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Epilepsy and Treatment

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Title: Epilepsy and Treatment


1
Epilepsy and Treatment
  • Anup Patel, M.D.
  • Pediatric Neurologist
  • Capitol Neurology

2
Epilepsy
  • Epilepsy is a treatable condition and
    epileptic patients can live a normal and healthy
    life. Epilepsy should not be a social taboo

3
Definitions
  • Epileptic seizure
  • The clinical manifestations (symptoms and signs)
    of excessive and hyper synchronous, usually self
    limited, activity of neurons in the cerebral
    cortex.
  • Epilepsy
  • A chronic disorder characterized by recurrent
    (more than 2) unprovoked seizures.

4
The ILAE classification of seizures
  • I. Partial (focal, local) seizures
  • A. Simple partial seizures (consciousness not
    impaired)
  • B. Complex partial seizures (with impairment of
    consciousness)
  • C.partial seizures evolving to generalized
    seizures

5
The ILAE classification of epileptic seizures
  • II. Generalized seizures
  • A. Absence seizures
  • 1. Absence seizures
  • 2. Atypical absence seizures
  • B. Myoclonic seizures
  • C. Clonic seizures
  • D. Tonic seizures
  • E. Tonic-clonic seizures
  • F. Atonic seizures (astatic seizures)
  • III. Unclassified seizures

6
The International League Against Epilepsy
classification of epilepsies and epileptic
syndromes
  • I. Localization-related (focal, local, partial)
    epilepsies and syndromes
  • A. Idiopathic (with age-related onset). At
    present, two syndromes are established 1. Benign
    childhood epilepsy with centro temporal spikes
  • 2. Childhood epilepsy with occipital paroxysms
  • B. Symptomatic. This category comprises syndromes
    of great individual variability.

7
The International League Against Epilepsy
classification of epilepsies and epileptic
syndromes
  • II. Generalized epilepsies and syndromes
  • A. Idiopathic (with age-related onset, in order
    of age appearance) 1. Benign neonatal familial
    convulsions 2. Benign neonatal convulsions 3.
    Benign myoclonic epilepsy in infancy 4.
    Childhood absence epilepsy (pyknolepsy, petit
    mal) 5. Juvenile absence epilepsy 6. Juvenile
    myoclonic epilepsy (impulsive petit mal) 7.
    Epilepsy with grand mal seizures on awakening

8
The International League Against Epilepsy
classification of epilepsies and epileptic
syndromes
  • II. Generalized epilepsies and syndromes
  • B. Idiopathic, symptomatic, or both (in order of
    age of appearance) 1. Infantile Spasms
  • 2. Lennox Gastaux
  • 3. Epilepsy with myoclonic-astatic seizures
  • 4. Epilepsy with myoclonic absences

9
The International League Against Epilepsy
classification of epilepsies and epileptic
syndromes
  • C. Symptomatic 1. Nonspecific cause, early
    myoclonic encephalopathy
  • 2. Specific syndromes. Epileptic seizures may
    complicate many disease states. Under this
    heading are included those diseases in which
    seizures are a presenting or predominant feature.

10
The International League Against Epilepsy
classification of epilepsies and epileptic
syndromes
  • III. Epilepsies and syndromes undetermined as to
    whether they are focal or generalized A. With
    both generalized and focal seizures 1.Neonatal
    seizures 2. Severe myoclonic epilepsy in
    infancy 3. Epilepsy with continuous spikes and
    waves during slow-wave sleep
  • 4. Acquired epileptic aphasia (Landau-Kleffner
    syndrome)
  • B. Without unequivocal generalized or focal
    features

11
The International League Against Epilepsy
classification of epilepsies and epileptic
syndromes
  • IV. Special syndromes A. Situation-related
    seizures 1. Febrile convulsions 2. Seizures
    related to other identifiable situations, such as
    stress, hormones, drugs, alcohol, or sleep
    deprivation B. Isolated, apparently unprovoked
    epileptic events C. Epilepsies characterized by
    the specific modes of seizures precipitated D.
    Chronic progressive epilepsia partialis continua
    of childhood

12
Seizure precipitants
  • Stress, emotion
  • Sleep/sleep deprivation
  • Hyperventilation
  • Fever
  • Medications, metabolic disturbance
  • Reflex epilepsy
  • Photic stimuli TV, flashing lights, visual
    patterns
  • Startle, music, reading, eating

13
Generalized Absence (GA) vs. Complex partial (CP)
seizures
14
Workup of a first unexplained seizure.
  • EEG
  • MRI brain
  • 1 Unexplained seizure does not necessitate AED
    treatment except
  • Recognized epileptic syndrome with high
    probability of recurrence.
  • Focal brain lesion.

15
EEG yield
  • 1st EEG 50
  • With repeated EEG and activation procedures the
    yield can go up to 90
  • No benefit after the fourth EEG, as it gives
    maximum yield

16
Treat or not to Treat
  • The risk of recurrence of seizures is about
    30-35 after the first unprovoked seizure
  • The risk of recurrence is about 60 after second
    seizure

17
Drug Therapy basic principles
  • Use a single drug whenever possible.
  • However, remember that roughly 60 of patients
    are controlled on monotherapy.
  • Start low and go slow
  • Increase the dose of that drug to either seizure
    control or toxicity (decreasing the dose if
    toxicity occurs).
  • If a drug does not control seizures without
    toxicity, switch to another appropriate drug used
    alone, and again increase the dose until seizure
    control occurs or toxicity intervenes.

18
Drug therapy
  • Partial and Secondarily
  • Generalized Seizures
  • Carbamazepine, phenytoin, and valproic acid are
    the first-line agents among most specialists for
    partial and secondarily generalized seizures
  • Gabapentin, lamotrigine, oxcarbazepine,
    tiagabine, topiramate, and levetiracetam are new
    anticonvulsants that are recommended for
    treatment of partial seizures.

19
Generalized seizures
  • Primary Generalized Seizures
  • Ethosuximide and valproic acid are effective for
    treating absence seizures, but ethosuximide is
    not effective for treatment of primary
    generalized tonic-clonic seizures.
  • lamotrigine
  • felbamate
  • zonisamide
  • topiramate
  • levetiracetam

20
Ketogenic diet
  • Ketosis improves seizure control
  • The basic protocol calls for a diet with a
    fat-tocarbohydrate-plus-protein ratio of 4 to 1
    on a caloric basis. A modification of the diet
    uses medium-chain triglyceride (MCT) oil and
    allows for a greater amount of carbohydrate. The
    MCT oil diet is not clearly more beneficial, nor
    is it better tolerated.
  • beneficial in a subset of patients who have not
    responded to antiepileptic drugs.

21
Common Pediatric Epilepsy Syndromes
  • Absence Epilepsy
  • Juvenile Myoclonic Epilepsy
  • Benign Rolandic Epilepsy
  • Infantile Spasms
  • Lennox Gastaux

22
Absence Seizures
  • age of onset 3-8 years
  • abrupt cessation of activity with change of
    facial expression and blank gaze
  • duration short usually lt 15 seconds
  • child returns to normal and no postictal period
  • automatisms sometimes
  • activated by hyperventilation
  • characteristic EEG 3 Hz spike wave
  • treat with AEDs (Ethosuxsimide, Valproate,
    Topamax, and Lamictal)
  • patients usually grow out of seizures by teen
    years

23
VPA- Absence Seizures
24
Absence Seizures
3 Hz Spike Wave
25
What is JME?
  • Also called Janz syndrome
  • First described in 1867
  • Triad includes myoclonic jerks, absence, tonic
    clonic seizures
  • Normal development
  • Normal imaging

26
What is JME?
  • A common epilepsy syndrome 10-15 of all
    epilepsies
  • Age of onset 12-18 years
  • FM
  • Accounts for 25 of patients with idiopathic
    generalized epilepsies.
  • Most have myoclonic jerks, 85 have GTCs, and
    15-38 have absence

27
Juvenile Myoclonic Epilepsy
  • EEG with 3-6 Hz multispike and wave
  • Photosensitivity in 27-41
  • Focal EEG abnormalities in up to 55
  • Triggers AM wakening, lack of sleep, fatigue,
    ETOH, and fasting
  • Requires life-long treatment
  • Little data on effective treatment

28
Treating JME
  • Depakote
  • Keppra
  • Zonegran
  • Topamax
  • Lamictal

29
Juvenile Myoclonic Epilepsy
30
Benign Rolandic Epilepsy
  • autonomic dominant
  • onset 3-13yrs with peak 6-8 years
  • usually nocturnal or during sleep
  • infrequent episodes that awake the child with
    drooling, speech arrest, ipsilateral facial
    twitching or twisted to one side that are only
    minutes in duration
  • can sometimes generalize
  • development and exam are normal
  • characteristic EEG that shows Midtemporal (T3,T4)
    and Central (C3,C4) spikes
  • treatment usually not indicated if infrequent but
    can treat with AEDs
  • usually outgrown by 14 years

31
Benign Rolandic Epilepsy
32
Benign Occipital Epilepsy
  • onset 15mos-15years, usually 4-8 years
  • initial seizure manifestations include visual
    hallucinations (flashing lights), blindness,
    amaurosis, micropsia, metamorphopsia,
  • loss of consciousness can occur
  • can have migraine and nausea afterward
  • different seizure types (GTC, CPS, unilateral
    clonic) and occur mostly when transitioning from
    wakefulness to sleep
  • EEG shows occipital spike wave 1.5-2.5 Hz and
    eye opening enhances and sleep inhibits

33
Infantile Spasms (IS)
  • specific type of seizure seen in infancy and
    early childhood
  • onset is predominantly in the first year of life,
    typically lt 1 year
  • characteristic EEG called hypsarrhythmia
  • typical pattern is a sudden bending forward and
    stiffening of the body, arms, and legs. Although
    there can also be arching of the torso.

34
Infantile Spasms (IS)
  • Spasms tend to begin soon after arousal from
    sleep. Individual spasms typically last for 1 to
    5 seconds and occur in clusters, ranging from 2
    to 100 spasms at a time.
  • Infantile spasms usually stop by age 5, but are
    often replaced by other seizure types.
  • West Syndrome is characterized by infantile
    spasms, hypsarrhythmia, and mental retardation.

35
Infantile Spasms (IS)
  • Etiology
  • Cerebral malformations 35, Perinatal insult 15,
    Metabolic 15,
  • Tuberous Sclerosis 10
  • Treatment usually starts with AEDs, steroids,
    ACTH, Vigabatrin, B6, Surgery (if lesions)
  • Prognosis depends on etiology. Worse prognosis
    with symptomatic as many, 50, go on to have
    other types of seizures
  • Many develop mental retardation or delayed
    development.

36
Infantile Spasms (IS)
37
Lennox Gastaut Syndrome
  • Childhood epileptic encephalopathy
    (Lennox-Gastaut syndrome LGS) is a devastating
    pediatric epilepsy syndrome
  • constituting 1-4 of childhood epilepsies
  • triad characterized by multiple types of
    seizures, mental retardation or regression, and
    characteristic EEG
  • abnormal EEG with generalized slow spike-and-wave
    discharges (1.5-2 Hz)

38
Lennox Gastaut Syndrome
  • most common seizure types are tonic-axial,
    atonic, and absence seizures, but myoclonic,
    generalized tonic-clonic, and partial seizures
    can be observed. Seizures often are resistant to
    therapy.
  • mean age at epilepsy onset is 3-5 years (range,
    1 d to 14 y)
  • 60 have underlying cause (TS, NF, perinatal
    insult) and 20 have history of Infantile Spasms
  • diagnosis by history, PE, and EEG
  • treatment is difficult

39
Lennox Gastaut Syndrome
40
Acquired Epileptiform Aphasia
  • Landau-Kleffner Syndrome
  • onset 2-12 years
  • acquired aphasia, verbal auditory agnosia,
    decreased spontaneous speech
  • difficulty understanding speech and child stops
    talking
  • several seizure types (GTC, Myoclonic, Absence)
  • neuropsychological disturbances in gt50 but
    intelligence is not affected

41
Acquired Epileptiform Aphasia
  • sometimes the child is diagnosed with Autism or
    being deaf
  • EEG is normal during wakefulness but during sleep
    there is spike wave mostly in parietal and
    temporal lobes, sometimes electrical status of
    sleep
  • treatment with AEDs and steroids shows good
    control
  • recovery of language is variable and if onset is
    before 6 years there is better outcome
  • less than 50 live independent lives

42
Landau-Kleffner Syndrome

Landau-Kleffner EEG Shows SW
43
Landau-Kleffner Syndrome
44
Epilepsy with Continuous Spike Waves During Slow
Wave Sleep (CSWS)
  • also called electrical status epilepticus of
    sleep
  • various seizure types occur during sleep
  • EEG shows continuous diffuse spike wave during
    slow wave sleep
  • prognosis guarded because of neuropsychological
    disturbance and intellectual regression
  • treated with AEDs and steroids

45

Autosomal Dominant Nocturnal Frontal Epilepsy
  • chromosome 20 q
  • bizarre behavior and motor symptoms during sleep
  • seizures begin in childhood and persists in
    adulthood and can come in clusters
  • most attacks occur when dozing or initiating
    sleep and can occur in clusters and a gasp or
    grunt will awake the child

46
Doose Syndrome
  • Myoclonic-Astatic Epilepsy (MAE)
  • Is often resistant to medication
  • Is an idiopathic generalized epilepsy and the
    seizures are generalized and different types
  • Onset of MAE occurs commonly between the first
    and fifth year of life, with the mean age being
    three.
  • Statistics show that it usually affects children
    who have previously developed normally, and boys
    are twice as likely as girls to develop MAE,
    other family members (immediate or extended) may
    also have epilepsy.
  • Treatment with AEDs

47
Jeavons Syndrome (Eyelid Myoclonia with Absences)
  • Eyelid myoclonia with absences has two
    components. The initial and more prominent is
    eyelid myoclonia. This may or may not progress to
    the second component, which is mild impairment of
    consciousness (absence). The seizure starts and
    ends abruptly with a duration of 3 to 5 seconds.
  • The patient exhibits eyelid myoclonia with
    absences mainly on eye-closure and intermittent
    photic stimulation.
  • These do not occur in the dark.

48
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