Transcript Seizures

Neuromuscular Disease(2)
Epilepsy
Department of Pediatrics
Soochow University Affiliated Children’s Hospital
Seizures (p130)
Main contents:
1) Emphasize the clinical features of epileptic
seizure and epilepsy.
2) Introduce the management
of epilepsy and
status epilepticus.
3) Emphasize the features of febrile seizure.
4) Introduce the features of
seizure-like disorders.
Topic 1. Epileptic seizures
Seizure prevalence:
□Seizures occur in 0.5-1% of the population.
□if febrile seizures are included, affect up to 3-
5% of children.
□prevalence of epilepsy is 0.3%-0.6%.
Topic 1. Epileptic seizures
Clinical features:
□investigation will not reveal an underlying cause in the
majority of cases.
□involving episodic involuntary movement and
behavioural or sensory activity.
□often associating with loss of consciousness.
□with an manifestation of abnormal electrical activity in
the brain.
Topic 1. Epileptic seizures
In making the diagnosis of epilepsy:
□a detailed history is fundamental.
□the clinical examination is usually unremarkable, but
must exclude:
1) raised intracranial pressure.
2) hypertension
3) neurocutaneous stigmata
4) metabolic or storage disorder.
□EEG is important in the diagnosis of EP.
Topic 1. Epileptic seizures
EEG (electroencephalogram)
□is a recording of
the electrical activity of the
brain.
□may provide useful information on the
diagnosis of epilepsy, the seizure type, its
treatment and prognosis.
partial seizures (interictal : T3、T4)
partial seizures ( fit : FP1、F3)
Topic 2. Classification of seizures
Table Types of epileptic seizure
Partial
Generalised
Simple (1.1.SPS,1.2.SPS)
Tonic-clonic (5.1.GTCS,5.2.GTCS)
Complex (3.1.CPS,3.2.CPS)
Tonic (6.TS)
Secondary generalization (4.PS-SGTC)
Clonic (7.CS)
Myoclonic (8.MCS)
Absences (9.TAS,10.AAS)
Atonic (11.AS)
Infantile spasms
Epileptic seizures can be divided into partial or generalized types
Topic 2. Classification of seizures
Partial seizures
□Simple partial seizures (SPS):
1) brief tonic or clonic movements of the face
and extremities.
2) without impaired consciousness.
□Complex partial seizures (CPS):
1) strange sensations or complex semipurposeful movements.
2) with altered or impaired consciousness.
Topic 2. Classification of seizures
Absence seizures (typical absences, petit
mal)

usually affect girls.

sudden loss of consciousness for less than 30
seconds.

with staring and eyelid flickering but no aura or
postictal state.

produce 3 per second spike and generalized
discharges on the EEG.
Topic 2. Classification of seizures
Atypical absence seizures
 associated with myoclonic movements.
 less than 3 per second discharges on EEG .
 adverse outcome is expected in those with
1) multiple seizures.
2) a positive family history.
3) low IQ(<90).
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Topic 2. Classification of seizures
Tonic-clonic seizures
□the most common type of convulsion.
□commencing with an aura and followed by
1) loss of consciousness.
2) tonic contractures.
3) rhythmic clonic movements.
4) above movements lasting several minutes.
□cyanosis, tongue biting and incontinence of urine during the fit.
□a postictal phase (e.g. headache, a period of sleep).
Topic 2. Classification of seizures
Myoclonic epilepsy
□sudden loss of muscle tone, which may result in injury.
□some forms: a positive family history and a benign course.
□others: mental retardation and are refractory to treatment.
Topic 2. Classification of seizures
Infantile spasms (West syndrome)
□Up to 20% are idiopathic; the remainder are secondary to :
1) hypoxic damage.
2) CNS infection.
3) trauma.
4) neurocutaneous syndromes.
5) storage diseases.
□usually commence around 3-6 months of age.
Topic 2. Classification of seizures
Infantile spasms (West syndrome)
□repetitive, symmetrical contractions of the neck, trunk and
limbs
1) flexor spasms (mostly).
2) extensor contractions (minority).
3) combination.
□The EEG is usually hypsarrhythmia.
□around 40% have evidence of cerebral palsy
have significant cognitive disability.
and 80%
□the treatment difficult, especially in
non-idiopathic types.
□Steroids and vigabatrin have been shown to be effective.
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Topic 2. Classification of seizures
Lennox-Gastaut syndrome (LGS)
□Multiple seizures often include myoclonic, tonic, atypical
absences .
□Most patients with significant motor and cognitive
impairment.
□LGS is often refractory to therapy and only 10% have a
reasonable outcome.
□Occasionally, recourse to surgical resection is necessary.
Topic 3. Management of epilepsy
1.general therapeutics
2.causative therapeutics any underlying cause
and complications must be controlled.
Topic 3. Management of epilepsy
3.therapeutic principle of AED (antiepileptic drug)
1)An expectant approach can be applied for:
□a single, non-febrile seizure.
□no abnormal physical signs.
□a normal EEG.
2)Having established a diagnosis of epilepsy, AED is started
early.
3)The drug of choice is dependent on the seizure type.
Anticonvulsant
Seizure type
VPA
(Sodium valproate)
Tonic-clonic, absences, myoclonic, atonic,
partial, Lennox-Gastaut
(Carbamazepine) CBZ
Tonic-clonic, partial, complex
(Ethosuximide) ESM
Absences
PB (Phenobarbitone)
Tonic-clonic, partial (in neonates)
PHT (Phenytoin)
Tonic-clonic, atonic, partial, status epilepticus
Benzodiazepine
(e.g. NZP,CZP)
Myoclonic, atonic, status epilepticus,
infantile spasms, partial, absences
LTG(Lamotrigine)
Tonic-clonic, atonic, absences,
myoclonic,partial,refractory seizures
VGB (Vigabatrin)
Infantile spasms, tonic-clonic,refractory, partial
Steroids
Infantile spasms, myoclonic, Landau-Kleffner
LEV (Levetiracetam)
Tonic-clonic, myoclonic, complex, refractory
GBP (Gabapentin)
Tonic-clonic, partial, complex
TPM (Topiramate)
Tonic-clonic, partial, Lennox-Gastaut
OXC (Oxcarbazepine)
Partial seizures
Topic 3. Management of epilepsy
4)Monotherapy principle:
□Having established a diagnosis of epilepsy, a single
anticonvulsant is started.
□If the child is still experiencing more than one seizure
per month, a second drug is added in combination.
After a long period of control (a few months), the initial
drug can be discontinued.
a second single drug also belongs to monotherapy.
Topic 3. Management of epilepsy
5)Note side effects:
After AED is started, the dose built up over a
number of weeks until fit control is adequate
without drug-related side effects.
Anticonvulsant
Side effects
VPA (Sodium valproate)
Tremor, liver dysfunction, thrombocytopenia
(Carbamazepine) CBZ
Sedation, ataxia, liver dysfunction, leukopenia
PB (Phenobarbitone)
Rashes, cardiorespiratory depression,
behavioural changes
PHT (Phenytoin)
Rashes, ataxia, hirsutism, gum hypertrophy, Cognitive and
liver dysfunction
Benzodiazepine
(e.g. NZP,CZP)
Sedation, excess salivation, behavioural changes, cognitive
and liver dysfunction
LTG (Lamotrigine)
Rashes, headache, fever, ataxia, liver dysfunction
VGB (Vigabatrin)
Sedation, increased appetite, visual field restriction
LEV (Levetiracetam)
Somnolence, ataxia, tremor, behavioural changes
GBP (Gabapentin)
Somnolence, behavioural changes
TPM (Topiramate)
Somnolence, anorexia, ataxia, behavioural changes
OXC (Oxcarbazepine)
Sedation, ataxia, liver dysfunction, leukopenia
Topic 3. Management of epilepsy
6)Serum anticonvulsant levels:
□It should be used only as a guide to management,
but may be helpful especially with VPA,PHT,PB.
□for most anticonvulsants, there is very little
correlation with serum levels, efficacy and side
effects.
Topic 3. Management of epilepsy
7)Cessation of medication can be considered
after a 1-2-year fit-free period in those patients
without risk factors.
□Weaning should take place over a minimum of
3 months.
□up to 70% of these patients will have no further
fits.
Topic 3. Management of epilepsy
Indicative of a good
outcome:
Indicative of a poor
outcome:
□ a single seizure type.
□ multiple seizure types.
□ short duration.
□ prolonged duration.
□ infrequent occurrence.
□ no neurological
impairment.
□ frequent episodes.
□ additional neurological
impairment.
□ refractoriness to AED.
□ early onset.
Topic 3. Management of epilepsy
4.Surgery
□is developing as a viable treatment option for
intractable epilepsy.
□particularly in those with a demonstrable clinical,
radiological or EEG focus.
Topic 3. Management of epilepsy
5.Vagal nerve stimulation (VNS):
□has been shown to reduce
seizures by up to 50% on
average.
□with intractable seizure
disorders.
□multiple anticonvulsants have
previously failed to achieve
satisfactory control.
Topic 4. Non-epileptic seizures
Non-epileptic seizures include:
□those related to a fever or to a CNS insult
such as trauma, infections
and metabolic
derangement.
□They do not necessarily lead to lifetime
seizure activity.
Topic 4. Non-epileptic seizures
Febrile convulsions (FC) characteristic :
□FC are the most common seizures in childhood, usually affecting
4% of children.
□children aged 6 months to 6 years.
□The convulsion is generally triggered with spikes of fever and
usually responds to cooling and antipyretic measures.
□They comprise symmetrical, generalized tonic-clonic seizures,
associated with loss of consciousness and no focal neurological
signs.
□seizures lasting less than 15 minutes,
□often with a positive family history.
Topic 4. Non-epileptic seizures
Following a febrile convulsion, all children
should be examined to exclude:
 a serious underlying infection. If in doubt, a
lumbar puncture should be performed.
 an underlying metabolic or CNS abnormality.
All those with atypical febrile convulsions
require nvestigation with neuroimaging and an
EEG.
Topic 4. Non-epileptic seizures
Febrile convulsion treatment:
 the administration of rectal diazepam during
subsequent convulsions.
 If indicated, prophylactic VPA or PB.
 There is a slightly increased risk of non-febrile seizure
activity later in life in 10% of children.
 There is no place for routine anticonvulsant therapy in
typical febrile seizures.
Topic 4. Non-epileptic seizures
Febrile convulsion adverse factors :
 continuing seizure activity include complex or
atypical initial seizures.
 a positive family history of epilepsy.
 preceding neurological abnormality.
Topic 5. Status epilepticus
status epilepticus (SE) definition:
 constant seizure activity
persisting beyond 30 minutes.
 recurrent fits
with no resumption of consciousness
persisting beyond 30min.
Topic 5. Status epilepticus
SE causes:
usually after prolonged FS.
patients with preceding neurological
or metabolic disease.
also occurs in those patients without
a CNS insult.
Topic 5. Status epilepticus
SE complication:
□cerebral hypoglycaemia.
□lactic acidosis.
□Hypoxia.
□5% of patients results in death.
Topic 5. Status epilepticus
Management of SE:
□This medical emergency requires active resuscitation.
□protection of the airway.
□correction of the metabolic derangements.
□anticonvulsants:
1)Intravenous diazepam is the first drug of choice.
2)be repeated and followed by PHT,VPA,PB.
3)In refractory cases, general anaesthesia with
thiopentone is required.
Topic 6. Seizure-like disorders
Seizure-like disorders
□Differentiate seizures from the non-convulsive
disorders, detailed history is essential.
□In particularly difficult cases, a period of inhospital observation and repeated EEGs
(preferably, continuous video-EEG) may be
necessary.
Topic 6. Seizure-like disorders
Table Seizure-like disorders
Preschool children
School children
Breath-holding attacks
Night terrors
Paroxysmal vertigo
Benign myoclonus
Syncopal attacks
Rage attacks
Narcolepsy
Munchausen syndrome
by proxy
Question
□ What are clinical features of infantile
spasms?
□ What are characteristics of febrile
convulsions?
□ What therapeutic intervention is
required at status epilepticus?