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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,
Language
and structure for organizing epilepsies
modifications
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
- Myoclonic
Seizure types thought to
- Myoclonic atonic
occur within and result from
- Myoclonic tonic
rapid engagement of
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

g. by age, etiology, seizure type, EEG abnormality


Future efforts in classification will depend on a biological mechanisms as these are understood

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