Epilepsy
syndromes
There
are many different epilepsy syndromes, each presenting with its own
unique combination of seizure type, typical age of onset, EEG findings,
treatment, and prognosis. The most widespread classification of the
epilepsies divides epilepsy syndromes by location or distribution of
seizures (as revealed by the appearance of the seizures and by EEG)
and by cause. Syndromes are divided into localization-related epilepsies,
generalized epilepsies, or epilepsies of unknown localization.
Localization-related epilepsies, sometimes
termed partial or focal epilepsies, arise from an epileptic focus, a
small portion of the brain that serves as the irritant driving the epileptic
response. Generalized epilepsies, in contrast, arise from many independent
foci (multifocal epilepsies) or from epileptic circuits that involve
the whole brain. Epilepsies of unknown localization remain unclear whether
they arise from a portion of the brain or from more widespread circuits.
Epilepsy syndromes are further divided
by presumptive cause: idiopathic, symptomatic, and cryptogenic. Idiopathic
epilepsies are generally thought to arise from genetic abnormalities
that lead to alteration of basic neuronal regulation. Symptomatic epilepsies
arise from the effects of an epileptic lesion, whether that lesion is
focal, such as a tumor, or a defect in metabolism causing widespread
injury to the brain. Cryptogenic epilepsies involve a presumptive lesion
that is otherwise difficult or impossible to uncover during evaluation.
Some epileptic syndromes are difficult
to fit within this classification scheme and fall in the unknown localization/etiology
category. People who only have had a single seizure, or those with seizures
that occur only after specific precipitants ("provoked seizures"),
have "epilepsies" that fall into this category. Febrile convulsions
are an example of seizures bound to a particular precipitant. Landau-Kleffner
syndrome is another epilepsy which, because of its variety of EEG distributions,
falls uneasily in clear categories. More confusingly, certain syndromes
like West syndrome featuring seizures such as Infantile spasms can be
classified as idiopathic, syndromic, or cryptogenic depending on cause
and can arise from both focal or generalized epileptic lesions.