalli RSomayajula SJayalakshmi S: An observational study on outcome of hemispherotomy in children with refractory epilepsy. Int J Surg 36 (Pt B):4774822016

    • Search Google Scholar
    • Export Citation
  • 34

    Paolicchi JMJayakar PDean PYaylali IMorrison GPrats A: Predictors of outcome in pediatric epilepsy surgery. Neurology 54:6426472000

    • Search Google Scholar
    • Export Citation
  • 35

    Pulsifer MBrandt JJacobs DVining EFreeman J: Developmental and behavioral outcome of cerebral hemispherectomy. Epilepsia 36 (Suppl 4):1441995 (Abstract)

    • Search Google Scholar
    • Export Citation
  • 36

    Ramesha KNRajesh BAshalatha RKesavadas CAbraham MRadhakrishnan VV: Rasmussen’s encephalitis: experience from a developing country based on a group of medically and surgically treated patients. Seizure 18:5675722009

    • Search Google Scholar
    • Export Citation
  • 37

    Rasmussen TOlszewski JLloydsmith D: Focal seizures due to chronic localized encephalitis. Neurology 8:4354451958

  • 38

    Sinclair DBAronyk KSnyder TMcKean JDSWheatley MGross D: Extratemporal resection for childhood epilepsy. Pediatr Neurol 30:1771852004

    • Search Google Scholar
    • Export Citation
  • 39

    Stiles J: Neural plasticity and cognitive development. Dev Neuropsychol 18:2372722000

  • 40

    Terra-Bustamante VCMachado HRdos Santos Oliveira RSerafini LNSouza-Oliveira CEscorsi-Rosset S: Rasmussen encephalitis: long-term outcome after surgery. Childs Nerv Syst 25:5835892009

    • Search Google Scholar
    • Export Citation
  • 41

    Thomas SGChacko AGThomas MMBabu KSRussell PSSDaniel RT: Outcomes of disconnective surgery in intractable pediatric hemispheric and subhemispheric epilepsy. Int J Pediatr 2012:5278912012

    • Search Google Scholar
    • Export Citation
  • 42

    Thomas SGDaniel RTChacko AGThomas MRussell PSS: Cognitive changes following surgery in intractable hemispheric and sub-hemispheric pediatric epilepsy. Childs Nerv Syst 26:106710732010

    • Search Google Scholar
    • Export Citation
  • 43

    Tiret LNivoche YHatton FDesmonts JMVourc’h G: Complications related to anaesthesia in infants and children. Br J Anaesth 61:2632691988

    • Search Google Scholar
    • Export Citation
  • 44

    Tonini CBeghi EBerg ATBogliun GGiordano LNewton RW: Predictors of epilepsy surgery outcome: a meta-analysis. Epilepsy Res 62:75872004

    • Search Google Scholar
    • Export Citation
  • 45

    Topçu MTuranli GAynaci FMYalnızoğlu DSaatçi IYiğit A: Rasmussen encephalitis in childhood. Childs Nerv Syst 15:3954031999

    • Search Google Scholar
    • Export Citation
  • 46

    Tubbs RSNimjee SMOakes WJ: Long-term follow-up in children with functional hemispherectomy for Rasmussen’s encephalitis. Childs Nerv Syst 21:4614652005

    • Search Google Scholar
    • Export Citation
  • 47

    van Schooneveld MMJJennekens-Schinkel Avan Rijen PCBraun KPJvan Nieuwenhuizen O: Hemispherectomy: a basis for mental development in children with epilepsy. Epileptic Disord 13:47552011

    • Search Google Scholar
    • Export Citation
  • 48

    Varadkar SBien CGKruse CAJensen FEBauer JPardo CA: Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol 13:1952052014

    • Search Google Scholar
    • Export Citation
  • 49

    Villarejo-Ortega FGarcía-Fernández MFournier-Del Castillo CFabregate-Fuente MÁlvarez-Linera JDe Prada-Vicente I: Seizure and developmental outcomes after hemispherectomy in children and adolescents with intractable epilepsy. Childs Nerv Syst 29:4754882013

    • Search Google Scholar
    • Export Citation
  • 50

    Villemure JGRasmussen T: Functional hemispherectomy in children. Neuropediatrics 24:53551993

  • 51

    Wang DDBenkli BAuguste KIGarcia PASullivan JBarkovich AJ: 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 14:5735842014

    • Search Google Scholar
    • Export Citation
Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 192 192 160
Full Text Views 32 32 20
PDF Downloads 39 39 27
EPUB Downloads 0 0 0
PubMed
Google Scholar