dr tina williams pleat frimley park hospital june 2011
TRANSCRIPT
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Dr Tina WilliamsPLEAT
Frimley Park Hospital June 2011
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Condition with recurrent, unprovoked seizures
Old Classification : ILAE 1989
Partial (Simple or Complex) and Generalised
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Axes 1 to 4*
More than just identifying seizure type
Attempting to identify a clear Epilepsy Syndrome
Specific Treatment, Prognosis
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Think Axes:◦Description – Sz or not, videos◦ ◦Seizure Type
◦Features of Identifiable Syndrome?
◦Specific Rx – NICE 2004, BNFc
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Description of episodes – Signs and Symptoms, Standardised Terminology
Video recordingsEvents related
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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)
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Seizure Types
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I Self Limited◦ Focal◦ Generalised
II Continuous (status epilepticus)◦ Focal◦ Generalised
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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
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Origin of symptoms and signs in focal seizures - Visual display over the dominant hemispheres
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I Generalized status epilepticus
II Focal status epilepticus
Epilepsia partialis continua
Aura continua Hemiconvulsive status
with hemiparesis
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Epilepsy Syndromes
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An epileptic disorder or condition characterised by cluster of signs and symptoms customarily occurring together.
List not exhaustive
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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
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Idiopathic focal epilepsies of infancy and childhood
Benign infantile seizures Benign childhood epilepsy
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Familial focal epilepsies (autosomal dominant)
Benign familial neonatal seizures
Benign familial infantile seizures
Autosomal dominant nocturnal frontal lobe epilepsy
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Idiopathic generalized epilepsies
Benign myoclonic epilepsy in infancy
Childhood absence epilepsy
Epilepsy with generalized tonic-clonic seizures only
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Epileptic encephalopathies
Early myoclonic encephalopathy
West syndrome
Lennox-Gaustaut syndrome
Landau-Kleffner syndrome
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Aetiology or Underlying Cause
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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
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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
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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
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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
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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.
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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
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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
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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
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Females Loss of skills – speech, purposeful hand
movements Develop stereotypic Hand movements Onset 3months-3 yrs Prognosis poor - Regression
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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
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Think Axes:◦Description – Sz or not, videos◦ ◦Seizure Type
◦Features of Identifiable Syndrome?
◦Specific Rx – NICE 2004, BNFc
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Questions?