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A CHILD WITH CONVULSION
NUR FARRA NAJWA
082015100035
• Recent classification, update and terminology of seizures,
epilepsy and status epilepticus according to ILAE
• Approach in child with first seizures
• Approach in child with epilepsy
• Approach in child with status epilepticus
• Febrile seizure in pediatric
• Understand the principle of anti-epileptic drug
• Sudden Unexpected Death of Epilepsy (SUDEP)
• Patients with “intractable epilepsy”
• Management of seizure disorder in general, including first
aid in seizure
• Basic advices to parents (counselling)
LEARNING OBJECTIVES
CLASSIFICATION
Partial Seizures (start in one place)
Simple (no loss of consciousness of memory)
Sensory
Motor
Sensory-Motor
Psychic (abnormal thoughts or perceptions)
Autonomic (heat, nausea, flushing, etc.)
Complex (consciousness or memory impaired)
With or without aura (warning)
With or without automatisms
Secondarily generalized
Generalized Seizures (apparent start over wide areas of brain)
Absence (petit mal)
Tonic-clonic (grand mal)
Atonic (drop seizures)
Myoclonic
Other
Unclassifiable seizures
Dreifuss et al. Proposal for revised clinical and
electroencephalographic classification of epileptic
seizures. From the Commission on Classification
and Terminology of the International League
Against Epilepsy. Epilepsia. 1981;22:489-501.
INTERNATIONAL CLASSIFICATION OF SEIZURES 1981
Motor
Tonic-clonic
Other motor
Non-Motor (Absence)
Unknown Onset
Motor
Non-Motor
focal to bilateral tonic-clonic
Generalized OnsetFocal Onset
Motor
Tonic-clonic
Other motor
Non-Motor
ILAE 2017 Classification of Seizure Types Basic Version 1
Unclassified 2
1 Definitions, other seizure types and descriptors are listed in the accompanying paper & glossary of terms
2 Due to inadequate information or inability to place in other categories
Aware
Impaired
Awareness
From Fisher et al. Instruction manual for the ILAE 2017 operational
classification of seizure types. Epilepsia doi: 10.1111/epi.13671
Motor
tonic-clonic
clonic
tonic
myoclonic
myoclonic-tonic-clonic
myoclonic-atonic
atonic
epileptic spasms2
Non-Motor (absence)
typical
atypical
myoclonic
eyelid myoclonia
Unknown Onset
Motor Onset
automatisms
atonic2
clonic
epileptic spasms2
hyperkinetic
myoclonic
tonic
Non-Motor Onset
autonomic
behavior arrest
cognitive
emotional
sensory
focal to bilateral tonic-clonic
Generalized OnsetFocal Onset
Aware
Impaired
Awareness
Motor
tonic-clonic
epileptic spasms
Non-Motor
behavior arrest
ILAE 2017 Classification of Seizure Types Expanded Version1
Unclassified3
1 Definitions, other seizure types and descriptors are listed in the
accompanying paper and glossary of terms.
2 These could be focal or generalized, with or without alteration of awareness
3 Due to inadequate information or inability to place in other categories
From Fisher et al. Instruction manual for the ILAE 2017 operational
classification of seizure types. Epilepsia doi: 10.1111/epi.13671
Rules for Classifying Seizures
Onset: Decide whether seizure onset is focal or generalized, using an 80% confidence level.
Awareness: For focal seizures, decide whether to classify by degree of awareness or to omit awareness as a classifier.
Impaired awareness at any point: A focal seizure is a focal impaired awareness seizure if awareness is impaired at
any point during the seizure.
Onset predominates: Classify a focal seizure by its first prominent sign or symptom. Do not count transient behavior
arrest.
Behavior arrest: A focal behavior arrest seizure shows arrest of behavior as the prominent feature of the entire
seizure.
Motor/Non-motor: A focal aware or impaired awareness seizure maybe further sub-classified by motor or non-motor
characteristics. Alternatively, a focal seizure can be characterized by motor or non-motor characteristics, without
specifying level of awareness. Example, a focal tonic seizure.
DEFINITION
Epilepsy
• A disorder of the brain characterized by an enduring predisposition to
generate epileptic seizures and by the neurobiologic, cognitive,
psychological, and social consequences of this condition.
An epileptic
seizure
• The transient occurrence of clinical manifestation of abnormal excessive or
synchronous neuronal activity in the brain.
An epileptic
syndrome
• An epileptic disorder characterized by a cluster of signs and symptoms.
• Syndromes are classified on age of onset, seizure type(s), clinical and
developmental features, EEG abnormalities and MRI brain findings.
• It has therapeutic and prognostic implications.
SEIZURES
• A transient occurrence of signs and/or
symptoms due to abnormal excessive or
synchronous neuronal activity in the brain
EPILEPSY
• A disorder of the brain characterized by an
enduring predisposition to generate epileptic
seizures and by the neurobiologic, cognitive,
psychological, and social consequences of this
condition
CONVULSION
• The word “convulsion” is not part of the 2017
seizure classification, but will undoubtedly
persist in popular usage
• Term used to mean substantial motor activity
during a seizure.
• Convulsions and seizures are considered
synonyms and the motor component is not
clear.
APPROACH TO A CHILD WITH A
FIRST SEIZURE
DEFINITION
• One or multiple unprovoked afebrile seizures
within 24 hours with recovery of
consciousness between seizures.
Notes:
• 25-50% of first unprovoked seizures in
children will recur.
• The child who is neurologically normal, with
no history of neurologic illness, and no evident
acute cause for the seizure
– Has an approximately 25% risk of a recurrent
seizure in the next year
– And a nearly 50% risk of seizure over the next 10
to 15 years.
STATISTICS
30% of child with
afebrile seizures later
has epilepsy
Risk is only 20% if EEG
and neurological
examination is n normal
4-10% of child
has seizures
during first 16
years of life
3% has episode
of epilepsy
50% epilepsy
cases start during
childhood
Clinical factors associated with an
increased risk of recurrent seizures are:
1. Prior neurologic insult
2. Significant MRI findings
3. Abnormal EEG
APPROACH
Detailed history to determine if event is a seizure
or a paroxysmal non- epileptic event as 30% of
patients referred as epilepsy do not have seizures.
A thorough clinical examination is important to
look for any possible underlying aetiology.
There is a need to exclude acute provoking factors.
Distinguish between provoked seizures secondary
to acute systemic, metabolic or toxic cerebral insult
and epilepsy.
Treating underlying cause of provoked seizures will
usually resolve the seizures and long term anti-
epileptic therapy is not required.
Determine
d seizure
focal or
generalize
d onset
Duration of
seizures and
state of
consciousness
History of aura
and child
behavior
before seizures
Asses the
cardiorespirator
y and metabolic
disturbance
Perform
general and
careful
neurological
examination
Examined
eye ground
and facial
feature.
Localizing
neurological
sign
EVALUATION OF FIRST SEIZURES
INITIAL EVALUATION
Physician
(evaluation of 1st
seizures)
Search for
potentially life
threatening
cause
Attempt history to
define factor
promoting
convulsion, details
of seizures, and
child postictal
state.
During of after
child/neonate
having suspected
seizures
Asses the
adequacy of A,B,C
Measure also the
temperature,
blood pressure
and glucose conc.
INVESTIGATIONS
Routine investigations such as FBC, BUSE, Ca, Mg, RBS if
• Child unwell (vomiting, diarrhoea etc).
• Child not ‘alert’, lethargic or failure to return to baseline alertness.
Lumbar puncture indicated if there is suspicion of brain
infection.
Toxicology screening considered if there is suspicion of
drug exposure.
EEG is recommended after all first afebrile unprovoked
seizures.
• EEG helps classify seizure type, epilepsy syndrome and predict
recurrence.
Neuroimaging (MRI preferred) indicated for:
• Persisting postictal focal deficit (Todd’s paresis).
• Condition of child not returned to baseline within several hours after
the seizure.
TREATMENT
• Treatment with antiepileptic drug is NOT
indicated in all patients with a first afebrile
seizure as it does not prevent development of
epilepsy or influence long term remission.
EPILEPSY
Operational (Practical) Clinical
Definition of Epilepsy
Any of the following conditions:
1. At least two unprovoked (or reflex) seizures
occurring >24 h apart.
2. One unprovoked (or reflex) seizure and a
probability of further seizures similar to the
general recurrence risk (at least 60%) after
two unprovoked seizures, occurring over the
next 10 years.
3. Diagnosis of an epilepsy syndrome.
Imitators of Epilepsy:
Paroxysmal Non-epileptic Events
• The first important step in the management of
childhood epilepsy is
– To differentiate epileptic seizures from paroxysmal
non-epileptic events.
A child with convulsion paeds present
CLASSIFICATION OF THE
EPILEPSIES
Purpose: for clinical diagnosis
Transparent language: use words that mean what they say
ILAE Classification of the Epilepsies
• Simplified the framework
• Levels of diagnosis
– seizure type, epilepsy type (focal, generalized, combined
generalized and focal, unknown) and epilepsy syndrome
• An etiologic diagnosis
– should be considered from when the patient first presents, and
at each step along the diagnostic pathway; a patient’s epilepsy
may be classified into more than one etiological category
Cont.
• The term “benign” is replaced by the terms self-limited
and pharmacoresponsive to be used where appropriate
• The term “developmental and epileptic encephalopathy”
can be applied in whole or in part where appropriate
• Genetic Generalized Epilepsies
– Idiopathic Generalized Epilepsies = CAE, JAE, JME,
GTCA
• Symptomatic Generalized Epiliepsies used for both
 Developmental and Epileptic Encephalopathies
 (static) Encephalopathy with Epilepsy
Unknown
Immune
Infectious
Structural
Etiology
Metabolic
Genetic
Co-morbidities
Epilepsy types
Focal Generalized
Combined
Generalized
& Focal
UnknownFocal
Epilepsy Syndromes
Seizure types
Generalized
onset
Unknown
onset
Focal
onset
Generalized seizures
• Originate at some point
within and rapidly engage
bilaterally distributed
networks
• Can include cortical and
subcortical structures
but not necessarily the
entire cortex
Generalized from
onset seizures are
defined as
“originating at
some point within,
and rapidly
engaging, bilaterally
distributed
networks.” 5
A child with convulsion paeds present
• Originate within
networks limited
to one hemisphere
• May be discretely
localized
or more widely
distributed.…
Focal seizures
Focal-onset seizures
are defined as
“originating within
networks limited to
one hemisphere. They
may be discretely
localized or more
widely distributed.
Focal seizures may
originate in subcortical
structures.”
Epilepsy types
Focal Generalized
Combined
Generalized
& Focal
UnknownFocal
• Where unable to make an Epilepsy Syndrome diagnosis
or a diagnosis of Etiology
• Many examples
– Temporal lobe epilepsy
– Generalized tonic-clonic seizures in a 5 year old with
generalized spike-wave
– Both focal impaired awareness seizures and absence
seizures in a patient
– Cannot tell if tonic-clonic seizure is focal or generalized
Generalized and Focal Epilepsies
• Combined focal and generalized epilepsies
Examples
–Dravet syndrome
Increasingly observed in clinical practice
Previously incorrectly allocated to unknown
• What do with
– Multifocal epilepsies?
– Hemispheric epilepsies?
 focal
 focal
Old term
‘Idiopathic Generalized Epilepsies’
Genetic Generalized Epilepsies
Childhood
Absence
Epilepsy
Generalized
Tonic-Clonic
Seizures Alone
Juvenile
Absence
Epilepsy
Juvenile
Myoclonic
Epilepsy
Epilepsy syndromes
• There are no approved ILAE
epilepsy syndromes
APPROACH TO A CHILD WITH
EPILEPSY
The diagnosis of epilepsy is mainly clinical.
Detailed history of the seizures;
•The setting in which the seizure occurs
•Child’s behaviour preceding, during and after the event is
critical
Video (via mobile phone camera) of the actual
event is very helpful.
The antenatal, birth, past medical history,
developmental milestones and family history
should be recorded meticulously.
Look for
• Dysmorphism
• Neurocutaneous signs
Do thorough CNS and
developmental examination.
Perform general and systemic
examinations to look for clues
of underlying aetiology
INVESTIGATIONS
Are recommended when a second
afebrile seizure occurs :
• To exclude metabolic cause and before
starting anti-epileptic drug therapy.
– Full blood count, biochemical investigations such
as electrolytes, calcium, magnesium, glucose, liver
and renal function tests
• Metabolic and genetic studies in
– Clinically indicated cases with epilepsy,
developmental delay where aetiology is not found
from history and physical examination.
EEG
1. To support the clinical diagnosis of epileptic seizures
2. To classify the seizure type and epileptic syndrome
3. Helps in selection of anti-epileptic drug and
prognosis.
• EEG during sleep
– Increases yield of abnormalities and
– Is important for patients with seizures predominantly
during sleep.
• A ‘normal’ EEG
– Does not exclude epilepsy as it is a clinical diagnosis and
– The yield of abnormalities from a single EEG recording is
low.
Neuroimaging
• CT scan is indicated
– in emergency setting during acute illness.
• However, MRI should be considered in
– if there are any atypical features or
– if the seizures are difficult to be controlled.
MRI IS INDICATED IN: MRI IS NOT INDICATED IN:
1. Epilepsy occurring in the first
year of life, except febrile
seizures.
2. Focal epilepsy except childhood
epilepsy with centrotemporal
spikes.
3. Developmental delay or
regression.
4. Difficult to control / refractory
epilepsy.
1. Childhood epilepsy with
centrotemporal spikes
(previously called Benign Rolandic
epilepsy).
2. Genetic generalized epilepsies
(e.g. Childhood absence epilepsy,
Juvenile absence epilepsy, Juvenile
myoclonic epilepsy)
A child with convulsion paeds present
A child with convulsion paeds present
STATUS EPILEPTICUS (SE)
DEFINITION (ILAE 2015)
 When an adult has a seizure or seizures
lasting more than a defined time period, they
are said to be in status epilepticus, or SE.
SE is a condition resulting either from the failure of the
mechanisms responsible for seizure termination, or from
the initiation of mechanisms which lead to abnormally
prolonged seizures (after time point t1) it is a condition
that can have long term consequences
(after time point t2) including
– Neuronal death
– Neuronal injury
– Alteration of neuronal network
– Depending of type and duration of seizures
This definition is conceptual, with two
operational dimensions
• The first is the length of the seizure and the
time point (t1) beyond which the seizure
should be regarded as “continuous seizure
activity.”
• The second time point (t2) is the time of
ongoing seizure activity after which there is a
risk of long-term consequences.
t1 t2
Continuous seizures activity
Risk of long term consequences
A child with convulsion paeds present
A new diagnostic classification system of SE
is proposed, which will provide a framework for
clinical diagnosis, investigation, and therapeutic
approaches for each patient.
There Are Four Axes
1. Semiology
– Lists different forms of SE divided into those with prominent motor systems,
those without prominent motor systems, and currently indeterminate
conditions (such as acute confusional states with epileptiform EEG patterns)
2. Etiology
– Divided into subcategories of known and unknown causes
3. Elecroencephalography (EEG)
– Adopts the latest recommendations by consensus panels to use the following
descriptors for the EEG:
– Name of pattern, morphology, location, time-related features, modulation,
and effect of intervention.
4. Age.
– Divides age groups into neonatal, infancy, childhood, adolescent and
adulthood, and elderly.
TYPES OF SE
(Simplified from ILAE 2015)
1. Convulsive SE (seizures with prominent
motor symptoms)
2. Non-convulsive SE (seizures on EEG only)
3. Focal motor SE
Treatment for convulsive SE should be
initiated when there is..
1. Continuous seizure or
2. Two or more discrete seizures lasting >5 min,
between which there is incomplete
recovery (see Algorithm).
3. Timing and treatment of NCSE and focal
motor SE may be more variable (consult
neurologist).
A child with convulsion paeds present
DOs
Optimize vital functions throughout control of
status epilepticus.
Consider intubation early if
• Airway/gas exchange compromised
• Elevated ICP suspected or
• If seizures persist >30 minutes.
Identify and treat underlying cause
(commonly: infectious and autoimmune encephalitides,
traumatic/ hypoxic injuries, metabolic strokes, specific
epilepsy syndromes and AED withdrawal in patients with
epilepsy).
Give adequate loading doses followed by
maintenance, drug levels for phenobarbitone
and phenytoin are useful to monitor and guide
treatment.
If using multiple drugs,
use those with different
mechanisms of action
and avoid phenytoin and
Phenobarbitone
combination if possible.
Consider therapeutic
hypothermia early in
cases of refractory SE.
DON’Ts
Avoid excessive time lag between
doses/steps of treatment.
Be careful with drugs that may exacerbate certain
forms of seizures
•Benzodiazepines in tonic SE,
•Carbamazepine in NCSE,
•Valproate/ phenobarbitone in mitochondrial disease
Avoid propofol in
•Patients on ketogenic diet and
•Those needing steroids/ catecholamines (Risk of propofol infusion
syndrome).
Do not treat ALL abnormal movements and episodes
of stiffening as seizures.
•Movement disorder and dystonia from paroxysmal autonomic
instability are common comorbid conditions.
•Hence, video EEG monitoring +/- neurological consult may be
required.
FEBRILE SEIZURES
DEFINITION
Seizures occurring in association with fever in
children between 3 months and 6 years of age,
in whom there is no evidence of intracranial
pathology or metabolic derangement.
NELSON
Febrile seizures are seizures that occur between
the age of 6 and 60 month with a temperature
of 38°C (100.4°F) or higher,
that are not the result of central nervous system
infection or any metabolic imbalance,
and that occur in the absence of a history of
prior afebrile seizures
ILAE 2009
Simple febrile seizure is defined as a short
(<15 min) generalized seizure, not recurring
within 24h,
that occurs during a febrile illness not resulting
from an acute disease of the nervous system in a
child aged between 6 months and 5 years,
with no neurologic deficits and no previous
afebrile seizures
A child with convulsion paeds present
MANAGEMENT
A child with convulsion paeds present
Not all children need
hospital admission.
The main reasons are:
• To exclude intracranial pathology
Especially infection.
• Fear of recurrent seizures.
• To investigate and treat the cause
of fever besides
meningitis/encephalitis.
• To allay parental anxiety,
especially if they are staying far
from hospital.
INVESTIGATIONS
Will depend on clinical assessment of
the individual case :
• Blood counts,
• Blood sugar,
• Lumbar puncture,
• Urinalysis,
• Chest x-ray,
• Blood culture etc,
Lumbar Puncture
Must be done if (unless
contraindicated)
1. Any symptoms or signs
suggestive of intracranial
infection
2. Persistent lethargy and not
fully interactive
Should be considered if
1. Age < 12 months old
especially if child has not
received Hib and
pneumococcal
immunization
2. Prior antibiotic therapy
Cont.
• Serum calcium and electrolytes
– Rarely necessary.
• EEG
– Is not indicated even if multiple recurrences or
complex febrile seizures.
• Parents should be counselled on the benign
nature of the condition.
CONTROL FEVER
• Avoid excessive clothing
• Use antipyretic
– Syrup or rectal paracetamol 15 mg/kg 6 hourly
Parents should also be advised on
first aid measures during a seizure
• Parents of children with high risk of recurrent
febrile seizures including those with febrile
status epilepticus should be supplied with
– Rectal Diazepam (dose : 0.5 mg/kg).
• They should be advised on how to administer
it if the seizures last more than 5 minutes.
 Prevention of recurrent febrile
seizures.
• Antiepileptic drugs are not recommended for
prevention of recurrent febrile seizures
because:
1. The risks and potential side effects of
medications outweigh the benefits
2. No medication has been shown to prevent the
future onset of epilepsy.
3. Febrile seizures have an excellent outcome with
no neurological deficit nor any effect on
intelligence.
A child with convulsion paeds present
A child with convulsion paeds present
PRINCIPLES OF ANTIEPILEPTIC
DRUG (AED) THERAPY FOR
SEIZURES/EPILEPSY
A child with convulsion paeds present
A child with convulsion paeds present
1. Attempt to classify the seizure type(s) and epilepsy
syndrome.
2. Treatment recommended if ≥ 2 episodes
(recurrence risk up to 80%).
3. Monotherapy as far as possible.
4. Increase dose gradually until seizures controlled or
maximum dose reached or side effects occur.
5. Add on the second drug if first drug failed.
6. Rational combination therapy (usually 2 or
maximum 3 drugs)
1. Beware of AED-induced seizure
2. Trial of vitamins and co-factors considered in
infantile epilepsies not responding to AED.
3. In children not responding to treatment with 2
AEDs, complete re-evaluation required for epilepsy
surgery / trial of ketogenic diet.
4. Risk of carbamazepine-induced hypersensitivity
reactions, is increased in patients with the HLA-
B*1502 allele – consider testing if test easily
available.
5. Avoid starting female of childbearing potential with
sodium valproate
1. Drug level monitoring is not routinely done
(except phenytoin)
2. Vitamin D supplementation should be
considered for children with risk factors for
Vitamin D deficiency
3. When withdrawal of medication is planned
(generally after being seizure free for 2 years)
4. If seizures recur, the last dose reduction is
reversed and medical advice sought.
A child with convulsion paeds present
A child with convulsion paeds present
A child with convulsion paeds present
A child with convulsion paeds present
Sudden Unexpected Death in
Epilepsy
(SUDEP)
A child with convulsion paeds present
SUDEP
• The most common epilepsy related mortality in
patients with chronic epilepsy
• Incidence is unknown
• Ranges from 1-5 per 1,000 people with epilepsy.
• Precise etiology is unknown
• Risk factors include
– Polypharmacology,
– Poorly controlled generalized tonic–clonic seizures,
– Male gender,
– Age younger than 16 yr,
– Long duration of epilepsy, and frequent seizures.
Cont.
• Patients are usually found dead in their bed in a
prone position with evidence suggesting a recent
seizure.
• Potential mechanisms of SUDEP include
• Respiratory arrest or dysfunction,
• Drug-induced cardiac toxicity,
• CNS dysfunction (hypoventilation, arrhythmia, suppression
of brain electrical activity), or
• Pulmonary edema.
A child with convulsion paeds present
THE PATIENTS WITH
“INTRACTABLE EPILEPSY”
Please re-evaluate for the following
possibilities :
• Is it a seizure or a non-epileptic event?
• Wrong classification of epilepsy syndrome, thus
wrong choice of antiepileptic drug.
• Antiepileptic drug dose not optimised.
• Poor compliance to antiepileptic drug.
• Antiepileptic drug aggravating seizures.
• Lesional epilepsy, hence a potential epilepsy
surgery candidate.
• Progressive epilepsy or neurodegenerative
disorder.
WHEN TO REFER TO PAEDIATRIC
NEUROLOGIST?
Refer immediately
(to contact paediatric neurologist) :
1. Behavioural or developmental regression.
2. Infantile spasms..
Refer:
1. Poor seizure control despite monotherapy
with 2 different antiepileptic medications.
2. Difficult to control epilepsies beginning in the
first two years of life.
3. Structural lesion on neuroimaging
ADVICE FOR PARENTS/
COUNSELLING
• Educate the child and family about epilepsy
– Disease
– Management
– Limitation
• Establish therapeutic alliances
• Evaluate risk of involvement in athletic activities
• Evaluate for possible learning disabilities
• Resources in community
• Informed family about increase risk of mortality
• Educate what to do in epilepsy, medicine choices, side
effect of drugs
• Discuss about the potential complication of epilepsy
(Discourage the continuous observation of child in
wakeful and sleep state)
A child with convulsion paeds present
A child with convulsion paeds present
• Recent classification, update and terminology of seizures,
epilepsy and status epilepticus according to ILAE
• Approach in child with first seizures
• Approach in child with epilepsy
• Approach in child with status epilepticus
• Febrile seizure in pediatric
• Understand the principle of anti-epileptic drug
• Sudden Unexpected Death of Epilepsy (SUDEP)
• Patients with “intractable epilepsy”
• Management of seizure disorder in general, including first
aid in seizure
• Basic advices to parents (counselling)
SUMMARY
REFERENCES
• Nelson Textbook Of Pediatrics, 20th Edition,
Chapter:593, Seizures in Childhood, Mohamad
A. Mikati and Abeer J. Hani, Page:2823
• Paediatrics Protocol For Malaysian Hospital,
4th Edition, Kementerian Kesihatan Malaysia,
Chapter:46-48, Status Epilepticus, Epilepsy,
Febrile Seizure , Page: 243
• ILAE
THANK YOU

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A child with convulsion paeds present

  • 1. A CHILD WITH CONVULSION NUR FARRA NAJWA 082015100035
  • 2. • Recent classification, update and terminology of seizures, epilepsy and status epilepticus according to ILAE • Approach in child with first seizures • Approach in child with epilepsy • Approach in child with status epilepticus • Febrile seizure in pediatric • Understand the principle of anti-epileptic drug • Sudden Unexpected Death of Epilepsy (SUDEP) • Patients with “intractable epilepsy” • Management of seizure disorder in general, including first aid in seizure • Basic advices to parents (counselling) LEARNING OBJECTIVES
  • 4. Partial Seizures (start in one place) Simple (no loss of consciousness of memory) Sensory Motor Sensory-Motor Psychic (abnormal thoughts or perceptions) Autonomic (heat, nausea, flushing, etc.) Complex (consciousness or memory impaired) With or without aura (warning) With or without automatisms Secondarily generalized Generalized Seizures (apparent start over wide areas of brain) Absence (petit mal) Tonic-clonic (grand mal) Atonic (drop seizures) Myoclonic Other Unclassifiable seizures Dreifuss et al. Proposal for revised clinical and electroencephalographic classification of epileptic seizures. From the Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1981;22:489-501. INTERNATIONAL CLASSIFICATION OF SEIZURES 1981
  • 5. Motor Tonic-clonic Other motor Non-Motor (Absence) Unknown Onset Motor Non-Motor focal to bilateral tonic-clonic Generalized OnsetFocal Onset Motor Tonic-clonic Other motor Non-Motor ILAE 2017 Classification of Seizure Types Basic Version 1 Unclassified 2 1 Definitions, other seizure types and descriptors are listed in the accompanying paper & glossary of terms 2 Due to inadequate information or inability to place in other categories Aware Impaired Awareness From Fisher et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia doi: 10.1111/epi.13671
  • 6. Motor tonic-clonic clonic tonic myoclonic myoclonic-tonic-clonic myoclonic-atonic atonic epileptic spasms2 Non-Motor (absence) typical atypical myoclonic eyelid myoclonia Unknown Onset Motor Onset automatisms atonic2 clonic epileptic spasms2 hyperkinetic myoclonic tonic Non-Motor Onset autonomic behavior arrest cognitive emotional sensory focal to bilateral tonic-clonic Generalized OnsetFocal Onset Aware Impaired Awareness Motor tonic-clonic epileptic spasms Non-Motor behavior arrest ILAE 2017 Classification of Seizure Types Expanded Version1 Unclassified3 1 Definitions, other seizure types and descriptors are listed in the accompanying paper and glossary of terms. 2 These could be focal or generalized, with or without alteration of awareness 3 Due to inadequate information or inability to place in other categories From Fisher et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia doi: 10.1111/epi.13671
  • 7. Rules for Classifying Seizures Onset: Decide whether seizure onset is focal or generalized, using an 80% confidence level. Awareness: For focal seizures, decide whether to classify by degree of awareness or to omit awareness as a classifier. Impaired awareness at any point: A focal seizure is a focal impaired awareness seizure if awareness is impaired at any point during the seizure. Onset predominates: Classify a focal seizure by its first prominent sign or symptom. Do not count transient behavior arrest. Behavior arrest: A focal behavior arrest seizure shows arrest of behavior as the prominent feature of the entire seizure. Motor/Non-motor: A focal aware or impaired awareness seizure maybe further sub-classified by motor or non-motor characteristics. Alternatively, a focal seizure can be characterized by motor or non-motor characteristics, without specifying level of awareness. Example, a focal tonic seizure.
  • 8. DEFINITION Epilepsy • A disorder of the brain characterized by an enduring predisposition to generate epileptic seizures and by the neurobiologic, cognitive, psychological, and social consequences of this condition. An epileptic seizure • The transient occurrence of clinical manifestation of abnormal excessive or synchronous neuronal activity in the brain. An epileptic syndrome • An epileptic disorder characterized by a cluster of signs and symptoms. • Syndromes are classified on age of onset, seizure type(s), clinical and developmental features, EEG abnormalities and MRI brain findings. • It has therapeutic and prognostic implications.
  • 9. SEIZURES • A transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain EPILEPSY • A disorder of the brain characterized by an enduring predisposition to generate epileptic seizures and by the neurobiologic, cognitive, psychological, and social consequences of this condition
  • 10. CONVULSION • The word “convulsion” is not part of the 2017 seizure classification, but will undoubtedly persist in popular usage • Term used to mean substantial motor activity during a seizure. • Convulsions and seizures are considered synonyms and the motor component is not clear.
  • 11. APPROACH TO A CHILD WITH A FIRST SEIZURE
  • 12. DEFINITION • One or multiple unprovoked afebrile seizures within 24 hours with recovery of consciousness between seizures.
  • 13. Notes: • 25-50% of first unprovoked seizures in children will recur. • The child who is neurologically normal, with no history of neurologic illness, and no evident acute cause for the seizure – Has an approximately 25% risk of a recurrent seizure in the next year – And a nearly 50% risk of seizure over the next 10 to 15 years.
  • 14. STATISTICS 30% of child with afebrile seizures later has epilepsy Risk is only 20% if EEG and neurological examination is n normal 4-10% of child has seizures during first 16 years of life 3% has episode of epilepsy 50% epilepsy cases start during childhood
  • 15. Clinical factors associated with an increased risk of recurrent seizures are: 1. Prior neurologic insult 2. Significant MRI findings 3. Abnormal EEG
  • 17. Detailed history to determine if event is a seizure or a paroxysmal non- epileptic event as 30% of patients referred as epilepsy do not have seizures. A thorough clinical examination is important to look for any possible underlying aetiology. There is a need to exclude acute provoking factors. Distinguish between provoked seizures secondary to acute systemic, metabolic or toxic cerebral insult and epilepsy. Treating underlying cause of provoked seizures will usually resolve the seizures and long term anti- epileptic therapy is not required.
  • 18. Determine d seizure focal or generalize d onset Duration of seizures and state of consciousness History of aura and child behavior before seizures Asses the cardiorespirator y and metabolic disturbance Perform general and careful neurological examination Examined eye ground and facial feature. Localizing neurological sign
  • 20. INITIAL EVALUATION Physician (evaluation of 1st seizures) Search for potentially life threatening cause Attempt history to define factor promoting convulsion, details of seizures, and child postictal state. During of after child/neonate having suspected seizures Asses the adequacy of A,B,C Measure also the temperature, blood pressure and glucose conc.
  • 22. Routine investigations such as FBC, BUSE, Ca, Mg, RBS if • Child unwell (vomiting, diarrhoea etc). • Child not ‘alert’, lethargic or failure to return to baseline alertness. Lumbar puncture indicated if there is suspicion of brain infection. Toxicology screening considered if there is suspicion of drug exposure. EEG is recommended after all first afebrile unprovoked seizures. • EEG helps classify seizure type, epilepsy syndrome and predict recurrence. Neuroimaging (MRI preferred) indicated for: • Persisting postictal focal deficit (Todd’s paresis). • Condition of child not returned to baseline within several hours after the seizure.
  • 24. • Treatment with antiepileptic drug is NOT indicated in all patients with a first afebrile seizure as it does not prevent development of epilepsy or influence long term remission.
  • 27. Any of the following conditions: 1. At least two unprovoked (or reflex) seizures occurring >24 h apart. 2. One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years. 3. Diagnosis of an epilepsy syndrome.
  • 28. Imitators of Epilepsy: Paroxysmal Non-epileptic Events • The first important step in the management of childhood epilepsy is – To differentiate epileptic seizures from paroxysmal non-epileptic events.
  • 30. CLASSIFICATION OF THE EPILEPSIES Purpose: for clinical diagnosis Transparent language: use words that mean what they say
  • 31. ILAE Classification of the Epilepsies • Simplified the framework • Levels of diagnosis – seizure type, epilepsy type (focal, generalized, combined generalized and focal, unknown) and epilepsy syndrome • An etiologic diagnosis – should be considered from when the patient first presents, and at each step along the diagnostic pathway; a patient’s epilepsy may be classified into more than one etiological category
  • 32. Cont. • The term “benign” is replaced by the terms self-limited and pharmacoresponsive to be used where appropriate • The term “developmental and epileptic encephalopathy” can be applied in whole or in part where appropriate • Genetic Generalized Epilepsies – Idiopathic Generalized Epilepsies = CAE, JAE, JME, GTCA • Symptomatic Generalized Epiliepsies used for both  Developmental and Epileptic Encephalopathies  (static) Encephalopathy with Epilepsy
  • 33. Unknown Immune Infectious Structural Etiology Metabolic Genetic Co-morbidities Epilepsy types Focal Generalized Combined Generalized & Focal UnknownFocal Epilepsy Syndromes Seizure types Generalized onset Unknown onset Focal onset
  • 34. Generalized seizures • Originate at some point within and rapidly engage bilaterally distributed networks • Can include cortical and subcortical structures but not necessarily the entire cortex Generalized from onset seizures are defined as “originating at some point within, and rapidly engaging, bilaterally distributed networks.” 5
  • 36. • Originate within networks limited to one hemisphere • May be discretely localized or more widely distributed.… Focal seizures Focal-onset seizures are defined as “originating within networks limited to one hemisphere. They may be discretely localized or more widely distributed. Focal seizures may originate in subcortical structures.”
  • 37. Epilepsy types Focal Generalized Combined Generalized & Focal UnknownFocal • Where unable to make an Epilepsy Syndrome diagnosis or a diagnosis of Etiology • Many examples – Temporal lobe epilepsy – Generalized tonic-clonic seizures in a 5 year old with generalized spike-wave – Both focal impaired awareness seizures and absence seizures in a patient – Cannot tell if tonic-clonic seizure is focal or generalized
  • 38. Generalized and Focal Epilepsies • Combined focal and generalized epilepsies Examples –Dravet syndrome Increasingly observed in clinical practice Previously incorrectly allocated to unknown • What do with – Multifocal epilepsies? – Hemispheric epilepsies?  focal  focal
  • 39. Old term ‘Idiopathic Generalized Epilepsies’ Genetic Generalized Epilepsies Childhood Absence Epilepsy Generalized Tonic-Clonic Seizures Alone Juvenile Absence Epilepsy Juvenile Myoclonic Epilepsy
  • 40. Epilepsy syndromes • There are no approved ILAE epilepsy syndromes
  • 41. APPROACH TO A CHILD WITH EPILEPSY
  • 42. The diagnosis of epilepsy is mainly clinical. Detailed history of the seizures; •The setting in which the seizure occurs •Child’s behaviour preceding, during and after the event is critical Video (via mobile phone camera) of the actual event is very helpful. The antenatal, birth, past medical history, developmental milestones and family history should be recorded meticulously.
  • 43. Look for • Dysmorphism • Neurocutaneous signs Do thorough CNS and developmental examination. Perform general and systemic examinations to look for clues of underlying aetiology
  • 45. Are recommended when a second afebrile seizure occurs : • To exclude metabolic cause and before starting anti-epileptic drug therapy. – Full blood count, biochemical investigations such as electrolytes, calcium, magnesium, glucose, liver and renal function tests • Metabolic and genetic studies in – Clinically indicated cases with epilepsy, developmental delay where aetiology is not found from history and physical examination.
  • 46. EEG 1. To support the clinical diagnosis of epileptic seizures 2. To classify the seizure type and epileptic syndrome 3. Helps in selection of anti-epileptic drug and prognosis. • EEG during sleep – Increases yield of abnormalities and – Is important for patients with seizures predominantly during sleep. • A ‘normal’ EEG – Does not exclude epilepsy as it is a clinical diagnosis and – The yield of abnormalities from a single EEG recording is low.
  • 47. Neuroimaging • CT scan is indicated – in emergency setting during acute illness. • However, MRI should be considered in – if there are any atypical features or – if the seizures are difficult to be controlled.
  • 48. MRI IS INDICATED IN: MRI IS NOT INDICATED IN: 1. Epilepsy occurring in the first year of life, except febrile seizures. 2. Focal epilepsy except childhood epilepsy with centrotemporal spikes. 3. Developmental delay or regression. 4. Difficult to control / refractory epilepsy. 1. Childhood epilepsy with centrotemporal spikes (previously called Benign Rolandic epilepsy). 2. Genetic generalized epilepsies (e.g. Childhood absence epilepsy, Juvenile absence epilepsy, Juvenile myoclonic epilepsy)
  • 51. STATUS EPILEPTICUS (SE) DEFINITION (ILAE 2015)  When an adult has a seizure or seizures lasting more than a defined time period, they are said to be in status epilepticus, or SE.
  • 52. SE is a condition resulting either from the failure of the mechanisms responsible for seizure termination, or from the initiation of mechanisms which lead to abnormally prolonged seizures (after time point t1) it is a condition that can have long term consequences (after time point t2) including – Neuronal death – Neuronal injury – Alteration of neuronal network – Depending of type and duration of seizures
  • 53. This definition is conceptual, with two operational dimensions • The first is the length of the seizure and the time point (t1) beyond which the seizure should be regarded as “continuous seizure activity.” • The second time point (t2) is the time of ongoing seizure activity after which there is a risk of long-term consequences.
  • 54. t1 t2 Continuous seizures activity Risk of long term consequences
  • 56. A new diagnostic classification system of SE is proposed, which will provide a framework for clinical diagnosis, investigation, and therapeutic approaches for each patient.
  • 57. There Are Four Axes 1. Semiology – Lists different forms of SE divided into those with prominent motor systems, those without prominent motor systems, and currently indeterminate conditions (such as acute confusional states with epileptiform EEG patterns) 2. Etiology – Divided into subcategories of known and unknown causes 3. Elecroencephalography (EEG) – Adopts the latest recommendations by consensus panels to use the following descriptors for the EEG: – Name of pattern, morphology, location, time-related features, modulation, and effect of intervention. 4. Age. – Divides age groups into neonatal, infancy, childhood, adolescent and adulthood, and elderly.
  • 58. TYPES OF SE (Simplified from ILAE 2015) 1. Convulsive SE (seizures with prominent motor symptoms) 2. Non-convulsive SE (seizures on EEG only) 3. Focal motor SE
  • 59. Treatment for convulsive SE should be initiated when there is.. 1. Continuous seizure or 2. Two or more discrete seizures lasting >5 min, between which there is incomplete recovery (see Algorithm). 3. Timing and treatment of NCSE and focal motor SE may be more variable (consult neurologist).
  • 61. DOs
  • 62. Optimize vital functions throughout control of status epilepticus. Consider intubation early if • Airway/gas exchange compromised • Elevated ICP suspected or • If seizures persist >30 minutes. Identify and treat underlying cause (commonly: infectious and autoimmune encephalitides, traumatic/ hypoxic injuries, metabolic strokes, specific epilepsy syndromes and AED withdrawal in patients with epilepsy). Give adequate loading doses followed by maintenance, drug levels for phenobarbitone and phenytoin are useful to monitor and guide treatment.
  • 63. If using multiple drugs, use those with different mechanisms of action and avoid phenytoin and Phenobarbitone combination if possible. Consider therapeutic hypothermia early in cases of refractory SE.
  • 65. Avoid excessive time lag between doses/steps of treatment. Be careful with drugs that may exacerbate certain forms of seizures •Benzodiazepines in tonic SE, •Carbamazepine in NCSE, •Valproate/ phenobarbitone in mitochondrial disease Avoid propofol in •Patients on ketogenic diet and •Those needing steroids/ catecholamines (Risk of propofol infusion syndrome). Do not treat ALL abnormal movements and episodes of stiffening as seizures. •Movement disorder and dystonia from paroxysmal autonomic instability are common comorbid conditions. •Hence, video EEG monitoring +/- neurological consult may be required.
  • 67. DEFINITION Seizures occurring in association with fever in children between 3 months and 6 years of age, in whom there is no evidence of intracranial pathology or metabolic derangement.
  • 68. NELSON Febrile seizures are seizures that occur between the age of 6 and 60 month with a temperature of 38°C (100.4°F) or higher, that are not the result of central nervous system infection or any metabolic imbalance, and that occur in the absence of a history of prior afebrile seizures
  • 69. ILAE 2009 Simple febrile seizure is defined as a short (<15 min) generalized seizure, not recurring within 24h, that occurs during a febrile illness not resulting from an acute disease of the nervous system in a child aged between 6 months and 5 years, with no neurologic deficits and no previous afebrile seizures
  • 73. Not all children need hospital admission. The main reasons are: • To exclude intracranial pathology Especially infection. • Fear of recurrent seizures. • To investigate and treat the cause of fever besides meningitis/encephalitis. • To allay parental anxiety, especially if they are staying far from hospital.
  • 75. Will depend on clinical assessment of the individual case : • Blood counts, • Blood sugar, • Lumbar puncture, • Urinalysis, • Chest x-ray, • Blood culture etc,
  • 76. Lumbar Puncture Must be done if (unless contraindicated) 1. Any symptoms or signs suggestive of intracranial infection 2. Persistent lethargy and not fully interactive Should be considered if 1. Age < 12 months old especially if child has not received Hib and pneumococcal immunization 2. Prior antibiotic therapy
  • 77. Cont. • Serum calcium and electrolytes – Rarely necessary. • EEG – Is not indicated even if multiple recurrences or complex febrile seizures. • Parents should be counselled on the benign nature of the condition.
  • 78. CONTROL FEVER • Avoid excessive clothing • Use antipyretic – Syrup or rectal paracetamol 15 mg/kg 6 hourly
  • 79. Parents should also be advised on first aid measures during a seizure • Parents of children with high risk of recurrent febrile seizures including those with febrile status epilepticus should be supplied with – Rectal Diazepam (dose : 0.5 mg/kg). • They should be advised on how to administer it if the seizures last more than 5 minutes.
  • 80.  Prevention of recurrent febrile seizures. • Antiepileptic drugs are not recommended for prevention of recurrent febrile seizures because: 1. The risks and potential side effects of medications outweigh the benefits 2. No medication has been shown to prevent the future onset of epilepsy. 3. Febrile seizures have an excellent outcome with no neurological deficit nor any effect on intelligence.
  • 83. PRINCIPLES OF ANTIEPILEPTIC DRUG (AED) THERAPY FOR SEIZURES/EPILEPSY
  • 86. 1. Attempt to classify the seizure type(s) and epilepsy syndrome. 2. Treatment recommended if ≥ 2 episodes (recurrence risk up to 80%). 3. Monotherapy as far as possible. 4. Increase dose gradually until seizures controlled or maximum dose reached or side effects occur. 5. Add on the second drug if first drug failed. 6. Rational combination therapy (usually 2 or maximum 3 drugs)
  • 87. 1. Beware of AED-induced seizure 2. Trial of vitamins and co-factors considered in infantile epilepsies not responding to AED. 3. In children not responding to treatment with 2 AEDs, complete re-evaluation required for epilepsy surgery / trial of ketogenic diet. 4. Risk of carbamazepine-induced hypersensitivity reactions, is increased in patients with the HLA- B*1502 allele – consider testing if test easily available. 5. Avoid starting female of childbearing potential with sodium valproate
  • 88. 1. Drug level monitoring is not routinely done (except phenytoin) 2. Vitamin D supplementation should be considered for children with risk factors for Vitamin D deficiency 3. When withdrawal of medication is planned (generally after being seizure free for 2 years) 4. If seizures recur, the last dose reduction is reversed and medical advice sought.
  • 93. Sudden Unexpected Death in Epilepsy (SUDEP)
  • 95. SUDEP • The most common epilepsy related mortality in patients with chronic epilepsy • Incidence is unknown • Ranges from 1-5 per 1,000 people with epilepsy. • Precise etiology is unknown • Risk factors include – Polypharmacology, – Poorly controlled generalized tonic–clonic seizures, – Male gender, – Age younger than 16 yr, – Long duration of epilepsy, and frequent seizures.
  • 96. Cont. • Patients are usually found dead in their bed in a prone position with evidence suggesting a recent seizure. • Potential mechanisms of SUDEP include • Respiratory arrest or dysfunction, • Drug-induced cardiac toxicity, • CNS dysfunction (hypoventilation, arrhythmia, suppression of brain electrical activity), or • Pulmonary edema.
  • 99. Please re-evaluate for the following possibilities : • Is it a seizure or a non-epileptic event? • Wrong classification of epilepsy syndrome, thus wrong choice of antiepileptic drug. • Antiepileptic drug dose not optimised. • Poor compliance to antiepileptic drug. • Antiepileptic drug aggravating seizures. • Lesional epilepsy, hence a potential epilepsy surgery candidate. • Progressive epilepsy or neurodegenerative disorder.
  • 100. WHEN TO REFER TO PAEDIATRIC NEUROLOGIST?
  • 101. Refer immediately (to contact paediatric neurologist) : 1. Behavioural or developmental regression. 2. Infantile spasms..
  • 102. Refer: 1. Poor seizure control despite monotherapy with 2 different antiepileptic medications. 2. Difficult to control epilepsies beginning in the first two years of life. 3. Structural lesion on neuroimaging
  • 104. • Educate the child and family about epilepsy – Disease – Management – Limitation • Establish therapeutic alliances • Evaluate risk of involvement in athletic activities • Evaluate for possible learning disabilities • Resources in community • Informed family about increase risk of mortality • Educate what to do in epilepsy, medicine choices, side effect of drugs • Discuss about the potential complication of epilepsy (Discourage the continuous observation of child in wakeful and sleep state)
  • 107. • Recent classification, update and terminology of seizures, epilepsy and status epilepticus according to ILAE • Approach in child with first seizures • Approach in child with epilepsy • Approach in child with status epilepticus • Febrile seizure in pediatric • Understand the principle of anti-epileptic drug • Sudden Unexpected Death of Epilepsy (SUDEP) • Patients with “intractable epilepsy” • Management of seizure disorder in general, including first aid in seizure • Basic advices to parents (counselling) SUMMARY
  • 108. REFERENCES • Nelson Textbook Of Pediatrics, 20th Edition, Chapter:593, Seizures in Childhood, Mohamad A. Mikati and Abeer J. Hani, Page:2823 • Paediatrics Protocol For Malaysian Hospital, 4th Edition, Kementerian Kesihatan Malaysia, Chapter:46-48, Status Epilepticus, Epilepsy, Febrile Seizure , Page: 243 • ILAE

Notas do Editor

  1. Use words that mean what they say!! Target audience – not epileptologists GPs, medical students, paediatricians, neurologists, internists, psychiatrists, health workers, nurses, etc > 80% of medical population
  2. Here is a diagram that shows a conceptual network for generalized seizures involving the corticothalamic circuitry. Theoretically a generalized seizure could start at different points in the network and engage bilaterally distributed networks. Thus a seizure could start frontally or even parietally.The key point is that a generalized seizure can start from a focal point.
  3. Conceptual diagram with fMRI of GSW network
  4. Often a diagnosis regarding the type of epilepsy can be made (level 2: epilepsy classified by seizure type) and clinicians should strive to make a diagnosis at this level wherever possible. Added categories of “generalized and focal epilepsy” and “unknown if generalized or focal epilepsy”