A presentation on epilepsy and other different types of seizure disorders in children.

1. EPILEPSY & SEIZURE DISORDERS
IN CHILDREN
Prepared by; Joyce Mwatonoka
MD5, CHAS-UDOM
August 2017

2. OUTLINE
• Definition of terms
• Seizures and Epilepsy
• Status epilepticus and its management
• Febrile seizures

3. Definition of terms
• Tonic; increased tone or rigidity
• Atonic; lack of movement or flaccidity
• Clonic; rhythmic muscle contraction and
relaxation
• Myoclonic; brief jerks/twitches of a muscle or
group of muscles
• Aura; perceptual disturbance before the onset of
a seizure
• Automatisms; coordinated involuntary
movements such as chewing, lip smacking

4. Cont…
• Postictal state; events immediately after the
seizure, often include sleep, headache,
confusion, hemiparesis
• Provoked seizure ; caused by an underlying
disease or event (e.g., head injury, stroke,
central nervous system infection or tumor,
intracranial surgery, exposure to drugs or
toxins, drug or alcohol withdrawal, fever)

5. Seizures and Epilepsy
• A seizure or convulsion; is a paroxysmal,
transient change in motor activity and/or
behavior that results from abnormal electrical
activity in the brain
• Most seizures in children are provoked by
somatic disorders originating outside the brain,
such as high fever, infection, head trauma,
hypoxia, toxins, or cardiac arrhythmias
• Epilepsy is defined as recurrent, unprovoked
seizures

6. Epidemiology
• Seizures are common in the pediatric age
group and occur in up to 10% of children
• Less than one third of seizures in children are
caused by epilepsy
• The incidence of childhood epilepsy is 1% to
2%

7. Aetiology
• Idiopathic >60%
• Genetics; Epilepsy due to a single gene defect
is rare, more often an interaction of multiple
genes and environmental factors is
responsible. Eg; genes encoding for GABA
receptors (major components of inhibitory
circuit) and ion channels

8. Cont…
Acquired
• Injury (head trauma)
• Brain tumor (SOL)
• Stroke (leading cause un adults >35 years)
• Previous infection
• Birth defect eg; abnormal brain structure
• Autoimmune disorders

9. Commonest causes of convulsions in
neonates
• HIE
• CNS infection
• Hypoglycemia (Rx; 2ml/kg of 10% glucose)
• Hypocalcaemia (Rx; 2ml/kg of 10% calcium
gluconate)
Treat convulsions with phenobarbotal (loading
dose 20mg/kg IV), if they persist add 10mg/kg
up to a maximum dose of 40mg/kg

10. Pathophysiology
• A balance between excitation and inhibition is
essential for a normal brain function
• A seizure results when a sudden imbalance
occurs between the excitatory and inhibitory
forces within the network of cortical neurons
in favor of a sudden-onset net excitation
(synchronous firing of many neurons at once)
• Symptoms and type of seizure will depend on
affected area of the cortical network

11. Why are seizures more common in
children?
• Because their excitatory post-synaptic
potentials develop more quickly than the
inhibitory, therefore their brains are much
more excited than inhibited

12. Classification
Epileptic seizures are generally classified as focal
(or partial) or generalized seizures
1. Generalized Seizures
• Originate within and propagate throughout both
hemispheres
• Include loss of consciousness (LOC)
• May include tonic-clonic, atonia or myoclonic
jerking
• Widespread cellular, biochemical or structural
abnormalities

13. Cont…
• Primary generalized seizure; starts from the
both hemispheres at the same time (starts on
both sides)
• Secondary generalized seizure; starts from
one hemisphere then moves on to the other
hemisphere (ends up on both sides)

14. Grand mal (tonic-clonic) seizures
• Typically the seizure begins abruptly,
consciousness and control of posture are lost,
followed by tonic stiffening and upward deviation
of the eyes
• Pooling of secretions, pupillary dilation,
diaphoresis, and hypertension are common
• Clonic jerks follow the tonic phase
• In the post-ictal phase, the child might be
hypotonic. Irritability and headache are common
as the child awakens

15. Petit mal (absent) seizures
• The clinical hallmark is a brief (< 15 seconds) loss
of environmental awareness, which may be
accompanied by automatism eg; eye fluttering,
fumbling with the fingers, lip smacking
• Can be caused by a complex partial seizure
• They usually begin between 4-6 years
• Neurologic examination and brain imaging are
normal. The characteristic EEG patterns consist of
generalized 3-Hz spike-and-wave activity
• Can be provoked by hyperventilation or strobe
light stimulation

16. Atypical Absence, Myoclonic, and
Atonic Seizures
• Atypical absence seizures; episodes of impaired
consciousness with automatisms, autonomic
phenomena, and motor manifestations. Slower
EEG discharges (2 Hz)
• Myoclonic epilepsy; usually is associated with
multiple seizure types not all myoclonus are
epileptic in nature
• Atonic seizures; typically brief (lasting 1 to 2 sec),
are quite disabling because of a sudden loss of
postural tone, resulting in falls and injuries.

17. 2. Focal/Partial
• Originate within and propagate throughout one
hemisphere
• May have motor (tonic, clonic, myoclonic),
sensory, cognitive, emotional, psychic, or
autonomic abnormalities
• Usually associated with local structural
abnormalities
• Partial seizures are common in temporal lobe
epilepsy
• Focal may progress to generalized with LOC

18. Cont…
• Symptoms will vary according to where the
seizure occurs
• In the frontal lobe; speech, twisting, turning,
pedaling
• In the temporal lobe, a feeling of déjà vu
• In the parietal lobe, a numbness or tingling
• And in the occipital lobe, visual disturbance or
hallucinations

19. Cont…
a) Simple partial seizure;
• A partial seizures without dyscognitive features
• Usually the event is remembered in detail
b) Complex partial seizure;
• With dyscognitive features
• There is loss or changes in consciousness,
awareness and responsiveness
• Patient may have staring and automatism
• Typically frontal/temporal lobe onset

20. Cont…
• When focal seizures spread to involve the
whole brain and produce a generalized
seizure, they are said to have secondarily
generalized
• Such spread is classically described as
progression from face to arm to leg
(Jacksonian march)

21. Questions that help clarify the type of
seizure
• Was any warning noted before the spell? (aura)
• What did the patient do during the spell?
• Was the patient able to relate to the environment
during the spell and/or does the patient have
recollection of the spell?
•Was there urinary or fecal incontinence?
•How long did it last?
•How was the child behaving after the seizure/time
(Postictal)?

22. Epilepsy syndromes
• Benign childhood epilepsy with centrotemporal
spikes / Benign Rolandic epilepsy
• Childhood absence epilepsy
• Juvenile myoclonic epilepsy (of Janz)
• Infantile spasms (West’s syndrome)
• Lennox-Gastaut syndrome
• Benign neonatal convulsions
• Acquired epileptic aphasia (Landau-Kleffner
syndrome)

23. Lennox-Gastaut syndrome/Childhood
epileptic encephalopathy
• Is a severe epilepsy syndrome characterized by
frequent, multiple seizure types, a resistant to
medications, abnormal EEG findings and
moderate-severe intellectual disability
• Most children present before age 5 years
• Many children have underlying brain injury or
malformations

24. Dravet syndrome
• A rare, lifelong, genetic epileptic encephalopathy
• Non hereditary gene mutations in ion channels
• Presents in infancy (<1 year) with polymorphic
seizures, some degree of developmental disability
• First seizure usually associated with fever, then
occur without fever but can be triggered by slight
changes in body temperature, infections, flashing
light

25. SUDEP (sudden unexplained death in
epilepsy)
• High risk; people with difficult to treat
epilepsy, who have tonic clonic seizures, who
are on multiple seizure medications and who
have developmental delays
• Other causes of mortality ass/c Dravet
Syndrome include status epilepticus and
accidental death from injury or drawning

26. Ddx for Seizures
• Fever
• Meningitis/Encephalitis/Brai
n Abscess
• Sepsis
• Tetanus
• Birth Asphyxia
• Head Trauma (Including
Child Abuse)
• Metabolic (hyponatremia,
hyperbilirubinemia, DKA,
hypoxia)
• Toxins/Ingestions
• Drugs/Medications or
withdrawal from
drugs/alcohol
• Stoke/TIA
• Syncope/Arrhythmia
• Brain Tumor/Mass
• Psychogenic
• Night Terrors/Sleepwalking
• Migraine
• NAS

27. Physical examination
• Vital Signs
-blood pressure (HTN),
temperature (fever)
• Mental State/Alertness
-often lethargic/sleeping
immediately after seizure, h/o
psychiatric disease
• Eye
-retinal hemorrhages (trauma),
papilledema (increased ICP)
• Mouth
-tongue lacerations
• Neck
-stiffness (meningitis), carotid
bruit (stroke)
• Cardiovascular
-rhythm (syncope), murmur
(stroke/emboli)
• Neurological
-Cranial nerve deficits,
hemiparesis/paralysis (focal
deficit-stroke, mass), pronator
drift, deep tendon reflexes, gait
and coordination (frontal/motor)
• Skin

28. Investigations; Labs
• Prolactin levels; shortly after a seizure to assess
the etiology (epileptic vs nonepileptic); typically
elevated 3-4 fold, more likely to occur with
generalized tonic-clonic seizures; however, the
considerable variability of prolactin levels has
precluded their routine clinical use
• LP (especially <1 yrs) in pts with obtundation
(persistent or was present prior to the onset of a
spell) or in pts in whom meningitis or encephalitis
is suspected
• Serum levels of anticonvulsant agents to
determine baseline levels, potential toxicity, lack
of efficacy, treatment noncompliance

29. Other labs to R/O other seizure
etiologies
• BS for MPs
• FBP
• Urinalysis
• GLUCOSE (hypoglycemia)
• RFT; Electrolytes (hyponatremia, hypocalcemia),
BUN, Cr
• HIV
• Consider drug/alcohol screen
• Consider AST/ALT, Bilirubin (Hepatic Function)
• Consider ECG (arrhythmias)

30. Imaging studies
1. EEG;
•Should be performed (if possible) within 1-2
days on all patients with seizure of unknown
etiology/unprovoked
•May be able to diagnose seizure disorder
•May be able to predict future seizure/epilepsy
•Up to 60% of pediatric cases of new-onset
seizure will have epileptiform abnormalities

31. Cont…
•Often done with hyperventilation/light
stimulation to induce seizure activity
•May also perform sleep deprived EEG if initial
EEG is normal within 3 days for higher yield of
abnormalities
2. MRI/CT scan

33. Management
• Medications target sodium channels and GABA
receptors
• A ketogenic diet (high fat low carbohydrate) has been
shown to reduce or prevent seizures in many children
whose seizures could not be controlled by medications.
Reason unclear.
• Nerve stimulation therapies (eg; vagus ns). The
stimulating device acts as a pacemaker for the brain
• Surgery to remove part of the brain that causes seizure
(if it’s small and does not have any vital function)

34. Cont…
• Goal of treatment is to achieve a seizure-free
status without adverse effects
• Monotherapy is important, because it
decreases the likelihood of adverse effects and
avoids drug interaction
• Start anticonvulsants if;
a) The child had >1 unprovoked seizures OR
b) An abnormal sleep-deprived EEG

35. Meds; type dependent

36. Side effects of antiepileptics
• Phenytoin; PGS, hypotension, arrhythmias,
poor coordination, nystagmus, TEN peripheral
neuropathy, status epilepticus
• Phenobarbotone; sedation
• Valproic acid; thrombocytopenia,
encephalopathy, hypothermia
• Hepatotoxicity

37. STATUS EPILEPTICUS
• Is a neurologic emergency and is defined as
ongoing seizure activity or repetitive seizures
without return of consciousness for greater
than 30 minutes
• 14% risk of new neurologic deficits
• Mortality rate of status epilepticus (4% to 5%)
is related to the underlying etiology

38. Etiology
• New-onset epilepsy of any type
• Drug intoxication or drug withdrawal (especially
missed anticonvulsant doses among children with
preexisting epilepsy)
• Acute head trauma
• Infection
• Ischemic stroke, intracranial hemorrhage
• Metabolic disorders eg; hypoglycemia, electrolyte
imbalance
• Hypoxia

39. Management; stabilization
• The first priority of treatment is to ensure an
adequate airway, breathing, and circulation
• Vital signs should be obtained and oxygen
administered if needed
• If respirations are inadequate, positive-
pressure ventilation may be required
• IV access should be obtained

40. Cont…
• In patients with no history of seizures, laboratory
evaluation should be undertaken (glucose, basic
metabolic panel, toxicology, anticonvulsants
levels,CBC)
Pharmacologic management (several)
• Initial management is usually with a
benzodiazepine. Lorazepam (0.05-0.1 mg/kg),
diazepam (0.2-0.3mg/kg IV, 0.5 mg/kg rectal),
and midazolam (0.1-0.2 mg/kg) all are effective
agents

41. Cont…
• If the seizure does not resolve after two (10min
apart) doses of benzodiazepine, a second-line
agent must be administered
• Either IV phenytoin (15-18mg/kg)-over 60min or
fosphenytoin (10-20mg/kg) is effective, but
cardiac monitoring is required to evaluate for
arrhythmia
• If the seizures persist, a loading dose of
phenobarbital (15mg/kg)-over 15min or valproic
acid (20mg/kg) is appropriate

42. Cont…
• Alternatively, continuous infusions, such as
midazolam or pentobarbital, can be employed as
third-line agents (with appropriate airway
protection, BP support, and strong consideration
of continuous video-EEG monitoring to evaluate
for ongoing subclinical seizures)
• If this approach is ineffective, GA
• When status epilepticus stops, maintenance
therapy is initiated with the appropriate
anticonvulsant

43. FEBRILE SEIZURES
• Between 6 months and 6 years with temp >38o C
not due to other causes (CNS infection, metabolic
abnormality, etc)
• Seizures in the setting of fever may be caused by
CNS infections (meningitis, encephalitis, brain
abscess), unrecognized epilepsy triggered by
fever, or febrile seizures
• The most common cause of seizures among
children between 6 months and 6 years of age,
occurring in about 4% of all children

44. Simple febrile seizures;
• Are generalized at onset,
• Last less than 15 minutes,
• And occur only once in a 24-hour period in a
neurologically and developmentally normal
child

45. Complex/atypical febrile seizures;
• If there are focal features,
• The seizure lasts longer than 15 minutes or
• Recurs within 24 hours, or if
• The child has preexisting neurologic
challenges

46. Cont…
• The prognosis of children with simple febrile
convulsions is excellent
• Intellectual achievements are normal
• Febrile seizures recur in 30% to 50% of
children
• The risk of subsequent epilepsy is not
substantially greater than that for the general
population (approximately 2%)

47. Factors that increase the risk for the
development of epilepsy
• Abnormal neurologic examination or
development
• Family history of epilepsy
• Complex febrile seizures
-Patients with 2 risk factors have up to 10%
chance of developing afebrile seizures
-Increased mortality; seizures before 1yr,
triggered by temp <39 C

48. Management of febrile seizures
• Most children require no treatment because they
are brief and benign
• Rectal diazepam can be administered during a
seizure to abort a prolonged, or for children with
a history of prolonged febrile seizures
• Undress the child, PCM/Ibuprofen after seizure
• Because of the potential for side effects, daily
administration of anticonvulsants is not
recommended
• Administration of antipyretics during febrile
illnesses does not prevent febrile seizures

49. References;
• Nelson Essentials of Pediatrics 7th Edition
• Nelson Text Book of Pediatrics 18th Edition
• JESSICA A. WILDEN, MD, and AARON A.
COHEN-GADOL, MD, Indiana University School
of Medicine, Indianapolis, Indiana Am Fam
Physician. 2012 Aug 15;86(4):334-340.
• WHO Guidelines for the management of
common childhood illnesses 2nd edition 2013

50. THANK YOU FOR YOUR ATTENTION