Pediatric Epilepsy Flashcards

1
Q

What are some non-epileptic mimicing events?

A

There are many types of events that appear to be seizures but are not. EEG and video-EEG monitoring are helpful in differentiating these events from seizures.

Breath holding spells, Hyperventilation attacks

Motor tics,Movement disorders

Parasomnias (nightmares, night terrors, sleep walking)

Syncope

Spasmus nutans

Rumination

Sleep myoclonus

Head banging

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2
Q

What are psychogenic nonepileptic seizures (PNES) (formery pseudoseizures)?

A

Paroxysmal episodes that resemble epileptic seizures; however, PNES are psychological in origin.

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3
Q

How can PNES be diagnosed?

A

–Video-EEG monitoring in an epilepsy monitoring unit (EMU) is helpful in diagnosis of this condition.

•Epileptiform activity is seen on the EEG during seizures but NOT during psychogenic nonepileptic seizures.

–To make matters more confusing, some patients have both types of events.

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4
Q

What are some characteristics of psychogenic seizures?

A

may be of prolonged duration

only in daytime only

injury, tongue biting, urinary incontinence, and postictal confusion are rare

motor activity is prolonged, uncoordinated

psychiatric disturbances common

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5
Q

What are the lab/imaging findings of psychogenic seizures?

A

Interictal and ictal EEGs normal

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6
Q

Describe the findings of epileptic seizures

A

usually stereotypes

brief duration

can occur nocturnally or during the day

injury, tongue biting, and urinary continence common (especially with tonic-clonic seizures)

EEGs abnormal

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7
Q
A
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8
Q

What is Diastat?

A

Diastat (rectal diazepam) is for status epilepticus and seizure clusters

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9
Q

What should you do if a person has a seizure in the hospital?

A

ABCs: Place the patient on his/her side and Administer oxygen.

Ask the nurse to administer a benzodiazepine (lorazepam, midazolam, diazepam).

Consider loading with an antiepileptic medication.

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10
Q
A
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11
Q

What are the two main types of seizures?

A

partial/focal

generalized

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12
Q
A
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13
Q

What is a partial seizure?

A

those in which the first clinical and EEG changes indicate initial activation of a system of neurons limited to part of one cerebral hemisphere

NOTE: Generalized seizures are those in which the first clinical changes indicate initial involvement of both hemispheres

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14
Q

What are the types of partial seizures?

A

–Simple partial

–Complex partial

The classification of partial seizures depends on whether or not consciousness is impaired

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15
Q

What is ‘impaired’ consciousnes?

A

Impaired consciousness is defined as the inability to respond normally to exogenous stimuli.

When consciousness is preserved, the seizure is classified as a simple partial seizure.

When consciousness is impaired, the seizure is classified as complex partial.

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16
Q

What are the types of generalized seizures?

A

–Absence or atypical absence

–Myoclonic

–Clonic

–Tonic

–Tonic-Clonic

–Atonic

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17
Q

Describe absence seizures?

A

–Characterized by the sudden onset of interruption of activity.

•If walking, the patient will stop. If eating, the food will stop on the way to the patient’s mouth. If talking, speech will slow or stop.

–Usually the patient will be unresponsive if spoken to.

–Lasts a few seconds to half a minute.

–Stops as rapidly as it started.

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18
Q

Absence seizures may be e induced by what?

A

hyperventilation.

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19
Q
A
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20
Q

Absence seizures need to differentiated from complex partial seizures. Describe absence seizures

A

Childhood in onset

no aura or warning

abrupt onset and lasting only seconds in duration, and termination is abrupt

automatisms are simple

provocation by hyperventilation common

no postictal phase

neuroimagina normal

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21
Q

Absence seizures need to differentiated from complex partial seizures. Describe complex partial seizures

A

age of onset at any age

aura or warning frequent

onset and termination gradual, and lasting minutes

frequency only occasional

postictal phase common, marked by confusion and fatigue

neuroimaging may show focal lesions

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22
Q

What is the most common type of childhood seizure?

A

Febrile seizures (affecting 2-5% of children in the USA and peaking in incidence at 18 months of age)

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23
Q

Define febrile seizures

A

“Seizures that occur in febrile children between the ages of 6 and 60 months who do not have an intracranial infection, metabolic disturbance, or history of afebrile seizures.”

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24
Q

What are the types of febrile seizure?

A

simple (isolated (once in a 24 hr period), generalized, and brief (less than 15 min))

complex (multiple occurences in 24 hr, focal, and prolonged (15+ min)) (Associated with a higher risk of afebrile seizures but not of febrile seizure recurrence)

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25
Q

How common is recurrence of febrile seizures?

A

32% experienced recurrent seizures –(17.1% - 1 recurrence, 9% - 2 recurrences, 6% - > 3 recurrences)

90% of children have recurrence within 1 year of onset

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26
Q

What are the risk factors for recurrence of a febrile seizure?

A

–young age at onset (< 18 months)

–febrile seizure in 1st degree relative

–low grade fever in E.R.

–brief duration between fever and seizure (< 1 hr.)

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27
Q

What are the risk factors for epilepsy in children with febrile seizures?

A

–Complex febrile seizures

–A family history of epilepsy

–Neurologic impairment prior to the febrile seizure

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28
Q

How should the neurodiagnostic Evaluation of the Child with a First Simple Febrile Seizure be handled?

A

Practice Guideline (AAP)

Lumbar puncture

–strongly considered if < 12 mo. old

–considered if >12, < 18 mo. old

–not routine, but recommended if meningeal signs are present in a patient who is > 18 mo. old

–strongly considered in all children on prior antibiotic treatment

EEG

–Should not be performed in the evaluation of neurologically healthy child with a first simple febrile seizure.

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29
Q

More on Neurodiagnostic Evaluation of the Child with a First Simple Febrile Seizure

A

Blood studies

–Should not be routinely performed (electrolytes, calcium, magnesium, CBC)

–Laboratory testing directed towards identifying the source of the fever

Neuroimaging –Should not be performed

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30
Q

Describe the long term managemnet of children with simple febrile seizures

A

-Neither continuous nor intermittent anticonvulsant therapy is recommended for children with 1 or more simple febrile seizures.

“In situations in which parental anxiety associated with febrile seizures is severe, intermittent oral diazepam at the onset of febrile illness may be effective in preventing recurrence.”

Antipyretics will not prevent febrile seizures.

31
Q

More on the long term management of children with SIMPLE febrile seizures

A

I would discharge the patient with Diastat (rectal diazepam) prn seizure more than 3 minutes or seizure cluster. However, if the family is giving oral diazepam, they should not administer Diastat until a physician states that they may do so safely.

32
Q

What is the FEBSTAT study?

A

The “Consequences of Prolonged Febrile Seizures in Childhood” (FEBSTAT) study was a prospective, multicenter study designed to study the acute and long term consequences of febrile status epilepticus.

33
Q

What were the major findings of the FEBSTAT study?

A

Children with FSE are at risk for acute hippocampal injury and a substantial number also have abnormalities in hippocampal development.

34
Q

Febrile Status Epilepticus-
The FEBSTAT Study

A

–Focal EEG slowing or attenuation are present in EEGs obtained within 72 hours in a substantial proportion of children with febrile status epilepticus (FSE) and are highly associated with MRI evidence of acute hippocampal injury.

–FSE rarely causes cerebrospinal fluid (CSF) pleocytosis, so CSF pleocytosis should not be attributed to FSE.

35
Q

What infection is associated with FSE?

A

HHV-6B infection is commonly associated with FSE. HHV-7 infection is less often associated with FSE. Together, they account for one third of FSE.

36
Q

Practice Parameter: Evaluating a First Non-Febrile Seizure in Children

(AAN, CNS, AES)

  • Applies to partial-onset, tonic, or generalized tonic-clonic seizures without a precipitating cause.
  • The parameter does NOT include patients with epilepsy. It only addresses the first, nonfebrile seizure.
A

•Immediate evaluation: Stabilization of the child, Determine if a seizure has occurred, and, if so, determine the cause of the seizure

•Laboratory studies: Ordered based on individual clinical circumstances (chemistries, blood count) (option); Toxicology screening should be considered if any question of drug exposure (option)

•Lumbar puncture (option): Limited value in general but used when there is concern about possible meningitis/encephalitis

•EEG: Recommended as part of the evaluation (standard) (–Include wake, sleep, hyperventilation, photic stimulation); Helps determine seizure type, epilepsy syndrome

Neuroimaging: If is performed, MRI is the preferred modality (guideline).

37
Q

What EEG pattern in a first non-febrile seizure in children is predictive of seziure recurrence?

A

•Focal slowing, epileptiform discharges predictive of seizure recurrence

38
Q

Practice Parameter: Treatment of the Child with a First Unprovoked Seizure

(AAN, CNS)

A

Treatment after a first unprovoked seizure appears to decrease the risk of a second seizure (partial-onset or generalized tonic-clonic)

However, There is no benefit of treatment with regard to prognosis for long-term seizure remission

Anti-epileptic drugs (AEDs) carry risks of side effects (systemic, behavioral, cognitive)

The decision to treat or not must be based on a risk-benefit assessment, individualized, take into account medical issues and patient preference

39
Q

Describe the diangostic assessment of children with status epilepticus (SE)

A

Anti-epileptic drug (AED) levels should be considered when a child with epilepsy on an AED develops SE.

Toxicology testing may be considered.

There is insufficient data to support or refute whether blood cultures or LP should be done on a routine basis if there is no clinical suspicion of infection.

An EEG may be considered.

Neuroimaging may be considered if there are clinical indications or the etiology is unknown

40
Q

What is epilepsy?

A

nas the occurrence of multiple unprovoked seizures separated by more than 24 hours.

41
Q
A
42
Q

Describe Infantile spasms

A

Epilepsy syndrome initially beginning at 3-7 months of life and maked by seizures (infantile spams) that are brief, bilateral, and symmetric contraction of the muscles of the neck, trunk, and extremities

43
Q

What part of the body is most commonly affected by Infantile spasms?

A

Most commonly, flexor spasms are seen

–The head is flexed, the arms are extended, and the legs are drawn up. The patient may cry or giggle. He/she may flush or turn pale or cyanotic. Parents may report stomach crunches.

Patients may have extensor spasms characterized by extension of arms, legs, and trunk.

Less common presentations include head nodding or lightening attacks, which involve a single, momentary shock-like contraction of the entire body.

44
Q

What does an EEG show with infantile spasms?

A

shows hypsarrhythmia.

Between seizures, there is high voltage, chaotic activity. (Looks the same if you hold EEG upside down.) There may be diffuse voltage depression during seizures.

45
Q

What is West Syndrome?

A

West syndrome is the triad of infantile spasms, hypsarrhythmia, and developmental arrest/regression.

46
Q

These EEGs show background slowing (high amplitude delta waves) and multifocal spikes.

A
47
Q

How should infantile spasms be tx?

A

Some etiologies of infantile spasms have specific treatments.

Treatments: ACTH, vigabatrin, topiramate, zonisamide, valproic acid, benzodiazepines, ketogenic diet.

48
Q

How should pyridoxine dependent infantile spasms be tx? PKU dependent? Maple syrup urine disease dependent?

A

pyridoxine dependent- vitB6

PKU dependent- diet

Maple syrup urine disease dependent- diet

49
Q

How should tumor/AVM induced infantile spasms be tx? Biotinidase deficiency induced? Menkes’ disease induced?

A

tumor/AVM induced infantile spasms- surgery

Biotinidase deficiency induced- biotinidase

Menkes’ disease induced- histidinate

50
Q

How should tuberous sclerosis induced infantile seizures be tx? Glucose transporter deficiency induced? Focal cortical dysplasia induced?

A

Tuberous sclerosis: vigabatrin

Glucose transporter deficiency: ketogenic diet

Focal cortical dysplasia: resection

51
Q

What is Lennox-Gastaut?

A

Seizure syndrome with onset at 1-8 yo (9-39% have infantile spasms) and presenting with the triad of:

At least 2 seizure types including tonic, atypical absence, and atonic seizures.

Slowing of mental development.

EEG: “slow spike and wave” –slow background, bursts of diffuse slow spike-wave patterns of 1.5-2.5 Hz.

52
Q

Recall that absence seizures are associated with generalized 3 Hz spike and wave discharges. Atypical absence seizures, which are seen in some patients with Lennox-Gastaut syndrome, are characterized by generalized 1.5-2.5 Hz spike and wave discharges.

Atypical absence seizures last longer than absence seizures and are seen in children with developmental delay. Patients with absence seizures typically have normal intelligence.

A
53
Q

The seizures of Lennox-Gastaut are life-long and difficult to control. What are some tx options?

A

valproic acid, lamotrigine, topiramate, zonisamide, felbamate, benzodiazepines, ketogenic diet, corpus callosotomy, vagus nerve stimulator.

54
Q

Describe childhood absence epilepsy (CAE)

A

Epilepsy syndrome presenting at 4-8 yo with normal neurologic status and marked by absence seizures that occur multiple times per day with normal, generalized 3Hz spike-and-wave discharged (may be trigger by hyperventilation)

Normal EEG background

55
Q

How is childhood absence epilepsy (CAE) tx? prognosis?

A

Tx: Ethosuximide is the preferred treatment if the patient only has absence seizures, but CAE may also be treated with valproic acid or lamotrigine. Tegretol may worsen seizures.

Benign outcome. Treat 2-3 years.

56
Q

There was a multi-center NIH-sponsored trial comparing lamotrigine, ethosuximide, and valproic acid for childhood absence epilepsy. It showed that ethosuximide and valproic acid are more effective than lamotrigine, but ethosuximide has less impact on attention.

A
57
Q

Describe juvenile myoclonic epilepsy

A

onset in adolescence with seizures that may be tonic-clonic, myoclonic, or absence

The usual age at onset of absence seizures is 7 to 13 years. The usual onset of myoclonic jerks is 12 to 18 years. The usual onset of generalized tonic-clonic seizures 13 to 20 years.

58
Q

Describe the myoclonic seizures of JME

A

The myoclonic seizures are brief, bilateral but not always symmetric, flexor jerks of the arms, which may be repetitive. The jerk sometimes affects the legs, causing the patient to fall. (Ask the patient if he/she tends to drop objects in the morning. Patients with myoclonic jerks may report spilling orange juice, for example.)

The highest frequency of myoclonic jerks is in the morning.

Consciousness is usually retained so that the patient is aware of the jerking movement.

Diagnosis is often delayed until a generalized tonic-clonic seizure brings the patient to medical attention.

59
Q

More about JME

A

Seizures are precipitated by sleep deprivation, alcohol ingestion, stress, and awakening from nocturnal or daytime sleep. Menstruation may also be a trigger. Photic stimulation is also a precipitating factor. (Patients can be given photic stimulation during an EEG to produce epileptiform discharges.)

Neurologic examination is typically normal.

Neuroimaging is normal.

60
Q

How is JME tx?

A

Usually requires life-long treatment.

Treatment: valproic acid, topiramate, levetiracetam, lamotrigine, zonisamide.

61
Q

What causes JME?

A

Hereditary disorder. Thought to be autosomal dominant. Linked to chromosome 6.

62
Q

What is the most common form of benign partial epilepsy of childhood (all before are generalized)?

A

Benign Rolandic Epilepsy (BRE)

63
Q

Where does Benign Rolandic Epilepsy (BRE) (Also known as benign epilepsy with centro-temporal spikes (BECT) and benign focal epilepsy of childhood (BFEC)) originate?

A

Epileptiform discharges arise from the lower Rolandic area of the brain.

64
Q

Describe BRE (including age of onset)

A

Onset 4 to 12 years, peak age 8-9 years.

Normal neurologic status and imaging.

Most specific type of seizure: facial motor seizures.

65
Q

What is the most common presentation of BRE?

A

nocturnal generalized tonic-clonic seizures (80% of patients).

66
Q

How is BRE tx? Prognosis?

A

Usually respond to carbamazepine or valproate but may not need to be treated.

Seizures usually remit by age 14 to 16.

67
Q

What is the classic EEG finding of BRE?

A

central-temporal spikes.

68
Q

This diagram shows which medications are used to treat partial seizures, which medications treat generalized seizures, and which medications treat both.

A

Ethosuximide only treats absence seizures, but absence seizures can be treated with other medications such as lamotrigine or valproate. Patients who have generalized tonic-clonic seizures in addition to absence seizures should not be treated with ethosuximide, as it will not treat the generalized tonic-clonic seizures.

69
Q

ACTH is only used to treat infantile spasms.

A
70
Q

What AEDs can tx both partial and generalized seizures?

A

Valproic acid (valproate), lamotrigine, topiramate, felbamate, and zonisamide are broad-spectrum agents. I would also add levetiracetam to this list. These medications can be used to treat generalized seizures or partial seizures.

71
Q

Medications such as ______, _______, ______, _____, _______, and ________ are more useful for treating partial seizures than generalized seizures. In fact, they may worsen generalized seizures.

A

phenytoin, carbamazepine, phenobarbital, gabapentin, tiagabine, and oxcarbazepine

72
Q

In this diagram, LEV stands for levetiracetam. As I mentioned before, I would consider this broad spectrum; it can be used for partial and generalized seizures.

A

Lacosamide is approved for patients over 17 years of age with partial seizures.

Rufinamide is approved for patients with Lennox-Gastaut syndrome over age 4 years.

73
Q

In 2000, Kwan and Brodie showed that only 4% became seizure-free with the third drug or multiple AEDs.

A

This diagram shows an approach to treating epilepsy.

The EMU is the epilepsy monitoring unit, where video-EEG monitoring is performed.