Pediatric Epilepsy Highlights Flashcards Preview

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Flashcards in Pediatric Epilepsy Highlights Deck (28):
1

Seizures

episodic events which are unexpected
and sudden resulting from abnormal and
excessive activity of neurons
– Involves the electrical functions of the brain

2

Epilepsy

chronic disease of recurrent
unprovoked seizures
– 2 or more unprovoked seizures separated by
greater than 24 hours or 1 seizure with studies
suggesting further risk for seizures

3

intractable epilepsies can be defined as seizures which do not respond to a trial of at least ________ and that approximately 30% of new onset seizure patients may develop intractable epilepsy

2-3 anticonvulsants

drug resistant

She said 2 notes say 3

4

epilepsy is a disorder of recurrent, spontaneous seizures epilepsy can be classified by:

generalized and partial syndromes or by etiologies

5

Generalized: sz involves entire brain

Partial: sz activity originates in ?

one part of the brain

6

Etiology of epilepsy
-Old classes?


• Symptomatic: known etiology
– 30% have structural abnormality: tumor or AVM
– Approximately ½ of patients with symptomatic remit
• Idiopathic: genetic predisposition
• Most idiopathic seizures affect children
• Cryptogenic: thought to have an etiology that is not yet proven


Benign:
– Easily treated with meds
– Normal intelligence
– Most often tests are normal and we don’t know why someone has sz
– Genetic predisposition
– Remission after a certain age (usually teens)

• Catastrophic:
– Intractable to medications
– Affect development with mental retardation and shortens
lifespan of child
– EEG, MRI and other tests are abnormal

trying to move away from these terms

7

New ILAE classification
Changes and proposals

• Replace idiopathic, symptomatic and cryptogenic with more meaning full categories: genetic, structural, metabolic and unknown
• Spasms are now included : epileptic spasms
• Benign and catastrophic are not used as benign have psych issues and catastrophic has a negative undertone.

Old classes of sz:
Generalized: sz involves entire brain

8

Febrile Seizures

: “an event in infancy or childhood, usually occurring between 3 months and 5 years of age, associated with fever but without evidence of intracranial infection or defined cause. Seizure with fever in children who have suffered a previous non-febrile seizure are excluded”

9

Age of Febrile Seizures

• Most common childhood seizure
• Incidence 2-4 % (US)
• Usual age 6 months to 3 years
• Almost all first febrile seizures occur by age 3
• Median age 18-22 months

10

Febrile Seizures stuff

Simple versus Complex
• Simple- tonic clonic
– Generalized
– Less than 10 – 15 minutes
– Do not recur within 24 hours

• Complex
– Focal in nature at onset or during
– Longer than 10 – 15 minutes
– Recur in less than 24 hours
• 20- 30% of febrile seizures are complex

11

timing febrile

Timing of Febrile Seizures
• Usually within first 24 hours of illness
• Not been shown to be rapid rise of fever
• No proof of occurrence with rise in fever
• Many children go on to tolerate higher
fever in the course of the illness

12

Important febrile stuff ***

Future Seizures?
• 25-40% will have a recurrent febrile sz
• ½ with recurrence will have a third
• Risk factors for recurrence
– Less than 1 yr age
– Positive family history
– Low grade fever
– Brief fever

13

Risk factors for recurrence

– Less than 1 yr age
– Positive family history
– Low grade fever
– Brief fever

14

other rf

Other Possible Risk Factors
• Epilepsy in first degree relative
• Complex febrile seizure
• Baseline neurodevelopment abnormalities
• Day care attendance

15

Will this Affect the Child’s
Cognition?

Nearly all children will have normal cognition
– Studies have looked at IQ and academic achievement
• Children with prolonged or complex events may
be at increased risk of cognitive problems, but
most do well
• Tendency to have sleep problems
– Nightmares
– Frequent nocturnal awakenings

16

Lennox Gastaut Syndrome

Generalized Seizures
– Tonic
– Atonic
– Myoclonic
– Atypical absence
– Cognitive dysfunction – not always present, and may evolve later
– Onset typical in early childhood but can happen in adolescent or
adulthood
– Onset between 1-7 years of age
– First seizure typically between 3-5 years of age

17

Lennox Gastaut Syndrome causes

Causes
• Malformations
• Hypoxic-ischemic injury
• Encephalitis
• Meningitis
• Tuberous sclerosis!!!
• Lennox Gastaut Syndrome is proceeded by infantile
spasms in 9-40% of cases.
• 30% of patients with infantile spasms will develop
LGS

18

Non medication treatments

• Ketogenic diet
• Vagal nerve stimulation
• Epilepsy surgery

19

Starvation: produces ketone bodies which have AED
effect and diet continues ketosis
– allows adequate amount of calories and protein to grow
• Unknown mechanism
• Two types: Cream and MCT oil
• Used in children more than adults: compliance
• Useful with all seizures: especially tonic, atonic, atypical
seizures, less for partial seizures
• Efficacy: 7% seizure free, 20% greater than 90%
reduction in seizures, 50% with reduction of more
than 50% in seizure frequency after 1yr (Johns
Hopkins 1997)

Ketogenic diet

Potentially serious or lifethreatening
effects
• Kidney stones 5-8%
• Prolonged QT interval
• Increased bruising with bleeding
• Pancreatitis
• Immunosuppression

20

A pacemaker like device where implanted in the left
chest wall and stimulates a nerve in the neck (vagus
nerve) that stops seizures
• Has a baseline stimulation that the doctor sets but also
has a magnet where it can be swiped over the device
to make it go quicker
– Useful in cases where patient has chronic intractable seizures
and will stop prolonged seizures or clusters of seizures and
prevent use of rectal diazepam
– Also helpful for auras to prevent spread
• Maybe longer efficacy time of 18 months
• Some use currently for depression as well
• Unknown mechanism of action

Vagal nerve stimulation

Side effects of VNS
• May include
– Temporary hoarseness/changes in voice tone
– Cough
– Tickling in the throat
– Shortness of breath
– Surgery: vocal cord paralysis and infection 1%
• Generally occur during the stimulation periods
• Typically decrease over time

21

Epilepsy surgery

Used for partial epilepsies:
– In adults: most temporal lobe and in children:
extratemporal or hemispherectomy
– Not usually used for generalized seizures unless
corpus callosotomy
• Estimated as many as 5000 new patients
annually in the US might benefit from
epilepsy surgery but only 1/3 receive
treatment

22

Types of epilepsy surgery

Focal: lesionectomy, lobectomy,
corticectomy, multiple subpial
transection
• Generalized: corpus callosotomy for drop
• Multilobar or hemispherectomy

23

Epilepsy surgery candidates

Drug resistant epilepsy syndromes where the natural
history and prognosis is terrible:
– Rasmussen’s, Sturge Weber, hemimegalencepahly, focal
cortical dysplasia, hippocampal sclerosis
• If the patients’ development status is regressing
rapidly
– issues to cerebral plasticity in children where more functional
recovery if performed early
• Presence of lesion or single focus where not critical for
function (i.e. motor, language and vision)
• Predominant seizure type and frequency
– failed at least 3 AED: one of which is new

24

Conclusions

Epilepsy is a common disorder noted in the very young
and old
• Classification of epilepsy can be based on semiology or
etiology. There is a newer classification system out
• Medication treatment is based on weighing the risks and
benefits for each individual patient
• Non medication treatments such as keto diet, vagal
nerve stimulation and epilepsy surgery can be used
for children with intractable, catastrophic
epilepsies

25

Ketogenic diet

Ketones vs glucose as energy source
Starvation treats sz-- Ketone bodies produced when starved
Cream or oil
Must eat “right” amount of calories, fat> carbs, proteins
Effective for 7% (vs 3% with meds)
s/e: hunger, hypogly, hangry, ketoacidosis, dehydration due to keto, chronic: constipation/ diarrhea, Zn supplement, kidney stone, prolonged qt

atkins/ low gly can also help

26

Vagal Stim

pace maker in L chest wall
Not often sz free but yes reduction
Magnet swipes can put into emergency mode
s/e: voice change, throat problems

27

Fail 2 meds then what?

se eplisy specialist
can take a long time to do, it shouldn't

28

Epilepsy surgery

Need to see where sz is
Often temporal lobe in adult, kids can do anywhere
Partial sz > generalized (g= corpus colus cut)
Focal or lobectomy, CC cut, or hemispherectomy (half brain)

Needs to be non-responsive to meds or will development bad, lesion non-critical to fx, sz type, fq