Febrile seizures,Febrile seizure plus,

first unprovoked seizures,epilepsy

Case discussion, tips and tricks

Setyo Handryastuti
UKK Neurologi IDAI
Case 1

EEG

2 yrs, normal,
high fever, gen • EEG and head CT has been
EEG : seiz, 6 episodes done due to recurrent episode
Imaging
epileptiform since 1 yrs. Freq • Epileptiform in EEG → treat
3-4 as epilepsy
times/episode • AED → how long ?

Therapy
Case 2

FS or
epilepsy
?

8 yrs, febrile • Epilepsy or not ?

Imaging
with high • EEG and CT has been done
?
fever. Hx FS at EEG ? • Therapy ?
2 and 3 yrs of
age

Therapy
?
Case 3

Therapy ?
• EEG and Imaging has been
done
EEG/Imagin • Epilepsy or not ?
Diagnosis ?
g? • AED → how long ?

7 yrs, first
afebrile
seizures ,
Hx FS at
toddler
Case 4

Therapy ?
• EEG and Imaging has been
done
Diagnosis EEG ? • Epilepsy or not ?
• AED → how long ?

7 yrs, first
unprovoked
seizures ,
No Hx of
seizures
Case 5

Diagnosis

• Imaging ?
• Normal EEG → epilepsy ?
Unprovoked
• AED treatment ?
Imaging seizures, 3 Therapy
? episodes in ?
6 months

Normal
EEG
Definition

• Seizures occurring in association with an acute systemic

Provoked (immune/infection), metabolic, toxic, CNS insult

seizures • HypoNa, hypo/hyperglicemia,hypoCa, trauma, stroke,CNS

infection

• Maybe related to epilepsy

Unprovoked • Seizures do not require an immediate precipitating event
seizures • Maybe related to underlying neurological disorders that may
predispose a child to recurrent seizures.

• A seizure in association with a febrile illness in the absence

Febrile of a CNS infection/acute electrolyte imbalance, more than 1

seizures month of age without prior afebrile seizures

• Temperature must be greater than 380C

Pellock’s Pediatric Epilepsy Diagnosis and therapy. 2017.

ILAE Comission Report.Epilepsia. 1997;38(5):614-8.
Rekomendasi Penatalaksanaan Kejnag Demam.UKK Neurologi IDAI 2016
Definition

Febrile seizure plus • Febrile seizures beyond the typical limit of 6 years
• Afebrile convulsion in addition to the FS
(FS+)

• Generalized epilepsy febrile seizure plus

GEFS+ • Typical FS, FS+, isolated afebrile convulsions

• A seizure, or flurry of seizures all occurring within 24

First unprovoked
hours in a patient over 1 month of age with no prior history
seizures of unprovoked seizures.

• > 2 unprovoked/reflex seizures occurring greater than 24 hours apart

Epilepsy • One unprovoked/reflex seizure and probability of further seizures similar to he

general recurrence risk at least 60%) after 2 unprovoked seizures

Pellock’s Pediatric Epilepsy Diagnosis and therapy.2017.

Report of QSS of the AAN and the Practice Committee of the CNS. Neurology.2003;60:166-75.
ILAE Comiision Report. Epilepsia2014;55:475-82
Febrile seizures
Clasification of Febrile seizures

Simple Febrile Seizures Complex Febrile Seizures

Duration < 15 minutes Prolonged (> 15 minutes)
Status epilepticus (>30 minutes)
Phenotype Generalized tonic-clonic Clonic and or tonic
Focal onset
Focal become generalized
Recurrence frequency No recurrence in 24 hours Recurrence within the same
febrile illness
Prior neurology None Present
diagnosis
Post ictal pathology None Present

Berg AT, Shinnar S. Epilepsia. 1996;37(2):126-33.

American Academy of Pediatrics, Subcommitee on Febrile Seizure. Pediatr. 2011;127:389-94.
Indication of EEG and Imaging

• Electroencephalography (EEG)
• Only focal seizures
• Does not predict epilepsy

• Imaging
• Not routinely in simple febrile seizures
• Focal seizures, persistent focal neurology abnormality

Rekomendasi Penatalaksanaan Kejang Demam.UKK Neurologi IDAI 2016

Wong V. HK Journal of Pediatr. 2002;7:143-151.
AAP, Subcommitee on Febrile Seizure. Pediatr. 2011;127:389-94.
Therapy

Intermitten prophylaxis : one of the following criteria

• Severe neurology deficit : Cerebral palsy
• Recurrence > 4 times/year
• Age less than 6 months
• Seizures occurred at body temperature less than 390C
• Body temperature increased very rapidly

Rekomendasi Penatalaksanaan Kejang Demam.UKK Neurologi IDAI 2016

Sugai K. Brain Dev. 2010;32:64-70.
Recommendations for the management of febrile seizures: Ad Hoc Task
Force of LICE Guidelines. Epilepsia.2009;50(1):2-6.
Therapy

Maintenance prophylaxis :
• Focal seizures
• Seizures >15 minutes
• Neurology deficit before and after seizures : cerebral
palsy, hydrocephalus, hemiparesis.
• Intermitten prophylaxis first before maintenance
prophylaxis.

Rekomendasi Penatalaksanaan Kejang Demam.UKK Neurologi IDAI 2016

Sugai K. Brain Dev. 2010;32:64-70.
American Academy of Pediatrics. Practice parameter: Long-term treatment
of the child with simple febrile seizures. Pediatrics. 1999;103:1307-9.
Case 1

EEG

2 yrs, normal, • No indication for EEG and

gen seiz, 6 imaging
EEG : Head-
episodes since 1
epileptiform
yrs. Freq 3-4 CT • Provoked seizures, febrile
times/episode seizures whatever EEG said
• Not epilepsy
• Prophylaxis therapy can be
considered

Therapy
FS+ and GEFS+
Generalized epilepsy febrile seizure plus

Epilepsygenetic.net
Generalized epilepsy febrile seizure plus

Neuropsykey.com
Generalized epilepsy febrile seizure plus

Neuropsykey.com
Therapy
• FS+
• Treated as febrile seizures
• No formal guidelines
• AED can not prevent development of epilepsy
• GEFS+
• Depends on the seizure type/broad-spectrum anticonvulsant
• No formal guidelines.
• Patients with sodium channel mutations, avoidance of sodium
channel blockers is wise.
• Avoidance of temperature changes and routine use of fever control
measures may be of some benefit.

Pellock’s Pediatric Epilepsy Diagnosis and therapy. 2017.

Swaimann KF, Ashwal A, Ferriero DM. Swaimann
Pediatric Neurologi. Edisi kelima. 2017.
Case 2

FS or
epilepsy
?

8 yrs, febrile
with high • Diagnosis : FS+
Head CT
?
fever. Hx FS at EEG ? • No indication for EEG and
2 and 3 yrs of imaging, except focal
age seizures
• Exclude CNS infection
• Intermittent prophylaxis
therapy can be considered
Therapy
?
Case 3

Therapy ?
• Diagnosis FS+
• Indication for EEG
EEG/Imagin • No indication for Imaging
Diagnosis ?
g? except focal seizures
• No indication for long-term
AED
7 yrs, first
afebrile
seizures ,
Hx FS at
toddler
First unprovoked seizures
Recurrence risk

• Recurrence risk :
• Overall : 27%-71%
• Without prior seizures : 27%-44%
• Occurring within 2 years : 40-50%
• Recurrence factors :
• Abnormal neurologic status
• Abnormal EEG
• Seizures occurred in wakefulness/sleep
• Seizures type
Pellock’s Pediatric Epilepsy Diagnosis and therapy. 2017.
Hitz D, Berg A, Bettis D. Neurology 2003;60:166-75.
Berg AT. Epilepsia.2008;49:13-8.
Shinnar S, berg AT, O’Dell C. Ann Neurol 2000;48:140-7.
Recurrence risk

• Symptomatic etiology and abnormal EEG findings are the

two most consistent known predictors of seizure recurrence
• Very high risk (>60 or 70%) vs moderately low (<30%) risks.
• Overall, the long-term outcome in terms of complete seizure
control is excellent : 90% of patients becoming completely
seizure-free

Berg TA. Epilepsia.2008;49: 13-8.

Examinations

• Laboratory tests should be based on individual clinical :

vomiting, diarrhea, dehydration, or failure to return to
baseline alertness. (Option)
• Toxicology screening should be considered across the entire
pediatric age range if there is any question of drug
exposure or substance abuse. (Option)
• Lumbal Puncture is of limited value in first non febrile
seizures and should be used primarily when there is concern
about CNS infection. (Option)

Hirtz D, Ashwal S, Berg TA, C. Camfield, P. Camfield.P. Neurology. 2000;55:616-23

Examination
• The EEG is recommended as part of the neurodiagnostic evaluation of
the child with first unprovoked seizure. (Standard)
• If a neuroimaging study is obtained, MRI is the preferred modality.
(Guideline)
• Emergent neuroimaging should be performed in a child of any age who
had postictal focal deficit (Todd’s paresis) not quickly resolving, or
who has not returned to baseline within several hours after the
seizure. (Option)
• Non urgent imaging studies with MRI should be considered in any child
with a significant cognitive or motor impairment of unknown etiology,
unexplained abnormalities on neurologic examination and focal seizures
(Option)

Hirtz D, Ashwal S, Berg TA, C. Camfield, P. Camfield.P. Neurology. 2000;55:616-23

Treat or not

• Treatment following a first unprovoked seizure:

• Reducing recurrence risk
• Does not alter prognosis
• The consequences of long-term drug therapy and its lack of
effect on long-term prognosis following a first seizure :
• No recommendation for treatment following a first unprovoked
seizure in either children or adults.

Pellock’s Pediatric Epilepsy Diagnosis and therapy. 2017.

Haut SR, Shinnar S. Semin in Neurol.2008;28:289-96.
Treat or not

• Therapy should balance risks and benefits.

• Assessment of risk requires :
• Risk of a seizure recurrence/ an adverse event
• The consequences of such an event.
• The acceptability of certain risks.
• The decision should be made jointly by the medical providers and
the patient and family after careful discussion.

Pellock’s Pediatric Epilepsy Diagnosis and therapy. 2017.

Haut SR, Shinnar S. Semin in Neurol.2008;28:289-96.
First unprovoked seizures : Treat or not
• Treatment with AED after a first seizure as opposed to
after a second seizure has not been shown to improve
prognosis for long-term seizure remission
(Class II evidence)
• Treatment has been shown in several studies combining both
children and adults to reduce the risk of seizure recurrence
(Class II evidence).

Hirtz D, Berg AT, Bettis D. Neurology 2003;60;166-175

First unprovoked seizures : Treat or not

• Must be based on a risk–benefit assessment that weighs :

• the risk of another seizure (both the statistical risk and the potential
consequences of a recurrence)
• The risk (cognitive, behavioral, and physical as well as psychosocial) of
chronic AED therapy.
• This decision must be individualized and take into account both
medical issues and patient and family preference.
• Treatment with AED is not indicated for the prevention of the
development of epilepsy (Level B).
• Treatment with AED may be considered where the benefits of
reducing the risk of a second seizure outweigh the risks of
pharmacologic and psychosocial side effects
(Level B).

Hirtz D, Berg AT, Bettis D. Neurology 2003;60;166-175

Case 4

Therapy ?
• First unprovoked seizures
• EEG are indicated
Diagnosis EEG ? • Imaging as indication
• No therapy
• Education and diazepam
rektal
7 yrs, first
unprovoked
seizures ,
No Hx of
seizures
Epilepsy : Pitfalls, tips and tricks
Pitfalls

• Overdiagnosis or uncertainty of diagnosis

• EEG influence the diagnosis
• Polytherapy with minimal dose for each AED
• Increasing the dose of AED without considering the trigger
factors
• Patients and family did not have clear information about
epilepsy
About EEG

53%
42% 40% 40% 34%

Time of EEG examination

About EEG

92%
80%
50%

Sleep-wake examination EEG

Treatment
• The patient had “ true ” epileptic seizures
• Not non epileptic seizures, metabolic abnormality etc
• The patient needs treatment
• Seizures type, frequency, severity of seizures
• No treatment needed if interval between seizures more than 6 months
• If you are in doubt whether epilepsy or not : Wait and see
• AED were depends on type of seizures or syndromes

The epilepsies 2005. h. 59-86

Kesepakatan UKK Neurologi IDAI
Antiepileptic drugs (AED)

• First line AEDs

• Valproic acid 15-40 mg/kgBW/days, in 2-3 divided dose
• Phenobarbital 4-6 mg/kgBW/days, in 2 divided dose
• Carbamazepin 10-30 mg/kgBW/days, in 2-3 divided dose
• Phenytoin 5-7 mg/kgBW/days, in 2-3 divided dose
• Second line AEDs
• Topiramate (Topamax)
• Lamotrigine (Lamictal)
• Levetiracetam (Keppra)
• ACTH, steroids
Choosing AEDS

Focal Focal- Generalize Myoclonik Abcense

generalized d
CBZ Effective Effective Effective Worsen Worsen

VPA Effective Effective Effective Effective Effective

PHT Effective Effective Effective Ineffective Worsen

PHB Effective Effective Effective Effective Worsen

Levetir Effective Effective Effective Effective Effective

TPX Effective Effective Effective Effective Effective

The epilepsies 2005. h. 59-86

Duration of AED

• Generalized tonic-clonic - 2 years seizures free

• Clinically and EEG improved – 2 years seizures free
• EEG still abnormal – 3 years seizures free
• Focal seizures or focal become generalized – 3 years
seizures free
• Absence – 2 years seizures free
• Juvenile Myoclonic Epilepsy – for a life time

UKK Neurologi PP-IDAI 2015

Tips and tricks

• Initial AED needs time to achieve therapeutic level →

seizures still occurred during that period
• Phenobarbital : 2 weeks
• Valproic acid : 1 minggu
• Avoidance trigger factors : AED compliance, irreguler/lack
of sleep or , physical stress, high fever, diarrhea/vomiting
• Adjust the dose of AED according to increased body weight
Epilepsy treatment
Refracter Surgery/others

Polytherapy Seizures
free

Alternative
Seizures Monotherapy
free

First Seizures
Monoterapi free
Paradigm in treatment of epilepsy

Newly diagnosis

47% seizures free

First AED

13% seizure free

Second AED

40%
Refracter
Rasional
Surgery
polytherapy
Brodie MJ and Kwan P, CNS Drugs 2001;15:1-12
Case 5

Diagnosis

Imaging Unprovoked Therapy • EEG and Imaging indicated if

?
seizures, 3
episodes ? focal seizures
• D/ : Epilepsy
• AED treatment

Normal
EEG
Conclusion

• Do not be confused
• Strict to definition
• Anamnesis is the most important thing
• Correct diagnosis
• Correct treatment
Thank you