Pediatric Epilepsy Highlights Flashcards
Seizures
episodic events which are unexpected
and sudden resulting from abnormal and
excessive activity of neurons
– Involves the electrical functions of the brain
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
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
epilepsy is a disorder of recurrent, spontaneous seizures epilepsy can be classified by:
generalized and partial syndromes or by etiologies
Generalized: sz involves entire brain
Partial: sz activity originates in ?
one part of the brain
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
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
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”
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
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
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
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
Risk factors for recurrence
– Less than 1 yr age
– Positive family history
– Low grade fever
– Brief fever
other rf
Other Possible Risk Factors • Epilepsy in first degree relative • Complex febrile seizure • Baseline neurodevelopment abnormalities • Day care attendance
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
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
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
Non medication treatments
- Ketogenic diet
- Vagal nerve stimulation
- Epilepsy surgery
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
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
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
Types of epilepsy surgery
Focal: lesionectomy, lobectomy, corticectomy, multiple subpial transection • Generalized: corpus callosotomy for drop • Multilobar or hemispherectomy
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
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
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
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
Fail 2 meds then what?
se eplisy specialist
can take a long time to do, it shouldn’t
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
