Pediatric Epilepsy Highlights Flashcards

1
Q

Seizures

A

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

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2
Q

Epilepsy

A

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

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3
Q

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

A

2-3 anticonvulsants

drug resistant

She said 2 notes say 3

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4
Q

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

A

generalized and partial syndromes or by etiologies

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5
Q

Generalized: sz involves entire brain

Partial: sz activity originates in ?

A

one part of the brain

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6
Q

Etiology of epilepsy

-Old classes?

A

• 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

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7
Q

New ILAE classification

Changes and proposals

A
  • 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

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8
Q

Febrile Seizures

A

: “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”

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9
Q

Age of Febrile Seizures

A
  • 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
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10
Q

Febrile Seizures stuff

A
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
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11
Q

timing febrile

A

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

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12
Q

Important febrile stuff ***

A
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
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13
Q

Risk factors for recurrence

A

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

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14
Q

other rf

A
Other Possible Risk Factors
• Epilepsy in first degree relative
• Complex febrile seizure
• Baseline neurodevelopment abnormalities
• Day care attendance
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15
Q

Will this Affect the Child’s

Cognition?

A

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

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16
Q

Lennox Gastaut Syndrome

A

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
Q

Lennox Gastaut Syndrome causes

A
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
Q

Non medication treatments

A
  • Ketogenic diet
  • Vagal nerve stimulation
  • Epilepsy surgery
19
Q

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)

A

Ketogenic diet

Potentially serious or lifethreatening
effects
• Kidney stones 5-8%
• Prolonged QT interval
• Increased bruising with bleeding
• Pancreatitis
• Immunosuppression
20
Q

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

A

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
Q

Epilepsy surgery

A
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
Q

Types of epilepsy surgery

A
Focal: lesionectomy, lobectomy,
corticectomy, multiple subpial
transection
• Generalized: corpus callosotomy for drop
• Multilobar or hemispherectomy
23
Q

Epilepsy surgery candidates

A

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
Q

Conclusions

A

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
Q

Ketogenic diet

A

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
Q

Vagal Stim

A

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
Q

Fail 2 meds then what?

A

se eplisy specialist

can take a long time to do, it shouldn’t

28
Q

Epilepsy surgery

A

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