Dr Tina WilliamsPLEAT

Frimley Park Hospital June 2011

Condition with recurrent, unprovoked seizures

Old Classification : ILAE 1989

Partial (Simple or Complex) and Generalised

Axes 1 to 4*

More than just identifying seizure type

Attempting to identify a clear Epilepsy Syndrome

Specific Treatment, Prognosis

Think Axes:◦Description – Sz or not, videos◦ ◦Seizure Type

◦Features of Identifiable Syndrome?

◦Specific Rx – NICE 2004, BNFc

Description of episodes – Signs and Symptoms, Standardised Terminology

Video recordingsEvents related

Focal (previously ‘partial’) seizure - initial activation of only part of one cerebral hemisphere occurs (although may generalize). (Luders 2001)

Generalized seizure – discharge from both cerebral hemispheres occurs. Loss of Consciousness may occur (Luders 2001)

Seizure Types

I Self Limited◦ Focal◦ Generalised

II Continuous (status epilepticus)◦ Focal◦ Generalised

 

I Generalized seizures

Tonic-clonic seizures

Clonic seizures

Typical absence seizures

Atypical absence seizures

Tonic seizures

Myoclonic seizures

Atonic seizures

 

II Focal seizures

Focal sensory seizures

Focal motor seizures (tonic/clonic/myoclonic

seizures)

With typical automatisms (Complex Partial Seizures)

 III Secondarily Generalized seizures

Origin of symptoms and signs in focal seizures - Visual display over the dominant hemispheres

I Generalized status epilepticus

II Focal status epilepticus

Epilepsia partialis continua

Aura continua Hemiconvulsive status

with hemiparesis

Epilepsy Syndromes

An epileptic disorder or condition characterised by cluster of signs and symptoms customarily occurring together.

List not exhaustive

Benign Idiopathic focal epilepsies of infancy and

childhood Familial focal epilepsies (autosomal dominant) Idiopathic generalized epilepsies

Malignant Symptomatic focal epilepsies (Focal Pathology

eg tumour, bleed, infarct) Epileptic encephalopathies

 Idiopathic focal epilepsies of infancy and childhood

Benign infantile seizures Benign childhood epilepsy

Familial focal epilepsies (autosomal dominant)

Benign familial neonatal seizures

Benign familial infantile seizures

Autosomal dominant nocturnal frontal lobe epilepsy

Idiopathic generalized epilepsies

Benign myoclonic epilepsy in infancy

Childhood absence epilepsy

Epilepsy with generalized tonic-clonic seizures only

Epileptic encephalopathies

Early myoclonic encephalopathy

West syndrome

Lennox-Gaustaut syndrome

Landau-Kleffner syndrome

Aetiology or Underlying Cause

Neurocutaneous Disorders Malformations due to abnormal cortical developments

Other cerebral malformations Tumours Bleeds/ Infarcts Chromosomal abnormalities Inherited metabolic disorders Pre/ perinatal ischaemic/ anoxic lesions or cerebral infections

Postnatal infections

Benign Rolandic Epilepsy Idiopathic, otherwise healthy children. EEG - high-voltage centrotemporal spikes

often followed by a slow wave. Onset usually 4-11yrs, peaks at 5-9yrs Boys:Girls - 6:4 Unilateral somatosensory aura, Speech

arrest, conscious in most cases Secondary generalisation: tonic/T-C

common May be nocturnal Rx – Carbamazepine usually Prognosis good

30% have a family Hx of Epilepsy Onset from 6 months – 3 years of age No other seizure types Usually upper extremities and head EEG may be normal, sleep EEG may show

changes. Prognosis : Good, up to 50% may have

developmental/ language delay

Onset 4-10 yrs; Peak 5-7yrs Female > Male Mild automatisms frequent, but major motor

involvement diagnosis. The EEG - characteristic "typical 3Hz spike-

wave" discharges. Prognosis is excellent in well-defined cases

of CAE with most patients "growing out" of their epilepsy

Onset 10-17 years, peak 10-12 years Male=Female More sporadic than CAE > 75% have tonic-clonic seizures EEG - spike-wave discharges most

prominent in the frontal region. Faster (3.5 Hz to 4.5 Hz) than in typical childhood absence epilepsy.

Prognosis: Respond well to Rx – Valproate, Ethosuxamide. If no other factors, prognosis good.

Usually abnormal brain eg TS - Invx Triad: infantile spasms, EEG pattern termed

hypsarrhythmia, and mental retardation Spasms affecting head and upper

extremities lasting 5-20seconds, clustering, sleep times

Rx: ACTH/Steroids/ Vigabatrin Prognosis – Seizure control often.

Developmental delay progresses

Childhood Epileptic Encephalopathy 1-4% of childhood epilepsies Multiple sz types, Dev Delay/regression

often follows EEG: Gen slow spike+wave discharges Common sz: tonic-axial, drops, atypical

absences, but can be myoclonic, gen tonic-clonic, focal.

Often resistant to Rx. Surgery to remove corpus callosum/

lobectomy works for select grous Ketogenic diet works in some

Onset 3-7 yrs Rare disorder Loss of expressive language → loss of

speech Rx – Speech Rx, AED Prognosis: Variable, Age of onset after 6yrs

is better

Females Loss of skills – speech, purposeful hand

movements Develop stereotypic Hand movements Onset 3months-3 yrs Prognosis poor - Regression

Severe Myoclonic Epilepsy of Infancy Begins in 1st year of life Febrile seizures, status then become afebrile Can be generalized, myoclonic, atypical

abscences, clonic, tonic-clonic, or focal EEGs - generalized and focal and multifocal

anomalies Rx – Difficult control Prognosis – Poor neurological outcome. 50%

severe

Think Axes:◦Description – Sz or not, videos◦ ◦Seizure Type

◦Features of Identifiable Syndrome?

◦Specific Rx – NICE 2004, BNFc

Questions?