Transcript Epilepsy and Treatment - The Indiana Society of

Epilepsy
and Treatment
Anup Patel, M.D.
Pediatric Neurologist
Capitol Neurology
Epilepsy
Epilepsy is a treatable
condition and epileptic
patients can live a normal and
healthy life. Epilepsy should
not be a social taboo
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.
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
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
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.
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
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
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.
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 (LandauKleffner syndrome)
B. Without unequivocal generalized or
focal features
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
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
Generalized Absence (GA) vs.
Complex partial (CP) seizures
Gen. Absences
CPS
Aura
-
+/-
Onset
Abrupt
Gradual or
abrupt
Duration
<15 sec
>30 sec
Termination
Abrupt
Usually
Gradual
Postictal S & S
-
Most often +
Frequency
Many daily
Weekly-monthly
PPT by HV
Usually
Unlikely
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.
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
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
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.
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.
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 tonicclonic seizures.
• lamotrigine
• felbamate
• zonisamide
• topiramate
• levetiracetam
Ketogenic diet
• Ketosis improves seizure control
• The basic protocol calls for a diet with a
fat-to–carbohydrate-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.
Common Pediatric Epilepsy
Syndromes
• Absence Epilepsy
• Juvenile Myoclonic Epilepsy
• Benign Rolandic Epilepsy
• Infantile Spasms
• Lennox Gastaux
Absence Seizures
• age of onset 3-8 years
• abrupt cessation of activity with change
of facial expression and blank gaze
• duration short usually < 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
VPA- Absence Seizures
Absence Seizures
3 Hz Spike &
Wave
What is JME?
• Also called Janz syndrome
• First described in 1867
• Triad includes myoclonic
jerks, absence, & tonic clonic
seizures
• Normal development
• Normal imaging
What is JME?
• A common epilepsy syndrome: 1015% of all epilepsies
• Age of onset 12-18 years
• F=M
• Accounts for 25% of patients with
idiopathic generalized epilepsies.
• Most have myoclonic jerks, 85%
have GTC’s, and 15-38% have
absence
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
Treating JME
• Depakote
• Keppra
• Zonegran
• Topamax
• Lamictal
Juvenile Myoclonic Epilepsy
Benign Rolandic Epilepsy
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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
Benign Rolandic Epilepsy
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
Infantile Spasms (IS)
• specific type of seizure seen in infancy
and early childhood
• onset is predominantly in the first year
of life, typically < 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.
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.
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.
Infantile Spasms (IS)
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)
Lennox Gastaut Syndrome
• most common seizure types are tonicaxial, 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
Lennox Gastaut Syndrome
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 >50% but intelligence is not
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
Landau-Kleffner Syndrome
LandauKleffner
EEG Shows
S&W
Landau-Kleffner
Syndrome
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
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
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
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.
Thank you!