Pathognomonic EEG Patterns
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Questions and Answers

What is the typical prognosis of Lennox-Gastaut Syndrome?

  • Poor, with limited response to treatment (correct)
  • Variable, depending on the underlying cause
  • Fair, with manageable seizures
  • Good, with full recovery

    • What is the age range of onset for Lennox-Gastaut Syndrome?

  • Birth to 1 year
  • 8-15 years
  • 15-25 years
  • 1-8 years (correct)

    • What percentage of Lennox-Gastaut Syndrome cases are preceded by infantile spasms?

  • 5-10%
  • 10-25% (correct)
  • 25-50%
  • 50-75%

    • What is the most common cause of Lennox-Gastaut Syndrome?

    <p>Structural or metabolic abnormalities (B)</p> Signup and view all the answers

    What is a characteristic feature of Severe Myoclonic Epilepsy of Infancy (SMEI)?

    <p>Normal early development, then later deterioration (B)</p> Signup and view all the answers

    What is the age range for the onset of febrile seizures?

    <p>Between 1-5 years old (D)</p> Signup and view all the answers

    What is the typical EEG finding in Severe Myoclonic Epilepsy of Infancy (SMEI)?

    <p>Polyspike-and-wave activity (C)</p> Signup and view all the answers

    What percentage of the general population is affected by febrile seizures?

    <p>3-5% (B)</p> Signup and view all the answers

    What is the genetic mutation commonly associated with Severe Myoclonic Epilepsy of Infancy (SMEI)?

    <p>SCN1A (D)</p> Signup and view all the answers

    What medication should be avoided in Severe Myoclonic Epilepsy of Infancy (SMEI)?

    <p>Sodium-channel medications (B)</p> Signup and view all the answers

    What is the recurrence risk of febrile seizures?

    <p>33% will have a second FS (B)</p> Signup and view all the answers

    What is the characteristic EEG pattern associated with Crutzfeldt-Jakob Disease (CJD)?

    <p>Generalized periodic discharges (A)</p> Signup and view all the answers

    What is the frequency range of the periodic discharges in Crutzfeldt-Jakob Disease (CJD)?

    <p>0.5-2 Hz (D)</p> Signup and view all the answers

    Which of the following is a characteristic of Subacute Sclerosing Panencephalitis (SSPE)?

    <p>Inflammation of the brain (encephalitis) (A)</p> Signup and view all the answers

    What is the genetic mutation associated with Genetic Epilepsy and Febrile Seizures Plus (GEFS+)?

    <p>SCN1A, SCN1B, GABRG2 (D)</p> Signup and view all the answers

    What percentage of individuals with Genetic Epilepsy and Febrile Seizures Plus (GEFS+) have a detectable genetic mutation?

    <p>10-20% (D)</p> Signup and view all the answers

    What is the typical age range of onset for Myoclonic Astatic Epilepsy (MAE)?

    <p>1-5 years old (A)</p> Signup and view all the answers

    What is the percentage of intellectual disability in patients with Infantile Spasms and West Syndrome?

    <p>75-90% (A)</p> Signup and view all the answers

    Which of the following EEG patterns is associated with Myoclonic Astatic Epilepsy (MAE)?

    <p>2-3 Hz generalized slow wave activity (B)</p> Signup and view all the answers

    What is the age of onset for Infantile Spasms?

    <p>4-8 months (D)</p> Signup and view all the answers

    What is the name of the triad associated with West Syndrome?

    <p>Hypsarrhythmia, Developmental Delay, and Seizures (A)</p> Signup and view all the answers

    What is the prognosis for patients with Myoclonic Astatic Epilepsy (MAE)?

    <p>Typically poor with severe intellectual disability (B)</p> Signup and view all the answers

    Which of the following syndromes is characterized by sudden or gradual aphasia and verbal auditory agnosia?

    <p>Landau-Kleffner Syndrome (D)</p> Signup and view all the answers

    What is the most common type of epilepsy syndrome?

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

    What is the term for a group of signs or symptoms that happen together and help identify a unique medical condition?

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

    What is the typical age range of onset for Panayiotopolous Syndrome?

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

    Which of the following medications is considered optimal initial therapy for Absence Epilepsy?

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

    What is the term for epilepsy syndromes that share specific signs or symptoms?

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

    What is the characteristic EEG pattern associated with Juvenile Myoclonic Epilepsy?

    <p>High amplitude, generalized 4-6 Hz polyspike and wave (B)</p> Signup and view all the answers

    What is the percentage of symptomatic cases of Infantile Spasms and West Syndrome?

    <p>75-85% (D)</p> Signup and view all the answers

    What is the typical prognosis for patients with Juvenile Myoclonic Epilepsy?

    <p>Typically good with normal cognitive function (A)</p> Signup and view all the answers

    What is the first line of treatment for Infantile Spasms and West Syndrome?

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

    Which of the following EEG patterns is often seen in SSPE?

    <p>Bilaterally synchronous, high-amplitude spike or slow-wave bursts (D)</p> Signup and view all the answers

    What is the typical cause of Herpes Simplex Encephalitis (HSE)?

    <p>Herpes simplex virus (HSV) (B)</p> Signup and view all the answers

    What is the characteristic EEG pattern in Anti-NMDA Receptor Encephalitis?

    <p>Extreme delta brush (D)</p> Signup and view all the answers

    What is the typical genetic abnormality associated with Angelman Syndrome?

    <p>Maternal deletion of 15q11 and 15q13 (D)</p> Signup and view all the answers

    What is the characteristic EEG pattern in Angelman Syndrome?

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

    What is the term for a specific EEG pattern that is highly indicative of a certain neurologic condition?

    <p>Pathognomonic EEG pattern (A)</p> Signup and view all the answers

    Which of the following is a common feature of SSPE?

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

    What is the term for a group of seizure disorders characterized by seizure types, age at onset, and EEG pattern?

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

    What is the percentage of intellectual disability in patients with Infantile Spasms and West Syndrome?

    <p>75-90% (A)</p> Signup and view all the answers

    What is the term for a specific EEG pattern that is highly indicative of a certain neurologic condition?

    <p>Pathognomonic EEG pattern (D)</p> Signup and view all the answers

    What is the age range of onset for Infantile Spasms?

    <p>4-8 months (C)</p> Signup and view all the answers

    What is the triad associated with West Syndrome?

    <p>Hypsarrhythmia, infantile spasms, and developmental delay (D)</p> Signup and view all the answers

    What is the term for a group of signs or symptoms that happen together and help identify a unique medical condition?

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

    What is the percentage of symptomatic cases of Infantile Spasms and West Syndrome?

    <p>75-85% (C)</p> Signup and view all the answers

    What is the first line of treatment for Infantile Spasms and West Syndrome?

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

    What is the term for epilepsy syndromes that share specific signs or symptoms?

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

    What percentage of Lennox-Gastaut Syndrome cases have a family history of epilepsy?

    <p>3-30% (C)</p> Signup and view all the answers

    What is the typical characteristic of the EEG pattern in Lennox-Gastaut Syndrome?

    <p>Slow waves, multifocal episodic activity (A)</p> Signup and view all the answers

    What is the main reason for the poor prognosis in Lennox-Gastaut Syndrome?

    <p>Resistance to ASDs (C)</p> Signup and view all the answers

    What is the typical age range for the onset of myoclonic seizures in Severe Myoclonic Epilepsy of Infancy (SMEI)?

    <p>1-4 years (A)</p> Signup and view all the answers

    Why should sodium-channel medications be avoided in Severe Myoclonic Epilepsy of Infancy (SMEI)?

    <p>They can worsen seizures (A)</p> Signup and view all the answers

    What is the significance of cannabidiol in the treatment of Lennox-Gastaut Syndrome and Severe Myoclonic Epilepsy of Infancy (SMEI)?

    <p>It is a last resort (B)</p> Signup and view all the answers

    What is the characteristic feature of Severe Myoclonic Epilepsy of Infancy (SMEI) in terms of development?

    <p>Normal development followed by deterioration (A)</p> Signup and view all the answers

    What is the genetic mutation commonly associated with Severe Myoclonic Epilepsy of Infancy (SMEI)?

    <p>SCN1A (B)</p> Signup and view all the answers

    Which of the following epilepsy syndromes is associated with global regression in cognition and behavior?

    <p>Epileptic Encephalopathy with Continuous Spike and Wave During Sleep (CSWS) (D)</p> Signup and view all the answers

    What is the typical EEG pattern seen in Myoclonic Astatic Epilepsy (MAE)?

    <p>All of the above (D)</p> Signup and view all the answers

    Which of the following epilepsy syndromes is associated with verbal auditory agnosia?

    <p>Landau-Kleffner Syndrome (C)</p> Signup and view all the answers

    What is the typical age range of onset for Juvenile Absence Epilepsy?

    <p>10-17 years old (D)</p> Signup and view all the answers

    Which of the following medications is optimal initial therapy for Absence Epilepsy?

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

    What is the characteristic EEG pattern seen in Juvenile Myoclonic Epilepsy?

    <p>High amplitude, generalized 4-6 Hz polyspike and wave (C)</p> Signup and view all the answers

    Which of the following epilepsy syndromes is associated with a family history of epilepsy?

    <p>Juvenile Myoclonic Epilepsy (B)</p> Signup and view all the answers

    What is the typical prognosis for patients with Myoclonic Astatic Epilepsy (MAE)?

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

    What is the characteristic feature of Epilepsy Syndromes with Pathognomonic EEG Patterns?

    <p>They are associated with specific EEG patterns that are highly indicative of a particular disease or condition (B)</p> Signup and view all the answers

    What is the significance of finding a pathognomonic EEG pattern in a patient?

    <p>It is highly suggestive of a particular disease or condition (D)</p> Signup and view all the answers

    What is the term for a sign or symptom that is specifically characteristic of a particular disease or condition?

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

    What is the genetic mutation commonly associated with Genetic Epilepsy and Febrile Seizures Plus (GEFS+)?

    <p>All of the above (D)</p> Signup and view all the answers

    What is the typical age range of onset for Febrile Seizures?

    <p>Between 1-5 years old (A)</p> Signup and view all the answers

    What is the characteristic EEG pattern associated with Crutzfeldt-Jakob Disease (CJD)?

    <p>Generalized periodic discharges (B)</p> Signup and view all the answers

    What is the significance of the presence of myoclonic jerks in Crutzfeldt-Jakob Disease (CJD)?

    <p>It is a common feature of the disease (A)</p> Signup and view all the answers

    What is the term for a group of signs or symptoms that happen together and help identify a unique medical condition?

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

    What is the characteristic EEG pattern associated with Subacute Sclerosing Panencephalitis (SSPE) as the disease progresses?

    <p>Burst-suppression pattern (A)</p> Signup and view all the answers

    What is the typical cause of Herpes Simplex Encephalitis (HSE)?

    <p>Herpes simplex virus (HSV) (C)</p> Signup and view all the answers

    What is the characteristic EEG pattern associated with Anti-NMDA Receptor Encephalitis?

    <p>Extreme delta brush (C)</p> Signup and view all the answers

    What is the typical genetic abnormality associated with Angelman Syndrome?

    <p>Small interstitial deletion between 15q11 and 15q13, maternally derived (B)</p> Signup and view all the answers

    What is the characteristic EEG pattern associated with Angelman Syndrome?

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

    What is the term for a specific EEG pattern that is highly indicative of a certain neurologic condition?

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

    What is the characteristic EEG pattern associated with Subacute Sclerosing Panencephalitis (SSPE) initially?

    <p>Bilaterally synchronous, high-amplitude spike or slow-wave bursts (C)</p> Signup and view all the answers

    What is the term for a group of signs or symptoms that happen together and help identify a unique medical condition?

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

    Study Notes

    Epilepsy Syndromes

    • Epilepsy syndrome: a group of signs or symptoms that help identify a unique medical condition, characterized by specific signs or symptoms, type of seizure(s), age when seizures start, and pattern on EEG.
    • Types of epilepsy syndromes:
      • Symptomatic/Encephalopathic
      • Progressive and Infantile Myoclonic Epilepsies
      • Focal
      • Idiopathic
      • Febrile Seizure

    Infantile Spasms and West Syndrome

    • Infantile Spasms: flexor, extensor, lightning, or nods—most are mixed, onset 4-8 months, peak 5 mo.
    • West Syndrome: triad of infantile spasms, hypsarrhythmia, and developmental delay, onset 4-7 months, always before 1 year old, boys>girls.
    • Etiology: 75-85% symptomatic, 15-25% asymptomatic, underlying conditions include genetic, metabolic, congenital infection, and neonatal infection.
    • Treatment: steroids, vigabatrin, ketogenic diet, zonisamide, vitamin B6, prognosis partly based on early treatment, but typically poor.

    Lennox-Gastaut Syndrome

    • Triad: multiple seizures types, cognitive dysfunction, and slow GSW (1.5-2 Hz) on EEG.
    • Onset: 1-8 years, typically 3-5 yr, 10-25% cases preceded by infantile spasms.
    • Causes: 70-78% structural/metabolic, 30-22% unknown, family history of epilepsy in 3-30%.
    • Prognosis: overall refractory to ASDs and poor prognosis.

    Severe Myoclonic Epilepsy of Infancy (SMEI)

    • Prolonged febrile seizures in the 1st year of life, seizure-free period followed by myoclonic seizures at 1-4 yr old.
    • Normal early development, then later deterioration, pyramidal signs and ataxia.
    • EEG: slow SW, polyspike-and-wave, 70-80% mutation in SCN1A.

    Myoclonic Astatic Epilepsy (MAE) or Doose Syndrome

    • Type of generalized epilepsy, onset 1-5 years old.
    • EEG: 2-3 Hz gen SW, polyspike and 4-7 Hz central/vertex rhythmic theta activity.
    • MRI: normal, associated with SCN1A mutation, boys>girls (2:1).
    • Prognosis: typically poor with severe intellectual disability, ataxia, poor motor function, dysarthria, and poor language development.

    Epileptic Encephalopathy with Continuous Spike and Wave During Sleep (CSWS)

    • Epileptiform activity occupying >85% of NREM sleep, 2 syndromes: Landau-Kleffner Syndrome (LKS) and CSWS.
    • Landau-Kleffner Syndrome: typically age 3-10 y, sudden or gradual aphasia, verbal auditory agnosia, 2/3rd have seizures, ADHD.
    • CSWS: global regression in cognition and behavior, majority of patients have seizures, may have identifiable pathology.

    Panayiotopolous Syndrome (Early Childhood Onset “Occipital” Epilepsy)

    • Seizures: behavioral agitation, headache, autonomic symptoms, and motor (hemi-clonic or GTC), prolonged and nocturnal (2/3).
    • Interictal EEG: occipital spikes in sleep (classic), 85% have boys, frequent absence seizures (pyknolepsy).

    Absence Epilepsy

    • Not fully understood, abnormal oscillatory rhythms are believed to develop in thalamocortical pathways.
    • Treatment: ethosuxamide, lamotrigine, valproic acid, double-blinded RCT compared the 3 and found ethosuxamide provides the best combo of seizure control and fewest attentional side effects.

    Juvenile Absence Epilepsy

    • Onset 10-17 years old, less frequent absence seizures, more associated with GTCs, less severe impairment in consciousness.

    Juvenile Myoclonic Epilepsy

    • Onset 12-18 years old, seizures upon awakening in AM or after nap, triggers: sleep deprivation, stress, fatigue, and alcohol.
    • EEG: interictal high amplitude, generalized 4-6 Hz polyspike and wave, 30% have photosensitivity, 40-50% with family history.

    Genetic Epilepsy and Febrile Seizures Plus (GEFS+)

    • FS after 6 yr old OR occurrence of other seizure types, almost any type of seizure can occur, associated mutations SCN1A, SCN1B, GABRG2.

    Epilepsy Disorders with Pathognomonic EEG Patterns

    • Pathognomonic: of a sign or symptom, specifically characteristic or indicative of a particular disease or condition.
    • Types of pathognomonic EEG patterns:
      • Periodic
      • Rhythmic
      • Other

    Crutzfeldt-Jakob Disease (CJD)

    • Rapidly progressive, invariably fatal neurodegenerative disorder, EEG: generalized periodic discharges, myoclonic jerks, and variations do occur.

    Subacute Sclerosing Panencephalitis (SSPE)

    • Progressive neurological disorder, EEG: generalized periodic discharges of longer interval than CJD, bilaterally synchronous, high-amplitude spike or slow-wave bursts.

    Herpes Encephalitis

    • Rare neurological disorder, EEG: lateralized periodic discharges (LPDs), bihemspheric independent discharges (BiPDs) are sometimes seen.

    Anti-NMDA Receptor Encephalitis

    • Autoimmune encephalitis, EEG: extreme delta brush, often seen with more prolonged illness.

    Angelman Syndrome

    • Neurodevelopmental disorder, EEG: notched delta, small interstitial deletion between 15q11 and 15q13, maternally derived.

    Epilepsy Syndromes

    • Epilepsy syndrome: a group of signs or symptoms that help identify a unique medical condition, characterized by specific signs or symptoms, type of seizure(s), age when seizures start, and pattern on EEG.
    • Types of epilepsy syndromes:
      • Symptomatic/Encephalopathic
      • Progressive and Infantile Myoclonic Epilepsies
      • Focal
      • Idiopathic
      • Febrile Seizure

    Infantile Spasms and West Syndrome

    • Infantile Spasms: flexor, extensor, lightning, or nods—most are mixed, onset 4-8 months, peak 5 mo.
    • West Syndrome: triad of infantile spasms, hypsarrhythmia, and developmental delay, onset 4-7 months, always before 1 year old, boys>girls.
    • Etiology: 75-85% symptomatic, 15-25% asymptomatic, underlying conditions include genetic, metabolic, congenital infection, and neonatal infection.
    • Treatment: steroids, vigabatrin, ketogenic diet, zonisamide, vitamin B6, prognosis partly based on early treatment, but typically poor.

    Lennox-Gastaut Syndrome

    • Triad: multiple seizures types, cognitive dysfunction, and slow GSW (1.5-2 Hz) on EEG.
    • Onset: 1-8 years, typically 3-5 yr, 10-25% cases preceded by infantile spasms.
    • Causes: 70-78% structural/metabolic, 30-22% unknown, family history of epilepsy in 3-30%.
    • Prognosis: overall refractory to ASDs and poor prognosis.

    Severe Myoclonic Epilepsy of Infancy (SMEI)

    • Prolonged febrile seizures in the 1st year of life, seizure-free period followed by myoclonic seizures at 1-4 yr old.
    • Normal early development, then later deterioration, pyramidal signs and ataxia.
    • EEG: slow SW, polyspike-and-wave, 70-80% mutation in SCN1A.

    Myoclonic Astatic Epilepsy (MAE) or Doose Syndrome

    • Type of generalized epilepsy, onset 1-5 years old.
    • EEG: 2-3 Hz gen SW, polyspike and 4-7 Hz central/vertex rhythmic theta activity.
    • MRI: normal, associated with SCN1A mutation, boys>girls (2:1).
    • Prognosis: typically poor with severe intellectual disability, ataxia, poor motor function, dysarthria, and poor language development.

    Epileptic Encephalopathy with Continuous Spike and Wave During Sleep (CSWS)

    • Epileptiform activity occupying >85% of NREM sleep, 2 syndromes: Landau-Kleffner Syndrome (LKS) and CSWS.
    • Landau-Kleffner Syndrome: typically age 3-10 y, sudden or gradual aphasia, verbal auditory agnosia, 2/3rd have seizures, ADHD.
    • CSWS: global regression in cognition and behavior, majority of patients have seizures, may have identifiable pathology.

    Panayiotopolous Syndrome (Early Childhood Onset “Occipital” Epilepsy)

    • Seizures: behavioral agitation, headache, autonomic symptoms, and motor (hemi-clonic or GTC), prolonged and nocturnal (2/3).
    • Interictal EEG: occipital spikes in sleep (classic), 85% have boys, frequent absence seizures (pyknolepsy).

    Absence Epilepsy

    • Not fully understood, abnormal oscillatory rhythms are believed to develop in thalamocortical pathways.
    • Treatment: ethosuxamide, lamotrigine, valproic acid, double-blinded RCT compared the 3 and found ethosuxamide provides the best combo of seizure control and fewest attentional side effects.

    Juvenile Absence Epilepsy

    • Onset 10-17 years old, less frequent absence seizures, more associated with GTCs, less severe impairment in consciousness.

    Juvenile Myoclonic Epilepsy

    • Onset 12-18 years old, seizures upon awakening in AM or after nap, triggers: sleep deprivation, stress, fatigue, and alcohol.
    • EEG: interictal high amplitude, generalized 4-6 Hz polyspike and wave, 30% have photosensitivity, 40-50% with family history.

    Genetic Epilepsy and Febrile Seizures Plus (GEFS+)

    • FS after 6 yr old OR occurrence of other seizure types, almost any type of seizure can occur, associated mutations SCN1A, SCN1B, GABRG2.

    Epilepsy Disorders with Pathognomonic EEG Patterns

    • Pathognomonic: of a sign or symptom, specifically characteristic or indicative of a particular disease or condition.
    • Types of pathognomonic EEG patterns:
      • Periodic
      • Rhythmic
      • Other

    Crutzfeldt-Jakob Disease (CJD)

    • Rapidly progressive, invariably fatal neurodegenerative disorder, EEG: generalized periodic discharges, myoclonic jerks, and variations do occur.

    Subacute Sclerosing Panencephalitis (SSPE)

    • Progressive neurological disorder, EEG: generalized periodic discharges of longer interval than CJD, bilaterally synchronous, high-amplitude spike or slow-wave bursts.

    Herpes Encephalitis

    • Rare neurological disorder, EEG: lateralized periodic discharges (LPDs), bihemspheric independent discharges (BiPDs) are sometimes seen.

    Anti-NMDA Receptor Encephalitis

    • Autoimmune encephalitis, EEG: extreme delta brush, often seen with more prolonged illness.

    Angelman Syndrome

    • Neurodevelopmental disorder, EEG: notched delta, small interstitial deletion between 15q11 and 15q13, maternally derived.

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    Epilepsy Syndromes and Disorders with Pathognomonic EEG Patterns PDF

    Description

    This quiz covers epilepsy syndromes and disorders, including pathognomonic EEG patterns, from pediatric to adulthood. Learn about epilepsy syndromes and their definitions.

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