Causes of Intractable Childhood Epilepsy

Listed below are the epilepsy syndromes recognized by the International League against Epilepsy (ILAE) .  The  syndromes in red font are often associated with drug resistance or intractability and have additional information listed on the ICE website under childhood epilepsies.  The link to the ILAE website for more information about the other syndromes is: www.ilae-epilepsy.org  

It is important to remember the inborn errors of metabolism that may lead to intractable epilepsy in infants and with early treatment, can change the course of illness and improve the outcome of these children, who may be able to lead a normal life if the deficiency  is treated promptly and appropriately:

  • Glucose Transporter Type 1 Deficiency Syndrome
  • Familial Pyridoxine(vitamin B6)-Dependent Epilepsy
  • Inborn errors of metabolism

 

International League Against Epilepsy (ILAE)

Commission on Classification and Terminology

Table 3: Electro-clinical syndromes and other epilepsies

 Electro-clinical syndromes arranged by age at onset *

Neonatal period
        Benign familial neonatal seizures (BFNS)
        Early myoclonic encephalopathy (EME)
        Ohtahara syndrome

Infancy
        Migrating partial seizures of infancy
        West syndrome (infantile spasms)
        Myoclonic epilepsy in infancy (MEI)
        Benign infantile seizures
        Benign familial infantile seizures
        Dravet syndrome
        Myoclonic encephalopathy in nonprogressive disorders

Childhood
        Febrile seizures plus (FS+) (can start in infancy)
        Early onset benign childhood occipital epilepsy (Panayiotopoulos type)
        Epilepsy with myoclonic atonic (previously astatic) seizures
        Benign epilepsy with centrotemporal spikes (BECTS)
        Autosomal-dominant nocturnal frontal lobe epilepsy (ADNFLE)
        Late onset childhood occipital epilepsy (Gastaut type)
        Epilepsy with myoclonic absences
        Lennox-Gastaut syndrome
        Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS)
                      including: Landau-Kleffner syndrome (LKS)
        Childhood absence epilepsy (CAE)

Adolescence – Adult
        Juvenile absence epilepsy (JAE)
        Juvenile myoclonic epilepsy (JME)
        Epilepsy with generalized tonic-clonic seizures alone
        Progressive myoclonus epilepsies (PME)
        Autosomal dominant partial epilepsy with auditory features (ADPEAF)
        Other familial temporal lobe epilepsies

Less Specific Age Relationship *
       
Familial focal epilepsy with variable foci (childhood to adult)
        Reflex epilepsies   

Distinctive Constellations
        Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE with HS)
        Rasmussen syndrome
        Gelastic seizures with hypothalamic hamartoma

Epilepsies that do not fit into any of these diagnostic categories can be distinguished first on the basis of the presence or absence of a known structural or metabolic condition (presumed cause) and then on the basis of the primary mode of seizure onset (generalized versus focal).

Epilepsies attributed to and organized by structural-metabolic causes (any of these can be intractable)
Malformations of Cortical development (hemimeganencephaly, hetertopias etc)
Neurocutaneous syndromes (Tuberous sclerosis complex, Sturge-Weber, etc)
Tumor
Infection , toxins

Trauma
Angioma
Peri-natal insults
Stroke
Etc.

Epilepsies of unknown cause

Conditions with epileptic seizures that are traditionally not diagnosed as a form of epilepsy per se.
        Benign neonatal seizures (BNS)
        Febrile seizures (FS)

*This arrangement does not reflect etiology.

 

Additionally, there are several syndromes and medical conditions in children that are associated with intractable epilepsy.  Listed below are these syndromes and links to organizations that support them:

Tubular Sclerosis www.tsaalliance.org  

Sturge Weber syndrome  www.sturge-weber.org

Fragile X syndrome www.fragilex.org

Down syndrome  www.ndss.org

Angelman’s syndrome  www.angelman.org

Rett syndrome  www.rettsyndrome.org

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