Epilepsy

Question 1

spell

Answer 1

non-specific term for a sudden paroxysmal event

Question 2

seizure

Answer 2

manifestation of abnormal excitation and synchronization of a group of cortical neurons

Question 3

epilepsy

Answer 3

a diagnosis of two or more unprovoked seizures separated by greater than 24 hours or one seizure with studies (EEG/MRI) suggesting further risk of seizures.

Question 4

syndrome

Answer 4

a constellation of common clinical pathological and electrographic features (lennox-gastaut syndrome)

Question 5

provoked seizure

Answer 5

a seizure triggered by a brain injury or insult that would have reasonably resulted in a seizure in any person and if removed or avoided would likely lead to cessation of seizures (alcohol, TBI, hypoglycemia, febrile seizures)

Question 6

types of seizures

Answer 6

focal onset
generalized onset
unknown onset

Question 7

most common area of seizures

Answer 7

temporal or frontal lobes

Question 8

types of epilepsy

Answer 8

generalized
focal
combines
unknown

Question 9

pathology of seizures

Answer 9

1) too much excitation - ionic (inward Na and Ca currents) and neurotransmitters (glutamate and aspartate)
2) too little inhibition - ionic (inward Cl and outward K currents) and neurotransmitters (GABA)

Question 10

B6

Answer 10

cofactor that converts glutamate to GABA (from excitation to inhibition)

Question 11

complications of generalized convulsive status epilepticus

Answer 11

rhabdomyolysis
hyperthermia
orthopaedic
aspiration pneumonia
neuronal injury (cerebral edema) 
cardiac/Resp. arrest
hepatic failure
renal failure
hypotension
death

Question 12

evolution of seizures

Answer 12

!) precipitating injury or initial epileptogenic trigger

2) latent period for months to years
3) emergence of chronic epilepsy/spontaneous recurrent seizures

Question 13

non-epileptic spells

Answer 13

also called psychogenic seizures/pseudoseizures
a form of conversion disorder
1/3 have identifiable risk factors (depression, anxiety, PTSD, abuse history, combat)
can coexist with epilepsy (mixed disorder)
treat with CBT

Question 14

seizure recurrence rates

Answer 14

about 50% with new onset seizures will have recurrence

Question 15

factors for treating seizures

Answer 15

focal neuro exam
lesion on imaging
unprovoked
abnormal EEG
focal seizure
long seizure

Question 16

factors against treating seizures

Answer 16

normal neuro exam
normal imaging
provoked
normal EEG
generalized seizure
short seizure

Question 17

AEDs for generalized/partial seizures

Answer 17

ethosuximide (absence only) 
valproate
lamotrigine
topiramate
levetiracetam
felbamate
rufinamide (gen. only) 
clobazam (gen. only)

Question 18

AEDs for partial-only seizures

Answer 18

phenobarbital
phenytoin
carbamazepine
oxcarbazepine
gabapentin 
tiagabine
zonisamide
pregabalin
lacosamide
ezogabine

Question 19

special populations with AEDs

Answer 19

elderly: reduce doses, avoid certain meds
pregnancy: close monitoring, dose changes, concern about birth defects
children: liquid formulations, reduced doses, certain meds approved

Question 20

AEDs without interactions

Answer 20

gabapentin, pregabalin, levetiracetam

Question 21

risks with surgical treatment of seizures

Answer 21

memory loss, language problems, weakness, incoordination, vision problems, common surgical risks

Question 22

vagal nerve stimulation for seizures

Answer 22

palliative, seizure freedom is rare, 50% have 30-70% reduction of symptoms
most common side effect is hoarseness

Question 23

responsive neurostimulation for seizures

Answer 23

neurostimulator implanted in the skull at sites of seizure onset, records and responds to seizures by delivering electrical impulses

Question 24

DBS for seizures

Answer 24

DBS for bilateral ant. nuclei of the thalamus, median seizure reduction 69% at 5 years, improved QOL

Question 25

factors that promote seizures

Answer 25

missing meds, sleep deprivation, alcohol use, recreational drug use, infections, menstration

Question 26

seizures and driving restriction

Answer 26

varies by state from no restriction to a while year restriction in NY and VT, mandatory reporting in western coast states

Question 27

treating drug resistant epilepsy

Answer 27

50% are seizure free with 1st drug 12% are seizure free with 2nd drug 4% are seizure free with 3rd drug 34% are drug resistant (pharmaco-resistant epilepsy) *only do surgery if they fail at least 2 different treatments that are appropriately selected

Question 28

absence seizures

Answer 28

characteristic 3Hz spike and wave discharge on EEG

Question 29

febrile seizures

Answer 29

seizure in infancy or childhood associated with fever but without evidence of intracranial infection or defined cause. Most common childhood seizure, usually presents 6months - 3 years. *seizure with fever in kids who have had previous non-febrile seizures are excluded

Question 30

simple febrile seizures

Answer 30

generalized (both sides) less than 10-15 minutes don't reoccur within 24 hours

Question 31

complex febrile seizures

Answer 31

focal at onset or during longer than 10-15 minutes recur in less than 24 hours *20-30%

Question 32

timing of febrile seizures

Answer 32

usually within first 24 hours of illness not due to rapid rise in fever, preventing fever with tylenol wont stop them can begin to tolerate a higher fever over the course of the illness

Question 33

recurrence with febrile fever

Answer 33

25-40% recur | 50% with one recurrence will have a third

Question 34

risk factors for recurrence of febrile fevers

Answer 34

``` less than 1 y/o positive family history low grade fever brief fever complex febrile fever baseline neurodevelopmental abnormality day care attendance (more frequent illnesses) ```

Question 35

development of epilepsy after febrile fevers

Answer 35

increased risk with complex febrile seizures, neurologically abnormal prior to seizures, family history of afebrile fevers treating with AEDs doesn't prevent later epilepsy

Question 36

lennox-gastaut syndrome

Answer 36

multiple seizure types (tonic - often nocturnal - atonic, myoclonic, atypical absence - slower 2Hz spike and wave - focal cognitive dysfunction can evolve later but not always onset usually 1-7y/o with first seizure 3-5y/o but can happen into adulthood *have to diagnose over time not at the first seizure

Question 37

causes of lennox-gastaut syndrome

Answer 37

cerebral malformations, hypoxic-ischemic injury, encephalitis, meningitis, tuberous sclerosis, preceeded by infantile spasm (9-40%)

Question 38

how long must you be on AEDs before considering weaning off

Answer 38

2 years seizure free | consider EEG prior to weaning

Question 39

medications that can worsen some seizures

Answer 39

lamotrigine: can worsen myoclonic seizures | phenytoin, carbamazepine, oxcarbazepine, and gabapentin can worsen generalized seizures

Question 40

important side effects of AEDs

Answer 40

avoid valproic acid in teenagers b/c polycystic kidney disease and fetal issues no valproic acid for children under 2y/o b/c fatal liver toxicity avoid topiramate or zonisamide with acidosis in kids

Question 41

ketogenic diet for drug resistant epilepsy

Answer 41

high fat, low carbs, protein control useful for kids more than adults useful for all seizure types, but less so for partial seizures 7% seizure free 20% have at least 90% seizure reduction 50% have at least 50% seizure reduction *b/c limited efficacy only use after meds fail

Question 42

adverse effects of ketogenic diets

Answer 42

hunger, hypoglycemia, drowsy, lethargy, irritability, nausea, vomiting (because of low blood sugar and ketone buildup), dehydration, refusal to eat, excessive ketosis (acidosis)

Question 43

chronic effects of ketogenic diets

Answer 43

refusal to eat, hyperlipidemia, low free and total carnitine, constipation or diarrhea, Zn deficiency (hair thinning and loss), acidosis, hyperuricemia, hypocalcemia, suboptimal growth, kidney stones, prolonged QT interval, increased bruising and bleeding, pancreatitis, immunosuppression