ILAE

 Proposal  for  Revised  Terminology  for  Organiza:on  of  Seizures  and  Epilepsies  2010  
Classifica)on  of  Seizures  

Generalized  seizures   Focal  seizures   Unknown  

Arising  within  and  rapidly  engaging   Origina=ng  within  networks   Insufficient  evidence    
bilaterally  distributed  networks   limited  to  one  hemisphere   to  characterize  as  
 focal,  generalized  or  both  

Tonic-­‐Clonic   Absence   Clonic   Tonic   Atonic   Myoclonic   Characterized  according  to  one   -­‐  Epilep:c  Spasms  
-  Myoclonic   or  more  features:   -­‐  Other  
-  Myoclonic-­‐atonic   Aura  
-  Myoclonic-­‐tonic   Motor  
Autonomic  
Typical   Absence  with     Atypical   Awareness/Responsiveness:  
special  features   altered  (dyscogni:ve)  or  retained  
-  Myoclonic  absence  
-  Eyelid  Myoclonia   May  evolve  to  

Bilateral  convulsive  seizure  

Changes  in  terminology  and  concepts  

New  Term  and  Concept   Examples   Old  Term  and  Concept  
E)ology  
Gene:c:  gene=c  defect  directly  contributes  to  the  epilepsy  and  seizures   Channelopathies,  Glut1  deficiency,  etc.   Idiopathic:  presumed  gene=c  
are  the  core  symptom  of  the  disorder  

Structural-­‐metabolic:  caused  by  a  structural  or  metabolic  disorder  of  the   Tuberous  sclerosis,  cor=cal  malforma=ons,   Symptoma:c:  secondary  to  a  known  or  presumed  disorder  
brain   etc.   of  the  brain  

Unknown:  the  cause  is  unknown  and  might  be  gene=c,  structural  or   Cryptogenic:  presumed  symptoma=c  
metabolic  

Terminology                              Terms  no  longer  recommended  

Self-­‐limited:  tendency  to  resolve  spontaneously  with  =me   Benign  
Pharmacoresponsive:  highly  likely  to  be  controlled  with  medica=on   Catastrophic  

Focal  seizures:  seizure  semiology  described  according  to  specific  subjec=ve  (auras),  motor,   Complex  par:al  
autonomic,  and  dyscogni=ve  features   Simple  par:al  
Evolving  to  a  bilateral  convulsive  seizure:  eg.  tonic,  clonic,  tonic-­‐clonic   Secondarily  generalized  

References:  1.Berg  AT  et  al.  Revised  terminology  and  concepts  for  organiza)on  of  seizures  and  epilepsies:  report  of  the  ILAE  Commission  on  Classifica)on  and  Terminology,  2005-­‐2009.    Epilepsia  2010;51:676-­‐685.      
2.Berg  AT,  Cross  JH.  Lancet  2010:9;459-­‐61.    3.Blume  WT  et  al.  Glossary  of  descrip)ve  terminology  for  ictal  semiology:  Report  of  the  ILAE  task  force  on  classifica)on  and  terminology.    Epilepsia  2001:42;1212-­‐1218.    
 ILAE  Proposal  for  Revised  Terminology  for  Organiza:on  of  Seizures  and  Epilepsies  2010  
Electroclinical  Syndromes  and  Other  Epilepsies  Grouped  by  Specificity  of  Diagnosis  

Electroclinical  syndromes    

One  example  of  how  syndromes  can  be  organized:  

Arranged  by  typical  age  at  onset*  

Neonatal  period   Infancy   Childhood   Adolescence  –  Adult   Variable  age  at  onset  
-  Benign  neonatal   -  Febrile  seizures^,  Febrile   -  Febrile  seizures^,  Febrile  seizures  plus  (FS+)   -  Juvenile  absence  epilepsy   -  Familial  focal  epilepsy  
seizures^   seizures  plus  (FS+)   -  Early  onset  childhood  occipital  epilepsy   (JAE)   with  variable  foci  
-  Benign  familial   -  Benign  infan)le  epilepsy   (Panayiotopoulos  syndrome)   -  Juvenile  myoclonic  epilepsy   (childhood  to  adult)  
neonatal  epilepsy   -  Benign  familial  infan)le   -  Epilepsy  with  myoclonic  atonic  (previously   (JME)   -  Progressive  
(BFNE)   epilepsy  (BFIE)   asta)c)  seizures   -  Epilepsy  with  generalized   myoclonus  epilepsies  
-  Ohtahara  syndrome   -  West  syndrome   -  Childhood  absence  epilepsy  (CAE)   tonic-­‐clonic  seizures  alone   (PME)  
-  Early  Myoclonic   -  Dravet  syndrome   -  Benign  epilepsy  with  centrotemporal  spikes   -  Autosomal  dominant   -  Reflex  epilepsies  
encephalopathy   -  Myoclonic  epilepsy  in   (BECTS)   epilepsy  with  auditory  
(EME)   infancy  (MEI)   -  Autosomal  dominant  nocturnal  frontal  lobe   features  (ADEAF)  
-  Myoclonic  encephalopathy   epilepsy  (ADNFLE)   -  Other  familial  temporal  lobe  
in  nonprogressive  disorders   -  Late  onset  childhood  occipital  epilepsy   epilepsies  
-  Epilepsy  of  infancy  with   (Gastaut  type)  
migra)ng  focal  seizures   -  Epilepsy  with  myoclonic  absences  
-  Lennox-­‐Gastaut  syndrome  (LGS)  
-  Epilep)c  encephalopathy  with  con)nuous  
spike-­‐and-­‐wave  during  sleep  (CSWS)+  
-  Landau-­‐Kleffner  syndrome  (LKS)  

Dis:nc:ve  constella:ons/surgical  syndromes   Nonsyndromic  epilepsies**  

Dis:nc:ve  constella:ons/Surgical  syndromes   Epilepsies  aRributed  to  and  organized  by     Epilepsies  of  
-  Mesial  temporal  lobe  epilepsy  with  hippocampal   structural-­‐metabolic  causes   unknown  cause  
sclerosis  (MTLE  with  HS)   -  Malforma)ons  of  cor)cal  development  
-  Rasmussen  syndrome   (hemimegalencephaly,  heterotopias,  etc.)  
-  Gelas)c  seizures  with  hypothalamic  hamartoma   -  Neurocutaneous  syndromes  (tuberous  sclerosis  
-  Hemiconvulsion-­‐hemiplegia-­‐epilepsy   complex,  Sturge-­‐Weber,  etc.)  
-  Tumor,  infec)on,  trauma,  angioma,  antenatal  and  
perinatal  insults,  stroke,  etc  

This  Proposal  is  a  work  in  progress…..  

*  The  arrangement  of  eletroclinical  syndromes  does  not  reflect  e)ology,   We  welcome  your  thoughts  on  this  proposal.  Please  visit  our  Classifica:on  &  Terminology  
^  Not  tradi)onally  diagnosed  as  epilepsy  
+  Some)mes  referred  to  as  Electrical  Status  Epilep)cus  during  Slow  Sleep  (ESES)  
Discussion  Group  at:  hRp://community.ilae-­‐epilepsy.org/home/  to  login  and    register  
**  Forms  of  epilepsies  not  mee)ng  criteria  for  specific  syndromes  or  constella)ons   your  comments.