11 Epilepsy in Children Steinberg

Question 1

What is the difference in race and seizure disorder?

Answer 1

Whites have highest prevalence

Question 2

What medical comorbidities are more likely to be found in patients with childhood epilepsy?

Answer 2

Depression. Anxiety. ADHD. Conduct problems. Developmental delay. Autism/autism spectrum disorder. HAs. Hearing or vision problems. Asthma. Allergies. Ear infections

Question 3

What is the problem with treating children after their first unprovoked seizure?

Answer 3

You would over treat ~60% of children since they often do not have a recurrence

Question 4

Why type of children have a higher recurrence rate for seizures?

Answer 4

Children with remote symptomatology (e.g. prior CNS infection, vascular insult, etc) or abnormal interictal EEG

Question 5

What is seizure recurrence rate like by EEG results?

Answer 5

Children with a normal EEG had a much lower risk of recurrence. Nonepileptiform EEG had higher recurrence, but Epileptiform EEG had the highest recurrence rates

Question 6

How often does it usually take for a recurrence to occur?

Answer 6

First recurrence usually occurs in the next 6 months

Question 7

What drugs are First-Line for Epilepsy with generalized Tonic-Clonic Seizures?

Answer 7

Oxcarbazepine*, Carbamazepine, Lamotrigine, Sodium Valproate

Question 8

What drugs are First-Line for Childhood and Juvenile Absence Seizures?

Answer 8

Lamotrigine, Ethosuximide. Valproic Acid

Question 9

What drugs are First-Line for Myoclonic Seizures?

Answer 9

Lamotrigine, Levetiracetam, Topiramate*, Valproic acid

Question 10

What drugs are First-Line for Idiopathic Generalized Epilepsy?

Answer 10

Lamotrigine, Topiramate, Valproic Acid

Question 11

What did the SANAD Study Group show in the comparison of AEDs for Partial Epilepsy?

Answer 11

Non-inferiority of LTG compared with CBZ. These two were better than others though

Question 12

For Childhood Epilepsy, what are Benign Epilepsies?

Answer 12

20-30%. Remission occurs after a few years and treatment can often be avoided

Question 13

For Childhood Epilepsy, what are Pharmacosensitive Epilepsies?

Answer 13

30%. Seizure control easily achieved by AEDs, spontaneous remission occurs after a few years (e.g. childhood absence)

Question 14

For Childhood Epilepsy, what are Pharmacodependent Epilepsies?

Answer 14

20%. Drug treatment controls seizure, but no spontaneous remission occurs (e.g. juvenile myclonic epilepsy, symptomatic focal epilepsy); drug withdrawal is followed by relapse and treatment will be lifelong

Question 15

For Childhood Epilepsy, what are Pharmacoresistant (or refractory) Epilepsies?

Answer 15

13-17%. Poor prognosis; resistance to drugs can usually be predicted early after an inadequate response to initial appropriate treatment

Question 16

What happens if a patient fails remission and goes into relapse?

Answer 16

Doesn’t mean its over, patients can often go back into remission

Question 17

What factors cause a child to be associated with decreased risk of relapse?

Answer 17

Log initial seizure frequency. Idiopathic partial epilepsy. Onset < 1 year old. Never taken AEDs

Question 18

What factors cause a child to be associated with increased risk of relapse?

Answer 18

Juvenile myoclonic epilepsy. Focal slowing on initial EEG

Question 19

Why do Topiramate concentrations in pediatrics differ?

Answer 19

Higher clearance in younger pediatric patients, concentration increases with age. Need higher doses in younger children to get the same % Fraction Seizure Free

Question 20

What are the characteristics of “Typical” Absence Seizure?

Answer 20

ABRUPT onset in childhood. Totaly lost consciousness. Short duration of seizure (usually < 10 sec), numerous and frequently in clusters. Caused by Idiopathic generalized epilepsy. Usually normal interictal EEG appearance

Question 21

What are the characteristics of “Atypical” Absence Seizure?

Answer 21

Often gradual onset of any age. Only partially impaired consciousness. Long duration (several minutes) and less frequent. Confusion, HA, emotional disturbance are common. Usually these patients have learning difficulties. Abnormal interictal EEG appearance

Question 22

When comparing Ethosuximide, Valproic Acid, and Lamotrigine on a graph, what happens to their Freedom from Treatment Failure?

Answer 22

They’re all relatively the same, but after ~16 weeks, Lamotrigine rapidly drops. Lamotrigine has a high lack of seizure control compared to the others (efficacy of LTG is the problem, not the ADRs or other drug profiles)

Question 23

What is Lennox-Gastaut Syndrome (LGS)?

Answer 23

One of the Epileptic encephalopathies (conditions where epileptiform abnormalities themselves contribute to a progressive disturbance in cerebral function)

Question 24

What is LGS characterized by?

Answer 24

1) Multiple generalized seizure types, typically tonic, atonic and myoclonic, and atypical absence. 2) An interictal EEG pattern characterized by diffuse slow spike-and-wave complexes during sleep and wakefullness. 3) Cognitive dysfunction; develops in most but not all patients

Question 25

What does LGS evolve from?

Answer 25

West Syndrome (infantile spasms) in ~20-30%of children; associated with a very poor prognosis

Question 26

What is 1st line treatment for LGS?

Answer 26

Valproic Acid

Question 27

What are the Electroencephalogram Features of LGS?

Answer 27

Slow spike-wave complexes; diffuse or bilateral fast rhythm patterns, or polyspikes

Question 28

Which medication for LGS has > 75% decrease in LGS?

Answer 28

Rufinamide. Superior to both Topiramate and Lamotrigine

Question 29

What is Clobazam?

Answer 29

1,5-BZP. Rapid and complete absorption, 80-90% albumin-bound; active metabolite is N-desmethylclobazam via CYP 3A4 then 2C19 to 4’-hydroxydesmethylclobazam; polymorphisms in 2C19 increase ratio of Ndm-CBM to CBM ratio; steady-state t1/2 CMB = 32 hrs, 57 hrs for Ndm-CBM

Question 30

What is Clobazam like in LGS?

Answer 30

After 1 month on CLB, 32% of patients became seizure-free and 20% had > 50% seizure reduction

Question 31

What is Intractable Epilepsy in Children?

Answer 31

Most suggest that at least 2 anti-convulsants have been tried for an adequate period and were not unsuccessful solely because of adverse effects. Need referral to an epilepsy center

Question 32

What are Febrile Seizures?

Answer 32

Most common seizure of young children. Can be extremely frightening and emotionally traumatic for parents to witness

Question 33

What is the ILAE Definition of “Febrile Seizure”?

Answer 33

A seizure occurring in childhood after one month of age, associated with a febrile illness not caused by an infection of the CNS, without previous neonatal seizures or a previous unprovoked seizure, and not meeting criteria for other acute symptomatic seizures. Can be simple or complex

Question 34

What is a Simple Febrile Seizure?

Answer 34

Generalized tonic-clonic seizures of brief duration (< 15 minutes, 95% < 5 minutes), ONCE in 24 hours)

Question 35

What is the risk of Febrile Seizures and Epilepsy after vaccination?

Answer 35

Whole-cell pertussis vaccine carries increased risk of febrile seizures. Vaccination with DTaP-IPV-Hib (Acellular pertussis vaccine) was not associated with an increased risk of epilepsy

Question 36

What are Complex Febrile Seizures?

Answer 36

Prolonged (> 10-15 minutes). Multiple (recurrent w/in the same febrile illness over a 24-hours period). Focal onset

Question 37

What is Febrile Seizure Reoccurrence like?

Answer 37

30-35% overall and increases to 50% if the initial febrile seizure occurs in a child < 1 yo

Question 38

What are some predictors of recurrent febrile seizures?

Answer 38

Influenza A viral infection. Onset of febrile seizure < 15 months of age. Temp < 40 (104) at time of first febrile seizure. < 1 hours between onset of fever and 1st febrile seizure

Question 39

If a patient has a febrile seizure, what should be done if there are signs of serious illness (lethargy beyond postictal state, unstable clinical status, petechiae, nuchal rigidity, irritability, bulging fontanelle)?

Answer 39

Admit, treat as suspected meningitis

Question 40

What should be done for complex febrile seizures or suspected antibiotic masking of meningeal symptoms?

Answer 40

Admit. Investigate source of fever and treat accordingly. Strongly consider lumbar puncture if age < 12 months. Always lumbar puncture if suspected meningitis, unless contraindications (e.g. raised intracranial pressure). Observation > 2 hours. Consider neuroimaging

Question 41

What is the treatment/prevention for Febrile Seizures?

Answer 41

Antipyretics no better than placebo if patient has 1 or more risk factor. Combo of Antipyretics and Low-Dose Diazepam not preventative. Valproic acid and Phenobarbital at low doses have prevented recurrent FS by 80-90%. Intranasal or buccal Midazolam appropriate for prolonged FS

Question 42

What is Prehospital pharmacotherapy for Status Epilepticus?

Answer 42

Benzodiazepines in various dosage forms

Question 43

What is Early pharmacotherapy for Status Epilepticus?

Answer 43

Lorazepam (slow but longer lasting). Midazolam, Diazepam + Phenytoin

Question 44

What is Additional pharmacotherapy for Status Epilepticus?

Answer 44

Phenobarbital, Valproic Acid, Levetiracetam

Question 45

What is Refractory pharmacotherapy for Status Epilepticus?

Answer 45

CI-Midazolam. Phenobarbital/Thiopental, Propofol

Question 46

What is the common drug used for status epilepticus?

Answer 46

Benzodiazepine, if that fails then 2nd line anticonvulsant, if that fails then RSI