THE ORGANIZATION OF THE

EPILEPSIES 2010

ILAE Commission for Classification and Terminology

Purpose of the International Classification

of Seizures and Epilepsies
To provide a common international
terminology and classification
Largely for clinical (treatment) purposes
Purpose of classification: to organize items

according to their fundamental relationships

2005-2009 Commission Report,

Epilepsia 2010;51:676-685

Main changes, modifications

Language and structure for organizing epilepsies
Generalized versus Focal Seizures

Etiology
Diagnostic specificity
New recommended terms
Organization

NO changes to electroclinical syndromes

A diagnosis can be made as previously

eg Lennox-Gastaut syndrome, childhood absence epilepsy

A diagnosis is not the same as a classification

Focal reconceptualized
For seizures:
Focal epileptic seizures are conceptualized as

originating within networks limited to one

hemisphere. These may be discretely
localized or more widely distributed.

Focal seizures
Blume et al, Epilepsia 2001

Without impairment of consciousness or awareness

Previous term: simple partial
With observable motor or autonomic components
eg. focal clonic, autonomic, hemiconvulsive
With subjective sensory or psychic phenomena
Aura - specific types
Where alteration of cognition is major feature
Previous term: complex partial
Dyscognitive

Focal seizures
Blume et al, Epilepsia 2001

Evolving to bilateral, convulsive seizure

Previous terms: partial seizure secondarily generalized;
secondarily generalized tonic-clonic seizure
With tonic, clonic or tonic and clonic components

Generalized - reconceptualized
For seizures
Generalized epileptic seizures are
conceptualized as originating at some point
within, and rapidly engaging, bilaterally
distributed networks. can include cortical
and subcortical structures, but not
necessarily include the entire cortex.

Generalized Seizures
Tonic-clonic (in any combination)
Absence
- Typical
- Atypical
- Absence with special features
Myoclonic absence
Eyelid myoclonia
Myoclonic
Seizure types thought to
- Myoclonic
occur within and result from
- Myoclonic atonic
rapid engagement of
- Myoclonic tonic

bilaterally distributed systems

Clonic
Tonic
Atonic

Recommended terminology for

etiology
Use terms which mean what they say:
Genetic
Structural-Metabolic
Unknown
Previously used terms denoting old concepts:
Idiopathic, cryptogenic, symptomatic

Genetic
Concept: the epilepsy is the direct result of a
known or inferred genetic defect(s). Seizures are
the core symptom of the disorder.
Evidence: Specific molecular genetic studies (well
replicated) or evidence from appropriately
designed family studies.
Genetic does not exclude the possibility of
environmental factors contributing

Structural-Metabolic
Concept: There is a distinct other structural or

metabolic condition or disease present.

eg. Tuberous sclerosis

Evidence: Must have demonstrated a

substantially increased risk of developing

epilepsy in association with the condition.

Unknown
Concept: The nature of the underlying cause is
as yet unknown.

New recommended terminology

Previously used terms no longer preferred
Classification as focal or generalized epilepsies
not always appropriate
use when appropriate
Catastrophic - emotionally laden term
Benign - does not recognize that co-morbidities occur,
this term is still used in syndrome names
Recommended terms
Self-limited: high likelihood of spontaneous remission
Pharmacoresponsive

Future developments in the

Classification of the Epilepsies
New terminology and concepts update the classification

to be consistent with current understanding of the

epilepsies in clinical practice
Currently there is no biologically based classification of

the epilepsies
Epilepsies can be organized in a flexible,

multidimensional way depending on the purpose

eg. by age, etiology, seizure type, EEG abnormality
Future efforts in classification will depend on a biological

mechanisms as these are understood