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Epilepsy Syndromes

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Epilepsy Syndromes Maja Ilic, MD Epileptologist Northeast Regional Epilepsy Group What is seizure and what is epilepsy? SEIZURE - Physiological: Manifestation of an ... – PowerPoint PPT presentation

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Title: Epilepsy Syndromes


1
Epilepsy Syndromes
  • Maja Ilic, MD
  • Epileptologist
  • Northeast Regional Epilepsy Group

2
What is seizure and what is epilepsy?
  • SEIZURE
  • - Physiological Manifestation of an abnormal
    and excessive synchronized discharge of a set of
    neurons
  • - Clinical Stereotypical, usually unprovoked,
    disturbance of consciousness, behavior, emotion,
    motor function or sensation as a result of the
    cortical neuronal discharge
  • EPILEPSY 2 or more seizures

3
  • Provoked seizures
  • High fever, febrile seizures
  • Massive sleep deprivation
  • Excessive use of stimulants
  • Withdrawal from sedative drugs or alcohol
  • Electrolyte disturbance
  • Hypoxia
  • Acute neurological illness

4
Epidemiology
  • gt 2 million people in US
  • 125,000 new cases per year
  • Cumulative incidence of epilepsy by age 80 3.1
  • Cumulative incidence of seizures by age 80 11
  • Many patients outgrow their epilepsy

5
Historical background
  • In an attempt to encompass a broader range of
    clinical features than is possible in a
    classification of seizure type
  • ILAE published a Classifications of Epilepsies
    and Epileptic Syndromes in 1985 and revised it in
    1989

6
Epilepsy syndromes
  • Syndrome is a cluster of signs and symptoms that
    occur together but unlike a disease do not have a
    single known cause or pathology.
  • Epileptic syndrome integrates all data, seizure
    type, EEG findings, age of onset, cause, family
    history, imaging studies, precipitating factors,
    etc. in order to make a final diagnosis of
    epilepsy.

7
Most Common Epilepsy Syndromes in Children
  • Childhood Absence Epilepsy
  • Juvenile Absence Epilepsy
  • Juvenile Myoclonic Epilepsy
  • Benign Epilepsy of Childhood with Centrotemporal
    Spikes
  • (Benign Rolandic Epilepsy)

8
Childhood Absence Epilepsy
9
Absence Epilepsy
  • Generalized seizures
  • Most common in the first decade, particularly
    ages 5-7 years of age

10
  • Clinical features
  • Usual duration 10 seconds
  • Ongoing activities cease
  • Motionless with a fixed blank stare
  • Attack ends suddenly, activity resumes
  • Clinical features
  • Occasionally mild clonic activity of
    eyelids, corner of mouth
  • Automatisms elevation of eyelids, licking,
    swallowing, scratching movements of hands

11
Absence Epilepsy
  • Most patients with typical absence have normal
    development and normal neurological exam
  • Generalized spikes on EEG
  • Precipitated by hyperventilation in all untreated
    patients
  • - Photic stimulation 15

12
Absence Epilepsy
  • 10 family history
  • Average age when seizures stop is 10 years old
  • Generally have a good prognosis
  • resolves in 80 percent of cases

13
  • Treatment
  • Ethosuximide
  • Depakote
  • Suppress absence in 80
  • Lamotrigine

14
Absence Epilepsy
  • Atypical
  • Complex automatisms
  • Slower, 2.5 Hz spike-slow wave
  • Focal and GTC seizures
  • Developmental delay
  • Abnormal background EEG
  • Typical
  • Simple automatisms
  • 3 Hz spike-slow wave
  • No other seizure type
  • Normal exam
  • Normal background EEG

15
  • Juvenile Absence Epilepsy

16
Juvenile Absence Epilepsy
  • Age of onset
  • Near or after puberty
  • Between 10-17 years
  • Normal intelligence neurological exam

17
Juvenile Absence Epilepsy
  • Seizures types
  • Absences
  • Generalized tonic-clonic seizures- in 80
  • (often shortly after waking)
  • Myoclonic seizures- in 15
  • GTC and myoclonic seizure more common and most
    likely to happen with awakening

18
Juvenile Absence Epilepsy
  • EEG
  • Normal background
  • Generalized spike wave discharges (faster, 4
    Hz)
  • Induced by HV, not photic stimulation
  • Treatment
  • Depakote
  • Lamictal

19
Juvenile Absence Epilepsy
  • Prognosis
  • At least 80 of patients can be treated with
    Depakote alone
  • Absences and GTC usually respond well to
    pharmacotherapy
  • Unlike CAE (in which most patients become seizure
    free) the long term evolution of JAE has not been
    properly characterized

20
Juvenile Myoclonic Epilepsy
21
Juvenile Myoclonic Epilepsy
  • Incidence
  • 10 of all epilepsies
  • Age of onset
  • 1218 years
  • Age of onset differs from age of diagnosis

22
Juvenile Myoclonic Epilepsy
  • Myoclonic jerks, generalized tonic-clonic
    seizures, and sometimes absence seizures
  • Myoclonic seizures
  • Jerks of neck, shoulder, arm or leg extensors
  • Usually bilaterally symmetric synchronous
  • More in upper extremities
  • Drop objects, interfere in morning activities

23
Juvenile Myoclonic Epilepsy
  • Usual age at onset
  • - Absence seizures is 7 -13 years
  • - Myoclonic jerks, 12- 18 years
  • - Generalized convulsions, 13 - 20 years

24
Juvenile Myoclonic Epilepsy
  • More likely to have seizures with sleep
    deprivation and alcohol ingestion
  • Risk for seizures is lifelong
  • Seizures recur when AEDs withdrawn
  • Photic stimulation often provokes a discharge
  • Seizures are usually well-controlled (Depakote,
    Lamictal)

25
Benign Epilepsy of Childhood with Centrotemporal
Spikes Benign Rolandic Epilepsy
26
BECT
  • Age of onset
  • Range 2 13 years
  • 80 between 5 10 years (Peak 9 years)
  • Typically resolves by age 16 years
  • Normal intelligence and neurological exam
  • Seizures usually happen after falling asleep or
    before awakening (75)

27
BECT
  • Most characteristic symptoms
  • Sensorimotor phenomena of face
  • Oropharyngeal Hypersalivation, guttural sounds,
    contraction of jaw, difficulty moving the tongue
    etc
  • Speech arrest
  • Clonic jerks at corner of mouth
  • Clonic jerks of one arm
  • No loss of consciousness
  • Can have secondarily generalized convulsions

28
BECT
  • EEG Spikes in midtemporal and central head
    region
  • More spikes in drowsiness and sleep
  • 30 of cases show spikes only during sleep

29
BECT
  • BECTS spontaneously stop with or without
    treatment (good prognosis)
  • If seizures are frequent and/or disturbing to
    patient and family, treatment with Tegretol or
    Trileptal (until 14-16, response)
  • AEDs given in about 50 of cases
  • Features suggesting risk of repeated seizures
  • Short interval between 1st 2nd attacks
  • Early onset

30
Febrile Seizures
  • Up to 4 of children
  • Not epilepsy
  • Often a family history, 10
  • (chromosomes 8q, 19p)
  • Seizures only occur with fever in children age 6
    months 6 years

31
Febrile Seizures
  • Simple
  • - 1 brief seizure (generalized)
  • Complex
  • Prolonged
  • More than 1
  • Focal

32
Febrile seizures
  • 13 incidence of epilepsy if at least 2 factors
  • History of non-febrile seizure
  • Abnormal neurological exam or development
  • Prolonged febrile seizure
  • Focal febrile seizure

33
Febrile seizures
  • Recurrence risk
  • Children with simple FS 30
  • Children with complex FS 50
  • Risk of epilepsy
  • - With history of simple FS 2-4
  • - With history of complex FS 6

34
Febrile Seizures
  • Increase risk of recurrence if 1st before 18
    months or lower temperature
  • Focal need MRI, EEG
  • Testing unnecessary with simple
  • Treatment usually not necessary

35
Epilepsy Prognosis
  • Depends on seizure type
  • Typical Absence 80 resolve
  • JME- respond well to treatment but need meds for
    life
  • Neurologically abnormal often difficult to
    control seizures

36
Drug Refractoriness of Different Seizure Types
  • Idiopathic partial 0-2
  • Childhood absence 10-30
  • Juvenile absence 10-35
  • Primary GTCS 20-30
  • Secondary GTCS 30-60
  • CPS 40-60
  • LGS 60-80
  • Infantile spasms 60-80

37
  • Most patients (gt70) will have excellent seizure
    control with medications
  • Some patients will continue to have seizures
    despite good medical therapy
  • Ketogenic diet
  • Vagal nerve stimulator
  • Epilepsy surgery

38
Treatment Goals
  • Prevent recurrence of seizures
  • Avoid side effects from AEDs
  • Attain therapeutic levels
  • Ensure compliance

39
General Guidelines for Use of AEDs
  • Select AED specific for seizure type and EEG
    findings
  • Start with single drug
  • Optimize AED
  • Balance seizure control vs. toxicity
  • Add second drug if first fails
  • Anticipate medication interactions

40
When to Treat After Single Seizure?
  • Definitely
  • With structural lesion
  • Brain tumor
  • Arteriovenous malformation
  • Infection, such as abscess, herpes encephalitis
  • Without structural lesion
  • EEG with definite epileptic pattern
  • History of previous seizure
  • History of previous brain injury
  • Status epilepticus at onset
  • Possibly
  • Unprovoked seizure
  • Probably not (although short-term therapy may be
    used)
  • Alcohol withdrawal
  • Drug abuse
  • Seizure in context of acute illness
  • Postimpact seizure
  • Specific benign epilepsy syndrome
  • Seizure provoked by excessive sleep deprivation

41
Medications
  • Trileptal, Tegretol, Keppra, Depakote, Lamictal,
    Phenobarbital,Topamax, Ethosuximide
  • Choice based on type of seizures, EEG findings,
    side effects, age and sex
  • 2nd AED may be added if seizures not controlled

42
Newer antiepiletic medications
  • Lacosamide (Vimpat)
  • Vigabatrin (Sabril)
  • Rufinamide (Banzel)
  • Perampanel (Fycompa)

43
Conclusion
  • Seizure type and diagnosis are only one element
    of a more comprehensive patient assessment that
    should result in a precise epilepsy syndrome
    diagnosis
  • Only an accurate diagnosis of a specific epilepsy
    syndrome allows patients and physicians to
    examine all treatment options

44
  • www.epilepsyfoundation.org
  • www.epilepsyadvocate.com
  • www.paceusa.org
  • www.epilepsy.com
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