Pediatric Epilepsy Syndromes

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Questions and Answers

What is the typical onset age range often cited for Self-Limited Epilepsy with Centrotemporal Spikes (SELECTS)?

  • 12-14 years
  • 8-9 years (correct)
  • 0-3 years
  • 4-6 years

SELECTS (Self-Limited Epilepsy with Centrotemporal Spikes) seizures often occur during which time?

  • Early morning
  • During school hours
  • Postprandial
  • Sleep (correct)

Which treatment is commonly used, particularly for seizures associated with Dravet Syndrome or Lennox-Gastaut Syndrome?

  • Ethosuximide
  • Phenobarbital
  • Carbamazepine
  • Cannabidiol (correct)

Which syndrome is associated with Hemiconvulsion-Hemiplegia-Epilepsy?

<p>HHE (Hemiconvulsion-Hemiplegia-Epilepsy Syndrome) (D)</p> Signup and view all the answers

A child presenting with seizures triggered primarily by eye closure likely has which syndrome?

<p>EEM (Epilepsy with Eyelid Myoclonia / Jeavons Syndrome) (B)</p> Signup and view all the answers

What EEG feature is commonly expected in generalized epilepsy syndromes such as Juvenile Myoclonic Epilepsy?

<p>Generalized polyspike-and-wave (B)</p> Signup and view all the answers

Which EEG pattern is most characteristic of West Syndrome (Infantile Spasms)?

<p>Hypsarrhythmia (D)</p> Signup and view all the answers

Which syndrome often features prominent autonomic symptoms like vomiting, pallor, or cyanosis during seizures?

<p>POLE (Panayiotopoulos Syndrome/Posterior Occipital Lobe Epilepsy) (B)</p> Signup and view all the answers

According to this source, what EEG feature is expected in Panayiotopoulos Syndrome / POLE?

<p>Generalized polyspike-and-wave (A)</p> Signup and view all the answers

In Dravet Syndrome, which medication is often avoided or may worsen seizures, despite being listed as an option here?

<p>Phenytoin (A)</p> Signup and view all the answers

Which syndrome label represents Developmental and Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep?

<p>DEE-SWAS (C)</p> Signup and view all the answers

Which of the following is a self-limited focal epilepsy syndrome of childhood?

<p>SELECTS (Self-Limited Epilepsy with Centrotemporal Spikes) (D)</p> Signup and view all the answers

Which condition is characterized by refractory status epilepticus or frequent seizures following a febrile illness in a previously healthy child?

<p>FIRES (Febrile Infection-Related Epilepsy Syndrome) (B)</p> Signup and view all the answers

Seizures that involve vomiting are characteristic of which syndrome listed?

<p>SELEAS (possible typo/acronym) (C)</p> Signup and view all the answers

What EEG abnormality, characterized by periods of high-voltage activity alternating with near-flat suppression, is seen in severe early infantile epileptic encephalopathies like Ohtahara Syndrome?

<p>Suppression-burst (A)</p> Signup and view all the answers

Which treatment is considered first-line for Infantile Spasms (West Syndrome), especially if associated with Tuberous Sclerosis Complex?

<p>Vigabatrin (C)</p> Signup and view all the answers

Which syndrome typically involves multiple seizure types (like tonic, atonic, atypical absence) and slow spike-and-wave patterns on EEG?

<p>LGS (Lennox-Gastaut Syndrome) (C)</p> Signup and view all the answers

Which epilepsy syndrome typically involves characteristic centrotemporal spikes on EEG, although not listed as an option here?

<p>SELECTS (Self-Limited Epilepsy with Centrotemporal Spikes) (A)</p> Signup and view all the answers

According to this source, what EEG feature is expected in SELECTS (Self-Limited Epilepsy with Centrotemporal Spikes)?

<p>Generalized polyspike-and-wave (D)</p> Signup and view all the answers

What is the typical age of onset for Lennox-Gastaut Syndrome (LGS)?

<p>3-6 years (A)</p> Signup and view all the answers

What is the typical age of onset cited in this source for SELECTS (Self-Limited Epilepsy with Centrotemporal Spikes)?

<p>3-6 years (D)</p> Signup and view all the answers

What is the typical onset age range for SELECTS (Self-Limited Epilepsy with Centrotemporal Spikes)?

<p>8-9 years (B)</p> Signup and view all the answers

During which state do SELECTS seizures most often occur?

<p>Sleep (D)</p> Signup and view all the answers

Which treatment is commonly used for seizures associated with Dravet Syndrome or Lennox-Gastaut Syndrome?

<p>Cannabidiol (C)</p> Signup and view all the answers

Which syndrome is associated with a history of hemiconvulsions and often results in hemiplegia and epilepsy?

<p>HHE (A)</p> Signup and view all the answers

A child presents with frequent eyelid myoclonia often triggered by eye closure. Which syndrome is most likely?

<p>EEM (A)</p> Signup and view all the answers

What EEG feature is typically expected in EEM (Epilepsy with Eyelid Myoclonia)?

<p>Generalized polyspike-and-wave (B)</p> Signup and view all the answers

Which EEG pattern is most characteristic of West Syndrome (Infantile Spasms)?

<p>Hypsarrhythmia (A)</p> Signup and view all the answers

In Dravet Syndrome, which medication is often contraindicated as it may worsen seizures?

<p>Phenytoin (D)</p> Signup and view all the answers

Which syndrome typically shows continuous or near-continuous spike-and-wave activity during slow-wave sleep on EEG?

<p>DEE-SWAS (B)</p> Signup and view all the answers

Which of the following epilepsy syndromes is typically self-limited, meaning it resolves with age?

<p>SELECTS (B)</p> Signup and view all the answers

Which condition is characterized by new onset of refractory status epilepticus following a febrile illness in previously healthy children?

<p>FIRES (C)</p> Signup and view all the answers

Seizures involving prominent autonomic features like vomiting, pallor, and cyanosis are characteristic of which syndrome?

<p>SELEAS (A)</p> Signup and view all the answers

What EEG abnormality, characterized by periods of high-voltage activity alternating with periods of near-flat suppression, is seen in syndromes like Ohtahara syndrome?

<p>Suppression-burst (B)</p> Signup and view all the answers

Which treatment is considered first-line for Infantile Spasms (West Syndrome)?

<p>Vigabatrin (A)</p> Signup and view all the answers

Which syndrome involves multiple seizure types (including tonic and atypical absence seizures), cognitive impairment, and slow spike-and-wave complexes on EEG?

<p>LGS (A)</p> Signup and view all the answers

What is a typical age of onset for Childhood Absence Epilepsy (CAE)?

<p>3-6 years (C)</p> Signup and view all the answers

Flashcards

Typical Seizure Onset Age

Typical onset age range for seizures is 8-9 years.

SELECTS Seizure Timing

SELECTS seizures frequently occur during sleep.

Common Seizure Treatment

Cannabidiol is a commonly used treatment.

Syndrome Associated with HHE

HHE (hemiconvulsion-hemiplegia-epilepsy syndrome) is associated.

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Triggered Seizures

EEM (Epilepsy with Electrical Status Epilepticus during Sleep)

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EEG Feature

Generalized polyspike-and-wave is the expected EEG feature.

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EEG Pattern

Hypsarrhythmia is the EEG pattern.

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Epilepsy Syndromes

SELECTS is an epilepsy syndrome.

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Conditions Characterized

FIRES is a characterized condition.

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Syndromes Involving Atypical

SELECTS stands for Self-Limited Epilepsies with centrotemporal Spikes.

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EEG Pattern

LGS EEG pattern is distinctive.

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EEG abnormality

Suppression-burst EEG.

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First-Line Treatment

Vigabatrin is a first-line treatment.

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Syndrome Displays

DEE-SWAS typically shows.

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Syndrome Features

POLE often features Generalized polyspike-and-wave.

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Dravet Syndrome Medication

Phenytoin is the medication used

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Pediatric Epilepsy

Lennox-Gastaut Syndrome is an epilepsy syndrome.

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What SELEAS stands for

SELEAS stands for Self-limited late-onset epilepsy with autonomic seizures

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LGS Onset Age

LGS typically has a onset age from 3-6 years.

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Study Notes

  • Childhood Occipital Visual Epilepsy (COVE) typically starts at 8-9 years of age
  • SeLECTS seizures often occur during sleep
  • Cannabidiol is commonly used to treat Lennox–Gastaut Syndrome
  • HHE syndrome is associated with prolonged hemiclonic seizures and subsequent hemiplegia
  • EEM syndrome occurs when seizures are triggered by light and eyelid fluttering
  • Epilepsy with Myoclonic-Atonic Seizures (EMAtS) demonstrates generalized polyspike-and-wave EEG features
  • Phenytoin should be avoided in Dravet Syndrome as it can worsen seizures
  • DEE-SWAS typically shows paroxysmal spike-wave occupying >85% of NREM sleep
  • SeLECTS usually remits by adolescence and has a very good prognosis
  • SeLECTS is often associated with GRIN2A variants and may evolve to EE-SWAS
  • FIRES is characterized by refractory status epilepticus following a febrile illness
  • SeLECTS usually remits by adolescence and has a good prognosis
  • The syndrome FIRES, is characterized by refractory status epilepticus following a febrile illness
  • SeLEAS involves seizures with vomiting, pallor, and retching
  • Suppression-burst EEG abnormality is seen in KCNQ2-related DEE
  • Vigabatrin is a first-line treatment for infantile spasms in tuberous sclerosis complex
  • HHE syndrome is associated with prolonged hemiclonic seizures and subsequent hemiplegia
  • DEE-SWAS is drug-resistant and may evolve to cognitive regression
  • LGS involves atypical absences, tonic and atonic seizures, and slow spike-and-wave EEG
  • SeLECTS typically begins between 3 and 14 years of age and features centrotemporal spikes
  • SeLEAS onset is typically between 3-6 years
  • Hypsarrhythmia is an EEG pattern characteristic of infantile spasms syndrome (West Syndrome)
  • POLE syndrome often features a photoparoxysmal response and visual aura on EEG

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