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</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</p> Signup and view all the answers

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

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

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

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

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

    <p>SCN1A</p> Signup and view all the answers

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

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

    What is the recurrence risk of febrile seizures?

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

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

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

    What is the age of onset for Infantile Spasms?

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

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

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

    What is the most common type of epilepsy syndrome?

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

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

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

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

    <p>Ethosuxamide</p> Signup and view all the answers

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

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

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

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

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

    <p>Steroids</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</p> Signup and view all the answers

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

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

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

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

    What is the typical genetic abnormality associated with Angelman Syndrome?

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

    What is the characteristic EEG pattern in Angelman Syndrome?

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

    Which of the following is a common feature of SSPE?

    <p>Clinical myoclonus</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</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%</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</p> Signup and view all the answers

    What is the age range of onset for Infantile Spasms?

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

    What is the triad associated with West Syndrome?

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

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

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

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

    <p>Steroids</p> Signup and view all the answers

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

    <p>Ethosuximide</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</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</p> Signup and view all the answers

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

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

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

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

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

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

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

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

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

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

    What is the characteristic EEG pattern associated with Angelman Syndrome?

    <p>Notched delta</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</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</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</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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    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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