Seizures Pediatrics-Schwartz Flashcards

1
Q

What is a seizure?

A

a phenomenon of heightened neuronal excitability and depolarization which spreads to neighboring neurons, columns, gyri, lobes to produce a loss of function or not

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2
Q

What is epilepsy?

A

***At least 2 unprovoked (or reflex) seizures occurring more than 24 hours apart.

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3
Q

T/F Epilepsy involves…One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrency risk after 2 unprovoked seizures (at least 60%), occurring over the next 10 years.

A

**True.

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4
Q

What is a semiology? Which seizure types have unique semiologies in pediatrics?

A

**progression of clinical signs that occur during the course of a seizure
neonatal seizures & febrile seizures

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5
Q

What is the prevalence of epilepsy in children?

A

Incidence 0.2% at birth. Drops to 0.1% age three and stabilizes at .05%. (USA)
Prevalence 0.7%
Life time incidence of epilepsy - 1/26.

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6
Q

When do neonatal seizures occur?

A

Occur up to 28 days post term but usually in first week of life

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7
Q

How can you differentiate seizures from normal movements in a neonate?

A

*****You can differentiate the motor phenomena of seizures from release phenomenon by whether the movement(s) is induced by stimulation (noise, tactile, passive movement) or stopped by repositioning.

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8
Q

What is a focal clonic epileptic seizure?

A

Repetitive rhythmic jerking of a limb, face or trunk

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9
Q

What is a focal tonic epileptic seizure?

A

Sustained posturing of a limb, eye deviation, assymetric trunk

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10
Q

T/F Generalized tonic “seizures” are non-epileptic.

A

**True.

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11
Q

What does a generalized tonic non seizure look like? What is it commonly due to?

A

*****sustained, symmetric posturing – non-epileptic
Limbs, neck, trunk.
Flexor extensor or mixed
Commonly due to GERD

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12
Q

Are myoclonic seizures epileptic? Spasms epileptic? Motor automatisms?

A

Myoclonic: epileptic or not
Spasms-epileptic
Motor Autospasms–non-epileptic, eye movements, oral movements

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13
Q

What are some causes of neonatal seizures?

A
***hypoxia
infection
intracranial hemorrhage
congenital CNS abnormalities
*****electrolyte disturbance-calcium, sodium, glucose, magnesium
metabolism problems
toxins
genetic issues
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14
Q

What is the treatment for neonatal epilepsy?

A

treat the cause–hypocalcemia, hypomagnesemia, hypoglycemia, hyponatremia, pyridoxine
Treat w/ AED

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15
Q

When do neonates not need AED?

A

Brief and infrequent focal motor, focal tonic or myoclonic seizures

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16
Q

When should you withdraw the AED treatment for a neonate?

A

2 weeks after the last seizure if the EEG is negative.

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17
Q

What are the medications used to treat epilepsy?

A

Levetiracetam
Phenobarbital
Phenytoin
If still seizing: ativan or midazolam + high dose PB or DPH

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18
Q

What is an example of a newer AED?

A

lacosamide

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19
Q

What are febrile seizures? Which age group do they usu affect?

A

common seizure of childhood

3mo-5yo

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20
Q

What percentage of febrile seizures are benign?

A

***85% are benign.
simple ones last less than 10 minutes
generalized & don’t recur w/i 24 hours

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21
Q

What is considered a complex febrile seizure? Is this benign?

A

longer than 10 min & recur in 24 hours…

not benign.

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22
Q

How should you evaluate febrile seizures?

A
  • ***lumbar puncture–done in children less than 1 yo

* *should be done in every child w/ a first complex febrile seizure or w/ meningeal signs

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23
Q

How do you treat prolonged febrile seizures? Recurrent seizures?

A

Febrile: benzodiazepine, ativan, midazolam
Recurrent: Diastat

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24
Q

What are the risk factors for febrile seizures?

A
Age (6 mo – 3 yrs)
Degree of temperature elevation
FS in 1st or 2nd degree relatives
Family hx of afebrile seziures
Slow development of child
Maternal smoking and EtOH during pregnancy
Day care attendance
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25
Q

What are the risk factors for recurrent febrile seizures?

A

First at <1 yr old Abnormal neonatal hx
Family hx of FS Family Hx of retardation
FS following low grade or brief fever
Epilepsy in 1st degree relative
Complex febrile seizures
Neurodevelopmental abnormalities
Attendance at day care

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26
Q

What are the risk factors for later epilepsy if someone has febrile seizures?

A

*****abnormal neonatal hx

family hx of retardation

27
Q

What are epileptic encephalopathies?

A

**Syndromes of recurrent seizures associated with regression of developmental milestones

28
Q

What are some examples of epileptic encephalopathies?

A
Ohtahara Syndrome
Dravet Syndrome
West Syndrome
Lennox Gastaut Syndrome
Landau Klefner
ESES
29
Q

Explain ohtahara’s Syndrome or EIEE.

A

early…10days-3 months
***EEG characterized by suppression-burst pattern
Seizures are tonic spasms, drop attacks and partial. Rare myoclonus can also occur

30
Q

What are the 2 types of ohtahara’s syndrome?

A

cryptogenic–15% of the time

symptomatic–85% shows hemimegencephaly or atrophy on imaging

31
Q

What is the prognosis of ohtahara’s syndrome?

A

100% intellectually disabled (profound MR) with severe seizures

32
Q

What is the treatment for ohtahara’s syndrome?

A

ACTH, Steroids, VPA, KD, TOP, CLBZ, FLB

33
Q

What is Dravet’s syndrome, SEI?

A

Unilateral clonic seizures, febrile and afebrile, in the first year of life in previously normal infant
Recur at 6-8 wk intervals. May cause status – convulsive or subtle
Followed by absence then partial seizures.

34
Q

What is the family hx link w/ dravet’s syndrome?

A

25-50% of the time there is a family hx of epilepsy or febrile seizures

35
Q

What is the genetic link w/ dravet’s syndrome?

A

*****Associated with molecular defects in three sodium channel subunit genes and a gamma-aminobutyric acid (GABA) subunit gene.

36
Q

What does an EEG & MRI show w/ dravet’s syndrome?

A

EEG – GSW or slow poly SW seen in sleep.
Background normal at onset but deteriorates with time
MRI – normal

37
Q

What’s the deal with intellectual decline w/ dravet’s syndrome?

A

Intellectual decline develops in 50%

Can be stopped if seizures controlled

38
Q

Which drugs can be used to treat dravet’s syndrome?

A

VPA, CLBZ, TPM, Bromide, Benzo, KD, FELB

39
Q

What is a drug that you should NOT use to treat dravet’s syndrome?

A

**Sodium channel blockers make the condition worse! (CBZ, DPH, OXC, LMT)

40
Q

What is West syndrome (SEEI)?

A

Characterized by infantile spasms – brief bilaterally symmetic contraction of muscles of neck, trunk and extremities
4-8 mo

41
Q

What does the EEG show w/ a west syndrome child?

A

***EEG – hypsarrhythmia, Slow waves or slow SW, BS, rarely normal

42
Q

What can west syndrome be misdiagnosed as?

A

Can be misdiagnosed as Morrow reflex, colic, startle responses

43
Q

What percentage of west syndrome is symptomatic? Cryptogenic?

A

symptomatic (85%)

cryptogenic (15%)

44
Q

How do you treat west syndrome?

A

Treatment – ACTH, Steroids, VGB, CLB, KD, TOP, RUF

45
Q

What is lennox gastaut syndrome?

A

***Triad of slow spike-wave on EEG, mental retardation and mixed seizure types – myoclonic jerks, atypical absences and drop attacks (tonic or atonic).
electrodecremental on EEG

46
Q

What is the age range for lennox gastaut syndrome?

A

2-14 yo

47
Q

What is acquired epileptic a phase-Landau-Klefner syndrome?

A
Ages 3-9 y/o, Males > Females
EEG shows multifocal SW
Child develops verbal agnosia and decreased spontaneous speech
Remits before age 15
Etiology unknown
Seizures may not be prominent feature
48
Q

What is the treatment for acquired epileptic aphasia?

A

steroids, KD, felbamate, early speech therapy. 40-50% recover to a normal life.

49
Q

What is electrical status epilepticus during sleep?

A

EEG – some bursts of SW while awake become continuous in sleep (1.5-2Hz)
Seizures – partial, generalized, atypical absence
**don’t meet milestones

50
Q

What are absence seizures? Not encephalopathy. Normal IQ

A

Bihemispheric initial involvement of 3 Hz GSW lasting 3-10 sec
primary generalized seizure
Average age of onset 5.7 yrs (3-9 y/o)
3 types: Childhood absence, juvenile absence, juvenile myoclonic epilepsy

51
Q

What are the medications used to treat absence seizures?

A

ETX, LTG, LEV, VPA, TOP, ZON

52
Q

What is juvenile myoclonic epilepsy?

A

Most common epilepsy syndrome
Possibly 20% of all epilepsies
Hallmark is myoclonic jerks, fast GSW (4-6 Hz) normal IQ and development
Lifelong (maybe 10% remit)

53
Q

What is the only indication for ETX as a treatment?

A

absence seizures

54
Q

What is the associated gene w/ juvenile myoclonic epilepsy?

A

myoclonic gene involved in spindle body directing cell division and neuronal migration

55
Q

What is benign focal epilepsy of childhood?

A

*****common childhood epilepsy: 13-23%
GTCs occur in sleep, some in daytime

Partial seizures are unilateral paresthesias +/- clonus of face, lips, tongue. Also salivation, speech arrest but preserved consciousness.
EEG – centro-temporal spikes, high amplitude, triphasic

56
Q

When does benign focal epilepsy of childhood remit?

A

***usu remits by age 16

57
Q

What are the similar symptoms of benign epilepsy of childhood with occipital paroxysms?

A

Ictal visual symptoms, amaurosis, hallucinations
High amplitude diphasic occipital spikes.
Eye opening suppresses spikes.

58
Q

Most common seizure disorder for little babies? Little older children? Children & adults?

A

Febrile Seizure (also most common type of SEIZURE)
Benign Focal Epilepsy of Childhood
Juvenile Myoclonic Epilepsy

59
Q

What is panayiotopolus syndrome?

A

Benign occipital; spikes occipital or anterior

With autonomic onset, autonomic status,

60
Q

What is atypical benign partial epilepsy of childhood?

A

CTSW bilateral. Focal ones produce focal atonias, when they generalize produce atonic drops. Nocturnal seizures = Rolandic, GTC or jerks. EEG in sleep = ECSWS. All recover

61
Q

What is hypothalamic hamartomas (gelastic) epilepsy?

A

laughing seizures

**Laughter without emotion, 10-30 sec, frequent
boys>girls
surgery on hypothalamus may be helpful.
can also present with autonomic, complex partial general tonic clonic seizures

62
Q

1 unprovoked seizure with no abnormality on imaging means you have epilepsy?

A

***NO

63
Q

1 unprovoked seizure w/ 1 abnormality on imaging means you have epilepsy?

A

***YES

64
Q

2 unprovoked seizures means you have epilepsy?

A

**YES