Dr Tina Williams PLEAT 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 recordings  Events 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

 IGeneralized 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 years, peak 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 1 st 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?