unable to upload the case scenario-sorry

2. • Case scenario…

3. Introduction
• One of the commonest neurological disorder in children
• Data from U.S:
• 120,000 children seen annually for new onset seizure
• 1% experience afebrile seizure by 14 years old
• 5% experience febrile seizure by 6 years old
• From those diagnosed epilepsy- >50% had their first seizure during
childhood or adolescent
• 1st seizure can cause significant anxiety to parents and medical
practitioners
A.B. Chelse, et. al, Initial Evaluation and Management of a First Seizure in Children; Pediatric Annals (Impact Factor: 0.29). 12/2013; 42(12):244-8. DOI: 10.3928/00904481-20131122-08

4. Provoked vs. Unprovoked Seizure
• Provoked- characterized by specific trigger e.g.
CNS infection, head trauma, fever, intoxication,
hypoxia
• Unprovoked- not associated with obvious
precipitating cause & may be related to epilepsy

5. Clinical History is the ‘KEY’ when
determining the likelihood recurrence after
a child experience FIRST afebrile seizure

6. 5 questions that we should try to answer
1. Was the episode an epileptic seizure?
2. What is the cause of the seizure?
3. What investigations should I do?
4. Does the child require treatment?
5. What else should I think about?
Aspasia Michoulas, Kevin Farrell, Mary Connolly: approach to a child with a first afebrile seizure; BC medical journal vol. 53 no. 6, July/August 2011

7. Q1: WAS THE EPISODE AN EPILEPTIC SEIZURE??
• Making correct diagnosis in 1st transient neurological event is
important
• Up to 30% of cases referred are misdiagnosed as epilepsy
• Need to identify more common non-epileptic paroxysmal
episodes

8. Common Imitators:
Can be group according to the characteristic of seizures they imitate
• Movement Imitators
• Loss of Consciousness Imitators
• Confusion Imitators
• Psychological Imitators

9. • Day dreaming
• Stereotypes & tics
• Panic/anxiety attack
• Psychogenic non-epileptic seizure ( PNES a.k.a
pseudoseizure)
• Sleep disorder
• Migraine
• Transient ischemic attack/ stroke
• Paroxysmal movement disorder
• Syncope or reflex anoxic seizure that may
include:
 Vasovagal syncope
 Breath holding spells
 Orthostatic hypotension
 Cardiac abnormality (arrhythmias or
prolonged QT syndrome)
 GERD (Sandifer syndrome)
 Hyperventilation syncope

10. History:
• Description of whole events by eyewitness
• Followed by focused questions
• Pre-ictal
• Ictal
• Post-ictal
• Other important questions to ask:
• Previous seizure episode/ febrile seizure
• Developmental history
• Past medical history, current medications, possible intoxication, social history
• Family history of epilepsy

11. Pre-ictal:
• Was there any warning before the spell? If so, what was
it
• Did the child complain of abdominal discomfort, fear or
any other unpleasant sensation before the spell
• What was the child doing prior to spell

12. • Was the child asleep or awake prior to event
• Was the child sleep deprived
• Were there any triggers for the spell
• Was the child well before the spell or was there
any fever or illness

13. Ictal
• Was the child conscious during event
• Did the child remember anything that occur during the spell
• Were there any repetitive behaviors during the episode e.g. lip
smacking, pulling shirts
• Did any body movement occur
• Was there any perioral cyanosis

14. • What was the child skin color during the event
• Did the child have incontinence during the spell
• How long does the spell last
• How many episode has the child experienced
• How often does the spell occur

15. Post-ictal
• How did the patient feel after the spell
• Did the child seem confuse and tired after the
spell
• How long did it take for the child to get back to
baseline condition

18. Situational clues to paroxysmal events at different age
INFANT TODDLER CHILD ADOLESCENT
In sleep Benign neonatal sleep
myoclonus
Night terrors • Parasomnias
• Confusion
arousal
• Parasomnias REM sleep
disorder (e.g. sleep
paralysis)
• Sleep walking
On feeding • GERD, Sandifer
Syndrome
• Shuddering spells
GERD (Sandifer syndrome) - -
Fever, intercurrent
illness
- Febrile seizure Syncope Syncope
Movement - (kineosogenic) Paroxysmal
dystonia, dyskinesia
(kineosogenic)
Paroxysmal
dystonia, dyskinesia
(kineosogenic) Paroxysmal
dystonia, dyskinesia
Pain, shock • Structural cardiac
or dysrhythmia
• Reflex anoxic
seizure
• Structural cardiac or
dysrhythmia
• Reflex anoxic seizure
• Cyanotic breath holding
• Structural
cardiac or
dysrhythmia
• Syncope
• Structural cardiac or
dysrhythmia
• Syncope

19. INFANT TODDLER CHILD ADOLESCENT
Hot weather,
prolonged
standing
- - - Syncope
Tired, bored, meal
times, bed times
Self-gratification
events
Self-gratification
events
- -
Tired, bored,
stress
- - Tics Tics
Excitement,
emotion
Shuddering spell - - Cataplexy
Boredom - Self gratification Ritualistic
behavior*
Ritualistic behavior*
Absorbed in TV or
computer games
- - Distracted Distracted
* Especially in children with learning difficulties

20. Types of seizure
TYPES DESCRIPTIONS
Jerk, shake Clonic (rhythmic contractions followed by slightly slower relaxation)
 Typically occurring immediately after a tonic phase as part of tonic-clonic seizure
or confined to a body part as focal seizure
Myoclonic seizures
 Isolated lightning-fast, brief contractions occurring singly or in short runs
 They occur as infantile spasms but also in older children
Spasms
 Sometimes referred to as tonic spasms
 Have a slightly longer phase of sustained contraction than myoclonic jerk
 Typically occur in runs
 They occur as infantile spasms but may happen in older children
** differentiating these seizure types may be challenging clinically, and is a
particularly important purpose of EEG

21. Types of seizure
TYPES DESCRIPTIONS
Stiff  Usually tonic seizure (sustained contraction for several seconds)
 They may be low-amplitude ‘vibratory’ element to the contraction that is different
from a clonic movement
Fall  Beware the phrase “drop attack”: it can be ambiguous
 Atonic seizure results in slump to the ground ‘as if a puppet had its string cut’
 Tonic seizure resulting in rigidity and can cause the child to fall “ like a felled tree”
 In large myoclonic jerk child can be thrown to the ground
 In some seizures these are combined, as in Doose syndrome
** many centres use EMG alongside EEG in order to determine whether, and for how long, there
is muscle contraction associated with a seizure. This helps to distinguish tonic, atonic and
myoclonic event

22. Types of seizure
TYPES DESCRIPTIONS
Vacancy  The word ‘absence’ creates a lot of confusion and diagnostic
imprecision
 An absence is a type of seizure (defined by its EEG features) that can
cause LOC, but LOC can also occurs in focal seizure
Most absence are brief, few seconds, but they may occur many times
per day
 Often associated with subtle automatism e.g. lip smacking, chewing,
fiddling with the hands, or eyelid flickering
Focal seizure (e.g. originating from temporal lobe) may be associated
with LOC and unresponsiveness to surrounding.
 They would typically be longer (30s or more) and less frequent than
absences with more marked confusion or agitation
 EEG can make a valuable contribution to differentiate these 2 types

23. Q2: WHAT IS THE CAUSE OF SEIZURE??
• ILAE has 3 broad causal categories:
• Symptomatic
• Idiopathic
• Presumed symptomatic (previously known as cryptogenic)
Rob Forsyth, Richard Newton (eds.). Paediatric neurology. 2nd ed. Oxford: Oxford university press, 2012

26. Aspasia Michoulas, Kevin Farrell, Mary Connolly: approach to a child with a first afebrile seizure; BC medical journal vol. 53 no. 6, July/August 2011.

27. Aspasia Michoulas, Kevin Farrell, Mary Connolly: approach to a child with a first afebrile seizure; BC medical journal vol. 53 no. 6, July/August 2011.

28. Examples of Epilepsy Syndromes
Onset Examples
Neonatal  Benign Familial Neonatal Seizures (BFNSs)
 Benign Neonatal Seizure (BNSs)
 Early Infantile Epileptic Encephalopathy (EIEE) (Ohtahara syndrome)
 Early Myoclonic Encephalopathy (EME)
Infant  Infantile Spasms (West Syndrome)
 Benign Myoclonic Epilepsy of Infancy (BMEI)
 Severe Myoclonic Epilepsy of Infancy (Dravet Syndrome)
Pre-schooler • Severe Myoclonic Epilepsy of Infancy (Dravet Syndrome)
• Some progressive myoclonic epilepsies
• Lennox Gastaut Syndrome
• Epilepsy with Myoclonic-Astatic Seizures (Doose syndrome)
Rob Forsyth, Richard Newton (eds.). Paediatric neurology. 2nd ed. Oxford: Oxford university press, 2012

29. Onset Examples
Child  Benign Childhood Epilepsy with centrotemporal spikes (BECTS- Rolandic
Seizure)
 Childhood absence epilepsy (CAE)
 Epilepsy with myoclonic absence (EMA)
 Childhood Epilepsy with Occipital Paroxysms (CEOP- Panayiotopolous type)
 Landau-Kleffner Syndrome (LKS)
Adolescent  CEOP (Gastaut type)
 Juvenile Absence Epilepsy (JAE)
 JME
 Epilepsy with GTCS seizures on awakening
Rob Forsyth, Richard Newton (eds.). Paediatric neurology. 2nd ed. Oxford: Oxford university press, 2012

30. Benign Childhood Epilepsy with
Centro-temporal Spikes
(BECTS) a.k.a Rolandic Seizure
THE MOST COMMON FOCAL ONSET EPILEPSY IN
CHILDHOOD, YET OFTEN UNDER-RECOGNIZED

31. • Syndrome of brief, simple, partial, hemifacial motor seizures,
frequently having associated somatosensory symptoms which have a
tendency to evolve into generalized tonic clonic seizures (Secondary
GTCS are reported between 1/3 to 2/3 of children)
• Both seizure types are often related to sleep
• Onset occurs between the ages of 3 and 13 years (peak 9–10 years) and
recovery occurs before the age of 15–16 years
• Genetic predisposition is common, and there is a male predominance

32. • EEG has blunt high-voltage centrotemporal spikes, often
followed by slow waves that are activated by sleep and tend
to shift or spread from side to side
• Duration usually brief 1-2min but may become longer if
seizures progress to convulsion
• Prognosis excellent with a less than 2% risk of developing
infrequent generalized seizures in adult life; absence
seizures may be more common than GTCS

33. Hemifacial sensorimotor seizures
• 1/3 of patients
• May be the only ictal manifestation but are often associated with
inability to talk and hypersalivation
• Mainly motor seizures- that may be entirely localized to lower lip
manifesting with sudden, continuous or burst of clonic contractions
• Lasting from few seconds to 1 minute

34. • Ipsilateral tonic deviation of the mouth is also common
• Rarely, hemifacial convulsions may appear nearly
simultaneously or spread to ipsilateral upper extremity,
involvement of leg is rare
• Hemifacial sensory seizures are less common and consist of
numbness in the corner of the mouth
• Conscious level preserved

35. Oropharyngo-laryngeal ictal manifestation
• Occurred 53% of seizures
• The most characteristic of all other ictal symptoms
• Consist of unilateral sensory and motor manifestation inside
the mouth, tongue, inner cheek, gums, teeth and pharyngo-
laryngeal region
• Sensory- unilateral numbness/ paresthesia
• Motor- strange sound e.g. death rattle, gargling, grunting,
guttural sounds and their combinations

36. Arrest of speech
• More of ananarthria (loss of power and coordination for
the articulation of words)
• Occurs in >40%
• Child is inarticulate and attempts to communicate with
gestures
• A few mainly laryngeal sounds, not words, may be uttered
particularly at the beginning
• Hypersalivation
• Often associated with other ictal symptoms

37. Management:
• May not need AED especially if seizures are
• Infrequent
• Mild or nocturnal
• Onset is close to the age of natural remission of this age-limited disorder
• May need AEDs if:
• Frequent seizures ± secondary GTCS
• Comorbid- tics, ADHD, learning disability
• AEDs may reduce GTCS but not focal seizure
• Empirically – carbamazepine is preferred
• Other AEDs- gabapentin, levetiracetam, sulthiame, sodium valproate

38. Q3: WHAT INVESTIGATIONS SHOULD I DO??
BLOOD INVESTIGATIONS
• FBC/ electrolytes/glucose level
• Additional tests:
• Hemorrhagic basis - Coagulation profile
• Toxicology – blood or urine
• Genetic disease - possible karyotypes and other test specific to
illness
• Metabolic disease – ammonia, lactate, IEM screening

39. LUMBAR PUNCTURE
• Indicated when suspicious of brain infection
EEG
• Most useful
• Recommended in all children who experienced unprovoked
seizure
• Still, abnormal EEG may not necessarily means epilepsy and
children with epilepsy may have normal EEG

40. NEUROIMAGING
• Best is MRI brain
• Indicated when:
• Onset before the age of 2 years old
• Focal onset seizure
• Focal neurological abnormality
• Abnormal EEG
• Abnormal neurological examination
• CT scan brain may be indicated to look for acute
symptomatic seizure e.g. post trauma

41. Q4: DOES THE CHILD REQUIRE TREATMENT??
• Usually not started on treatment until 2nd unprovoked seizure
• Indications for consideration of medication after 1st unprovoked seizure:
• Patient who presents with status epilepticus
• Patient with multiple risk factors to develop 2nd seizure
• Patient with abnormal EEG
• Starting treatment – case by case basis and after thorough discussion made
between physician and caregivers/ patients

46. Q5: WHAT ELSE SHOULD I THINK ABOUT??
Recurrence risk after 1st unprovoked seizure
• Two year risk 40-52%
• 24% with no risk factor and normal EEG
• 65% remote neurological insult and epileptiform EEG
• Dutch Study:10 children with epileptiform EEG patterns after their
first seizure had a 2-year risk of 71%
• Two unprovoked non febrile seizures, the chance by 4 years is 73%,
with a 95% confidence interval 60–90%
Berg AT, Shinnar S. The risk of recurrence following a first unprovoked seizure. Neurology 1991;41:965–72 Kim LG, Johnson TL, Marson AG, et al. Prediction of risk of seizure recurrence after a
single seizure and early epilepsy: further results from the MESS trial. Lancet Neurol 2006;5:317–322 Shinnar S, Berg AT, Moshe SL, et al. Risk of seizure recurrence following a first unprovoked
seizure in childhood: a prospective study. Pediatrics 1990;85:1076–1085

47. • Assessing a child fully correct assessments have to be
made at least 4 levels
• Disease: am I sure this is an epilepsy & not either a disease
causing recurrent acute symptomatic seizure, or a disease
causing events that are not seizure at all
• Seizure types: what semiology(s) am I seeing?
• Epilepsy syndrome
• Etiology

48. • If child’s epilepsy treatment is proving ineffective, review at each of these
four levels:
• Am I sure it is epilepsy?
• Have I misinterpreted the seizure descriptions and selected the wrong drugs?
• Have I missed a possibly progressive underlying cause?
• Referral to neurologist: (Malaysian paediatric protocol 3rd edition)
• Immediately if:
• Behavioral or developmental regression
• Infantile spasms
• Non-urgent if:
• Poor seizure control despite monotherapy with 2 different anti-epileptic
medication
• Difficulty to control epilepsies beginning in the first 2 years of life
• Structural lesion on neuroimaging

50. Epilepsy
and
Daily Life

51. • Psychosocial consequences of epilepsy can be
more debilitating than seizure disorder itself
• Informed choices about activities need to be
made on individual basis depending on type
and frequency of seizures and level of control
with medications
• Aim should be made to maximize
participations in all age- appropriate aspects
of life whilst taking realistic approach to risk
management

52. SCHOOLING
• Most attend mainstream school – may have evidence of
underachievement
• Neuropsychometry is recommended to define educational strengths
and weaknesses and aid tailoring educational support
• Other mechanisms of adverse impact on schooling include
• Nocturnal seizure and poor sleep
• Brief undetected epileptic discharge
• Drug toxicity
• Inappropriate teacher and parental expectations
• Absence from school
• Low self esteem
• Anxiety to poor adjustment and stressor at home

53. EMOTIONAL ADJUSTEMENT
• Involves living with unpredictability – may erode self esteem
and confidence
• Family prefer to live with predictable seizure pattern than risk
AED manipulation in attempt to control seizure
• Unwanted effects of medications e.g. weight gain, hirsutism –
detrimental to self esteem
• High and low of successive treatment failures
• Anxiety, depression

54. RESPONSES
• Establish good communication between
health, patient, family
• Education
• Encourage self-esteem, avoid
unnecessary restrictions
• Minimize time off for clinic
• Ensure full education
• Sensitive monitoring
• Avoid seizure trigger

55. IN AND AROUND WATER
• Should be accompanied
• Shower vs. bath tub
CYCLING
• Avoid traffic
• Protective gear
PHOTOSENSITIVITY
• Current IT gadgets/ TV screen /computer screen – flicker free
• Avoid getting too close with monitors

56. DRIVING
• Depends on requirement, e.g. must be seizure free for
at least 12 months
DEATH IN EPILEPSY
• Epilepsy-related death in children may be due to
• Complications of seizure
• Convulsive status epilepticus
• Related underlying disorder

57. • SUDEP (Sudden Unexpected, non-traumatic and
non-drowning death in in children with epilepsy)
• Risk factors for SUDEP
• Young adult male
• Early age of onset of seizure
• GTC seizures
• Increased frequency of seizure
• Polytherapy and poor adherence to AED treatment

58. Take Home Messages:
1. Differential diagnosis of 1st seizure is broad
2. Most important diagnostic modality in evaluation of 1st
seizure is the history and physical examination
3. Epilepsy may be diagnosed after 1 unprovoked seizure
and treatment may be initiated
4. 70% - may achieve full seizure control with treatment
5. AED decision- based on seizure type/epilepsy syndrome
6. Wrong AED may worsen seizure

59. References:
1. Rob Forsyth, Richard Newton (eds.). Paediatric neurology. 2nd ed. Oxford: Oxford university press, 2012
2. Aspasia Michoulas, Kevin Farrell, Mary Connolly: approach to a child with a first afebrile seizure; BC medical
journal vol. 53 no. 6, July/August 2011
3. A.B. Chelse, et. al, Initial Evaluation and Management of a First Seizure in Children; Pediatric Annals (Impact
Factor: 0.29). 12/2013; 42(12):244-8. DOI: 10.3928/00904481-20131122-08
4. Ghofrani M. Approach To The First Unprovoked Seizure- PART I. Iran J Child Neurol. 2013 Summer; 7(3): 1- 5
5. Robert S. Fisher et. al, A practical clinical definition of epilepsy; Epilepsia,55(4):475–482,2014
doi:10.1111/epi.12550
6. Malaysian Paediatric Protocol, 3rd Edition
7. Laura Speltz, M.D. , Assessing First Seizures in Children and Adolescents, A pediatric perspective, Gillette
Children’s Specialty Healthcare, volume 23, Number 1, 2014
8. The Epilepsies: Seizures, Syndromes and Management, Panayiotopoulos CP, Oxfordshire (UK): Bladon Medical
Publishing; 2005.

60. Thank you…