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
