Convulsive disorders in Children

(Febrile & Afebrile)

MBBS YEAR 3
DR.SWE ZIN AYE
zinaye.swe@qiup.edu.my
Learning Outcomes:
At the end of this session, the students are expected to:
• Recognise the features which distinguish between paroxysmal disorders and
epileptic seizures in children
• Awareness of the presence of other transient non-epileptic phenomena
resembling seizures
• Classify types of seizures and epilepsy syndrome.
• Understand febrile convulsion in medical care of children.
• Initial stabilization and investigations for a fitting child in emergency
department.
Seizure and paroxysmal disorders (‘funny
turns’)
Seizure = fit:
• a clinical manifestation in which there
is a sudden disturbance of
neurological function, usually
associated with an abnormal or
excessive neuronal discharges.
• Epilepsy
• Non-epilepsy—acute symptomatic
seizure
Non-seizure paroxysmal events
• often normal or benign phenomena,
although some are clinically significant
and consequential.
• Breath holding attack
• Reflex anoxic seizure
• Syncope
• Migraine
• Benign paroxysmal vertigo
• Long QT syndrome
Seizure and paroxysmal disorders (‘funny
turns’)
Seizure and paroxysmal disorders (‘funny
turns’)
• A thorough medical history from the patient and primary witnesses is the most
reliable tool for establishing the correct diagnosis.
• Pre-ictal
• Ictal
• Post-ictal
• The key to the diagnosis lies in a detailed history, which, together with clinical
examination, will determine the need for an EEG or other investigations.
Breath holding attack and reflex anoxic seizure
Breath holding attack and reflex anoxic seizure
Breath holding attack
• Occur in some toddlers when they are
upset.
• The child cries, holds his breath and
goes blue but rapidly recover fully.
• Drug therapy is unhelpful.
• Attacks resolve spontaneously, but
behaviour modification therapy, with
distraction, may help.
Reflex anoxic seizure
• Occur in infants or toddlers,a first-
degree relative with a history of faints.
• Commonest triggers are pain or
discomfort, particularly from minor
head trauma, cold food (such as ice-
cream or cold drinks), fright or fever.
• The child becomes very pale and falls
to the floor, brief generalised tonic–
clonic seizure and the child rapidly
recovers.
• The episodes are due to cardiac
asystole from vagal inhibition.
Causes of seizures
Epilepsy Non-epilepsy—acute symptomatic
• Idiopathic (70–80%) – cause unknown seizure
but presumed genetic • Febrile seizures
• Secondary • Metabolic
• Cerebral dysgenesis/malformation • Hypoglycaemia
• Cerebral vascular occlusion • Hypocalcaemia/hypomagnesaemia
• Cerebral damage, e.g. congenital • Hypo/hypernatraemia
infection, hypoxic-ischaemic • Head trauma
encephalopathy, intraventricular
haemorrhage/ischaemia • Meningitis/encephalitis
• Cerebral tumour • Poisons/toxins.
• Neurodegenerative disodrders
• Neuro-cutaneous syndromes
Non-epilepsy—acute symptomatic seizure
Febrile seizures
• A febrile seizure is a seizure accompanied by a fever in the absence of intracranial
infection due to bacterial meningitis or viral encephalitis.

• Affect 3% of children; have a genetic predisposition 10% risk if the child has a
first-degree relative with febrile seizures.
• Occur between 6 months and 6 years of age.
Febrile seizures
Management
• Because febrile seizures are brief and the outcome is benign, most children
require no treatment.
• 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.
• Administration of antipyretics during febrile illnesses does not prevent febrile
seizures.
Prognosis of febrile seizures
• About 30–40% will have further febrile seizures.
• This is more likely
• the younger the child,
• the shorter the duration of illness before the seizure,
• the lower the temperature at the time of seizure and
• if there is a positive family history.

• Simple febrile seizure: 1–2% chance of developing epilepsy, similar to the risk for
all children and no risk of brain damage.
• Complex febrile seizures: have an increased risk of 4–12% of subsequent epilepsy.
Epilepsy
Epilepsy is defined as recurrent, unprovoked seizures.

• Epileptic seizures are generally classified as: (International League Against

Epilepsy (ILAE) 2010 Classifications).
• focal (or partial), arising from one region of the cortex, or
• generalized seizures, which arise from both hemispheres, simultaneously

• The incidence of childhood epilepsy is 1% to 2%.

ILAE Classification of Seizures
Seizures

Partial Generalized

Simple Partial Absence

Complex Partial Myoclonic

Secondarily
Spasm
Generalized

Atonic and tonic

Tonic-Clonic
American Epilepsy Society 2008
Epilepsy is a clinical diagnosis
Partial seizures
• Onset in one of the cerebral
hemisphere
• Begin in a small group of
dysfunctional neurons
• May be preceded by an aura
which reflects the site of origin
Complex Partial seizures
• Altered conscious level, post-ictal
confusion or drowsiness.
• Automatism, amnesia for the
event.
Generalized seizures
• Always loss of consciousness
without warning
• Symmetrical
• Looks at the pattern, could
differentiate into absence,
myoclonic, tonic, tonic clonic,
atonic seizures
• It means caregivers must be able
to describe the event clearly.
Partial seizures
Frontal Occipital
• Clonic movements, which may • Distortion of vision
travel proximally (Jacksonian
march)
Parietal
• Asymmetrical tonic seizures
• Contralateral dysaesthesias
Temporal (altered sensation)
• Aura of smell / taste abnormalities • Distorted body image
• Distortion of sound and shape
• Automatism
• Deja-vu, jamais-vu
Partial seizures
Benign Rolandic Epilepsy or Benign* epilepsy,
with centrotemporal spikes (BECTS)
• Infrequent simple partial seizures -
tingling in mouth, on face, speech
arrest
• Seizures often occur during sleep
• Partial seizure (facial twitching and
ipsilateral jerking), may be generalized
tonic-clonic
• The most common benign epilepsy in
childhood
• Onset ages 2-12 yrs, peak 5-10 yrs
Benign Rolandic Epilepsy or Benign* epilepsy,
with centrotemporal spikes (BECTS)
• EEG shows focal sharp waves from the
Rolandic or centrotemporal area.
• Important to recognise as it is benign
and does not always require
treatment.
• Treatment: carbamazepine
• Almost all remit in adolescence
Absence seizures
• Transient loss of consciousness, with
an abrupt onset and termination,
unaccompanied by motor phenomena
except for some flickering of the
eyelids and minor alteration in muscle
tone.
• Two-thirds are female.
• 4–12 years
• May be typical (petit mal, <15
seconds) or aytpical (>15 seconds with
motor manisfestation),
• The episodes can be induced by
hyperventilation,
Absence seizures
• EEG-generalised 3/second spike and
wave discharge
• First line--Valproate, ethosuximide
• Second line--Lamotrigine
• Prognosis is good, with 95% remission
in adolescence; 5–10% may develop
tonic-clonic seizures in adult life.
• Developmentally normal but can
interfere with schooling.
Juvenile Myoclonic seizures
• Brief, often repetitive, jerking
movements of the limbs, neck or
trunk
• Present between ages of 10 and 20
years
• Female affected twice as often as
males
• Triggers: AM wakening, lack of sleep,
fatigue, ETOH, and fasting
• But tonic-clonic seizures and absences
also occur (this brought the patient to
seek treatment)
Juvenile Myoclinic seizures
• EEG with 3-6 Hz multi-spike and wave
• Photosensitivity in 27%-41%
• Focal EEG abnormalities in up to 55%
• Response to treatment (sodium
valproate) is usually good but lifelong
treatment
• Learning is unimpaired
Infantile Spasm
• Spasms of the head, trunk and limbs
followed by extension of the arms (so-
called ‘salaam spasms’).
• 4–6 months
• often on waking, but may occur many
times a day.
• Mistaken as colic:
• 2/3 of the children are neurologically
abnormal before onset of seizure (HIE,
brain malformation; we called them
symptomatic)
Infantile spasm
• EEG - hypsarrhythmia,
• a chaotic pattern of high-voltage slow
waves, and multi-focal sharp wave
discharges
• Treatment is with vigabatrin /ACTH/
corticosteroids;
• good response in 30–40%,
• Most will subsequently lose skills and
develop learning disability or epilepsy
Infantile spasm
West syndrome is the triad of:
1. infantile spasms,
2. developmental regression, and
3. a dramatically abnormal EEG
pattern (hypsar- rhythmia).
The underlying etiology of the spasms
dictates the prognosis; >200 different
etiologies
• tuberous sclerosis,
• malformations of cortical
development (lissencephaly),
• genetic syndromes (trisomy 21),
• acquired brain injury (stroke,
perinatal hypoxic-ischemic
encephalopathy),
• metabolic disorders
(phenylketonuria).
• Unkonown
Atonic drop seizure
• Often combined with a myoclonic
jerk, followed by
• a transient loss of muscle tone causing a
sudden fall to the floor or drop of the
head
• Although atonic seizures are typically
brief (lasting 1 to 2 seconds),
• they are quite disabling because of a
sudden loss of postural tone, resulting in
falls and injuries.
Diagnosis
• Primarily based on detailed history
• Eyewitness
• Video, if available
• Clinical examination to look for neurological abnormalities

Epilepsy syndromes
•Groups of characteristic clinical features related to age of onset of seizures, family history of
epilepsy, seizure type(s), and associated neurological symptoms and signs, aided by appropriate
investigations, including EEG and Brain imaging (CT brain or MRI brain).
•It is useful, helps the clinician to define the likely prognosis, provide reasonable genetic
counseling and choose most appropriate syndrome
Physical examination
• General examination
• Vital signs
• All systems

• Detailed neurological examination

• Developmental assessment
• Fundoscopy
• Head circumference
• Testing for neurological deficits
• Neurocutaneous stigmata
Investigations
• Routine blood tests
• Rule out hypoglycaemia, hypocalcaemia
• FBC, urea, LFT, BUSE before starting anticonvulsants
• EEG:
• Is recommended only after a second afebrile fit
• Help support the clinical diagnosis of epileptic seizures
• Identify specific epilepsy syndromes, hence choice of AED
• Localization of seizures foci in intractable epilepsy
• Normal EEG does not rule out epilepsy
• Abnormalities may be seen better during sleep
• 24-hour ambulatory EEG
• often interictal EEG is normal. Just 20 minutes recording
Investigations
• CT and MR brain (epileptic protocol)
• indicated if history suggestive of space occupying lesions, there is focal neurological signs
• Epilepsy occurring at the first year of life except febrile seizures
• partial epilepsy except benign rolandic epilepsy
• developmental delay or regression
• Metabolic screening for unexplained global delay
• Monitor drug levels (usually with carbamazepine, phenytoin, phenobarbitone)
• to check compliance, if seizures not well controlled despite adequate doses, or
• in situations of polypharmacy when drug interaction is suspected.
Status epilepticus
• Status epilepticus, a seizure lasting 30 min or repeated seizures for 30 min
without recovery of consciousness.
• After immediate primary assessment and resuscitation, the priority is to stop the
seizure as quickly as possible
Status epilepticus
Principles of anticonvulsant therapy for
Epilepsy
• Treatment recommended 2 or more episodes (recurrent risk up to 80%)
• Attempt to classify the seizure type(s) and epileptic syndrome.
• Monotherapy, choose most appropriate drug,
• Increase dose gradually until epilepsy controlled or maximum dose reached or
side effects occur
• Add on second drug if first drug failed. Optimize second drug, then try to
withdraw first drug (alternative monotherapy)
Principles of anticonvulsant therapy for
Epilepsy
• Rational combination therapy( (usually 2 or maximum 3 drugs) i.e. combines
drugs with different mechanism of action and consider their spectrum of efficacy,
drug interactions and adverse effects.
• Valproate and Lamotrigine and clonazepam is good combination
• Lamotrigine and carbamazepine (not good)
• Phenytoin and carbamazepine (not good)
 Which Drug? BLOCK Glutamate antagonists
EXCITATION Phenobarbitone
Felbamate
excitation

resting membrane
potential
X+ Topiramate
Na Channel blocker
Phenytoin
Carbamazepine
Lamotrigine
Topiramate
STABILISE Valproate
MEMBRANE Ca Channel blocker
POTENTIAL Zonisamide
Carbonic anhydrase inhibitor
Topiramate
Zonisamide
GABA agonists
inhibition - POTENTIATE
INHIBITION
Benzodiazepines
Diazepam, Midazolam
? Valproate
Unknown mode of action :
GABA transaminase inhibitor
Levetiracetam (Synaptic Vesicle 2A receptor), Vigabatrin
Paraldehyde
Selecting anticonvulsants according to seizure
types
Seizure type First choice Second choice
Generalized epilepsy
Tonic clonic valproate Purposely omitted. Please
check Our second line
Absence/aytpical absence valproate include:
Myoclonic Sodium valproate / Levetiracetam,
clonazepam lamotrigine,
Topiramate
Partial epilepsy Carbamazepine
Infantile spasm ACTH, prednisolone, Nitrazepam, clonazepam,
Vigabatrin valproate

Carbamazepine may cause worsening absence and myoclonic seizure.

Managing Pediatric Epilepsy
• Consider chewable/liquid formulations
• Weight-based dosing with frequent adjustments to account for growth
• Minimize missed school
• Develop safety plan with family
• For intractable epilepsy consider
Ketogenic diet
Surgery
Vagal nerve stimulation
How to withdraw drugs?
• Seizure-free two years, consider drug withdrawal.
• Withdrawal over 3-6 months (maybe longer if clonazepam or phenobarbitone).
• If seizures recur, the last dose reduction is reversed and medical advice sought
(withdrawal effect), try again.
Advice for parents
• Educate and counsel on epilepsy
• Emphasize compliance if on anticonvulsant
• Don’t stop the medication by themselves. This may precipitate breakthrough
seizures.
• In photosensitive seizures – watch TV in brightly lit room.
• Avoid sleep deprivation.
• Sudden unexpected death in epilepsy (SUDEP) is now believed to account for up
to 17% of all epilepsy-related death.
• No cycling in traffic, climbing sports, or swimming alone
• Know emergency treatment for seizure
• Inform teachers or school about the condition
Prognosis
The majority of patients with epilepsy have a good prognosis
•Many children outgrow epilepsy, the condition will remit with time e.g., Childhood
absence epilepsy, Benign epilesy with centro-temporal spikes
•Overall, 60-70% of patients become seizure free after treatment with epileptic
drugs.
•Factors that indicate a poor prognosis include
•Symptomatic cause
•High seizure frequency before AED treatment
•Generalized tonic-clonic seizures
•Generalized epileptiform activity on the EEG
•Family history of epilepsy
•Comorbid psychiatric history.
Conclusion
You should now be able to:
• Use your diagnostic reasoning to formulate a symptom-dependent list of
differential diagnoses for a child presenting with convulsion.

• Use your clinical reasoning to decide when it is necessary to investigate a child

with seizure and what investigations are needed

• Describe how to manage common illnesses causing convulsion in children

• Be able to explain to parents the management of seizure in children

References:
• Nelson Essentials of Pediatrics 7th edition 2015 by K. Marcdante MD,R. M.
Kliegman MD,R. E. Behrman MD
• Malaysia paediatrics protocol, 3rd Edition.
• Illustrated Textbook of Paediatrics (4th Edition) | International Edition by Tom
Lissauer, Will Carroll