Epilepsy Syndromes

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Epilepsy Syndromes
2012-09-07 06:07:45
Epilepsy Syndromes

Common Epilepsy Syndromes and Seizure Types
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  1. What are the characteristics of Atypical Absence Seizures?
    • Slower frequency of spike / wave dischages (1.5-2.5 Hz)
    • Last longer than typical absence seizures
    • Most commonly seen in children with developmental delay or other neurologic deficits
  2. Define Symptomatic Epilepsy.
    Seizures that result from a demonstrable structural lesion or known neurologic problem (e.g. TBI, meningitis, other neurologic disorder).
  3. Define Idiopathic Epilepsy.
    Seizures that are presumed to be of genetic origin in a patient with normal cognitive funciton, MRI, and exam.
  4. Define Cryptogenic Epilepsy.
    Seizures that are thought to be symptomatic but with an unknown underlying cause.
  5. What is an Epilepsy Syndrome?
    A constellation of specific seizure type(s) which occur in a specific patient profile with an identifiable EEG pattern.
  6. What EEG pattern is demonstrated here and what disorder is it associated with?
    Hypsarrhythmia characterized by asynchronous, chaotic, high amplitude slow activity with superimposed multifocal spikes.

    Infantile Spasms and West Syndrome
  7. What are the cardinal features of West Syndrome
    • Hypsarrhythmia
    • Infantile Spasms
    • Developmental Delay
  8. What are the cardinal fatures of Lennox-Gastaut Syndrome?
    • Two or more seizure types (tonic, atonic, atypical absence)
    • EEG showing slow spike and wave (1.5 - 2.5 Hz)
    • Developmental Delay

    Normally starts at 1-8 years of age and is often the natural progression of West Syndrome where it is preceeded by Infantile Spasms
  9. The EEG depicted here is most consistent with which epilepsy syndrome?
    Lennox-Gastaut Syndrtome
  10. What are the cardinal features of Landau-Kleffner Syndrome?
    • a.k.a. Acquired Epileptic Aphasia
    • Seen in children 3 - 7 years old
    • Patients develop word deafness with a receptive > expressive aphasia characterized by a delince in speech
    • EEG will show epileptiform dischages in the temporal and parietal regions and when performed during sleep may show Electrical Status Epilepticus of Sleep (ESES)
    • Treated with AED's like Keppra and Steroids
  11. What are the characteristic features of Childhood Absence Epilepsy (CAE)?
    • Generalized epilepsy syndrome
    • Chioldren 4 - 8 years old
    • Characterized by brief staring spells
    • EEG showed 3 Hz spike and wave discharges
    • Treated with Ethosuxamide, Depakote, or Lamictal
  12. The following EEG is most consistent with which epilepsy syndrome?
    Typical Absence Seizure demonstrating 3 Hz spike and wave discharges with a bifrontal predominance.
  13. What are the characteristic features of Juvenile Myoclonic Epilepsy (JME)?
    Generalized seizres starting at 13-20 years of age which may be provoked by sleep deprivation or drinking.

    Often associated with myoclonic jerks and absences which start earlier

    EEG shows fast (3.5 - 4.5 Hz) spike / polyspike and wave activity.
  14. The EEG depicted here would be most consistent with what epilepsy syndrome?
    Juvenile Myoclonic Epilepsy
  15. What EEG phenomenon is depicted in Panel B?
    Photoparoxysmal response in patient with generalized epilepsy
  16. What are the characteristic features of Benign Rolandic Epilepsy (BRE)?
    • The most common form of benign partial epilepsy in kids.
    • Presents 4 - 12 years old.
    • Noccturnal GTC's and simple partial motor seizures afffecting the face assocciated with drooling and speech arrest.
    • EEG shows centrotemporal spikes.
  17. The EEG depicted here would be most consistent with what epilepsy syndrome?
    Benign Rolandic Epilepsy
  18. What are the characteristic features of frontal lobe epilepsy?
    • Seizures that most often occur at night.
    • Often occur multiple times a night.
    • Variable semiology.
    • Brief with a rapid recovery.
    • Bizarre, hyperkinetic movements (e.g. pelvic thrusting and frenetic activity).
  19. What are the characteristic features of Panayiotopoulus Syndrome?
    • Onset between 2-8 years of age.
    • Complex partial seizures that tend to occur at night, be prolonged, and associated with autonomic features like emesis.
    • May be followed by hemiclonic or generalized seizure activity.
  20. What are the characteristic features of Gastaut-Type Epilpepsy?
    • Age of onset in the later half of the first decade.
    • Seizures characterized by visual symptoms which often generalize.
    • Seizures may be triggered by photic stimulation and followed by migrainous headaches.
  21. What are the five proggressive myoclonic epilepsies?
    • Unverricht-Lundborg (EPM 1)
    • Lafora Disease (EPM 2A and 2B)
    • Neuronal Ceroid Lipofuscinoses (NCL)
    • Sialidoses
    • Myoclonicc Epilepsy with Ragged-Red Fibers
  22. What are the characteristic features of Unverrichect-Lundborg Disease?
    • Progressive myoclonic epilepsy.
    • Starts at 6-18 years of age with stimulus-induced myoclonus with later onset cerebellar degeneration.
    • Defect in Cystatin B on Chromosome 21q22.3
    • Diagnosed by skin biopsy from the axillary eccrine glands.
  23. What are the characteristic features of Lafora Body Disease?
    • One of the Progressive Myoclonic Epilepsies
    • Rapidly progressive
    • Associated with dementia
    • Diagnosed by skin biopsy which demonstrates polglucosanj (Lafora) bodies in the eccrine sweat glands.
  24. What are the clinical features of GEFS+ (Generalized Epilepsy with Febrile Seizures Plus)?
    • Autosomal dominant disorder with variable penetrance and phenotypic expression
    • Caused by a mutation in either SCN1A or GABA1
    • Febrile seizures which either persisit beyond 6 years of age or are associated with afebrile, generalized seizures