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.
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.
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
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
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.
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).
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.
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.
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.
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
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
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.
Conceptual diagram with fMRI of GSW network
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”