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EPILEPSY

By dr. Yustiani Dikot, Sp.S(K)


DEFINITION

Abnormal and recurrent excessive


synchronized discharge of cerebral
neuron with clinical manifestation of
epileptic seizure which are an
intermittent stereotypical behavior,
emotion, motor function or sensation
PATHOPHYSIOLOGY
• Paroxysmal depolarization shift (PDS) of the
resting membrane potential, which triggers a
brief rapid burst of action potentials terminated
by a sustained after hyperpolarization
• PDS : result of imbalance between excitatory
(glutamate and aspartate) and inhibitory
(GABA) neurotransmitters
• Abnormalities of voltage controlled membrane
ion channels
• Imbalance between endogenous
neuromodulators, acetylcholine favoring
depolarization and dopamine enhancing
neuronal membrane stability
FOCAL EPILEPTOGENESIS
• Asynchronous burst firing in some
hypocampal and cortical neurons
• Generalized epileptogenesis :
asynchronous burst firing in abnormal
thalamocortical interaction
EPIDEMIOLOGY

• Developed countries :
annual incidence 50-70 cases per
100.000
• Developing countries : prevalence 1%
Incidence varies with age
Incidence of epilepsy in relation to age
ETIOLOGY

• Idiopathic
• Cryptogenic
• Symptomatic
Congenital anomalies
Tuberous sclerosis
Storage diseases

Birth trauma Genetic epilepsies Cerebral tumours

Intracranial
haemorrhage Intracranial Head Injuries
Infections

Febrile
Seizures
Drugs Cerebrovascular
Hypoxia and degenerations
Hypoglycaemia alcohol
Hypocalcaemia

0 1 5 10 20 60
Age (years)
Factors lowering seizure threshold
Common Occasional
•Sleep deprivation •Barbiturate withdrawal
•Alcohol withdrawal •Dehydration
•Television flicker •Benzodiazepine
•Epileptogenic drugs withdrawal
•Systemic infection
•Hyperventilation
•Head trauma
•Flashing lights
•Recreational drugs
•Diet and missed meals
•AED non-compliance
•Menstruation
•Specific “reflex” triggers
•Stress
•Intense exercise
International Classification of Epileptic Seizures
Partial seizures (beginning locally)
• Simple partial seizures (without impaired
consciousness)
– with motor symptoms
– with somatosensory or special sensory symptoms
• Complex partial seizures (with impaired
consciousness)
– simple partial onset followed by impaired consciousness
– impaired consciousness at onset
• Partial seizures evolving into secondary generalized
seizures
Generalized seizures (convulsive or non-
convulsive)
• Absence seizures
– Typical
– Atypical
• Myoclonic seizures
• Clonic seizures
• Tonic seizures
• Tonic clonic seizures
• Atonic seizures
• Unclassified seizures
Simplified Classification of Epileptic Seizures
Partial seizures
• Simple – preservation of awarness
• Complex – impairment of consciousnesss
• Secondary generalized
Generalized seizures
• Absence
• Myoclonic
• Tonic-clonic
• Tonic
• Atonic
International Classification of Epilepsies
and Epileptic Syndrome

Localization-related (focal, local or partial)


epilepsies and syndromes
• Idiopathic epilepsy with age-related onset
- benign childhood epilepsy with
centrotemporal spikes
- chilhood epilepsy with occipital paroxysms
• Symptomatic epilepsy
Generalized epilepsies and syndromes
• Idiopathic epilepsy with age-related onset (listed
in order of age at onset)
- benign neonatal familial convulsions
- benign neonatal non-familial convulsions
- benign myoclonic epilepsy in infancy
- childhood absence epilepsy (formerly known as
pyknolepsy)
- juvenile absence epilepsy
- juvenile myoclonic epilepsy (formerly known as
impulsive petit mal)
- epilepsy with generalized tonic-clonic seizures
on awaking
• Other idiopathic epilepsies
• Idiopathic or symptomatic epilepsy (listed in
order of age at onset)
- West syndrome (infantile spasms)
- Lennox-Gastaut syndrome (childhood epileptic
encephalopathy)
- epilepsy with myoclonic-astatic seizures
- epilepsy with myoclonic absence seizures
• Symptomatic epilepsy
• Non-specific syndromes
- early myoclonic encephalopathy
- early infantile epileptic encephalopathy
• Specific syndromes (epileptic seizures as a
complication of a disease, such as
phenylketonuria, juvenile Gaucher’s disease or
Lundborg’s progressive myoclonic epilepsy)
Epilepsies and syndromes with both
generalized and focal seizures
• Neonatal seizures
• Severe myoclonic epilepsy in infancy
• Epilepsy with continuous spike waves
during slow-wave sleep
• Acquired epileptic aphasia (Landau-
Kleffner syndrome)
Epilepsies without unequivocal generalized or
focal features
Special syndromes
• Situation-related seizures
- febrile convulsions
- seizures related to other identifiable situations,
such as stress, hormonal changes, drugs,
alcohol withdrawal or sleep deprivation
• Isolated, apparently unprovoked epileptic
events
• Epilepsies characterized by specific modes of
seizure precipitation
• Chronic progressive epilepsia partialis
continua of childhood
Diagnosis
• Interviews with patients or witness
– Circumstances surrounding the attacks
idiopathic and generalized
No seizure worning
No underlying brain lesions
Associated with a family history
– Symptomatic and localization related
Aura
Specific site of onset
Identifiable cause
– Recurrent episodes of seizures
– Symptoms occured during and after seizures
• Recording symptomatic events with videocamera
and continous ambulatory EEG monitoring
EEG
• To confirm the clinical diagnosis
• To support the classification of partial or
generalized seizures
• Routine trace 50% normal
• Diagnostic in non convulsion state
epileptic activities :
Hyperventilation
Photic stimulations
Sleep deprivation
EEG
BRAIN IMAGING

• Essential, particularly in partial onset


seizures
Computerized tomography (CT)
Magnetic resonance imaging (MRI)

Structural lesion
MRI
MRI
MRI
Scan
Scan should be repeated periodically :
• Suspicion of a tumour
• Worsening in neurological examination
or cognitive function
• Deterioration in the frequency or
severity of the seizures
Single Photon Emission CT (SPECT)
Positron Emission Tomography (PET)
MRI spectroscopy
Functional MRI

Functional cerebral changes


Useful adjuncts in candidate epileptic
surgery
DIFFERENTIAL DIAGNOSIS
• Migraine
• Transient Ischaemic Attacks
• Hyperventilation
• Tics
• Myoclonus
• Hemifacial spasm
• Syncope
• Sleep disorders
• Non Epileptic Attacks
• Narcolepsy
• Metabolic disorders
• Transient global amnesia
Management
Medical treatment :
• Establish a correct diagnosis of epileptic
seizure type and epileptic syndrome
• Decide treatment with epileptic drugs is
necessary
• Decide which drug should be used
• Patients and their families should receive
counselling regarding :
Aims of treatment
Prognosis and duration of the expected
treatment
Importance of compliance
Side effects
Surgical treatment
Proposed Indications for resective epileptic
surgery
• Intractable seizures
• Resectable structural abnormality as identified on
magnetic resonance imaging
• Confirmation that seizures arise from a visible lesion
(using video telemetry)
• Over 20% of seizures arising from the contralateral
temporal lobe in temporal lobe seizures
• Intelligence quotient > 70 points
• No significant psychiatry morbidity
• No medical contraindications
• Age < 45 years
Strategies for managing newly diagnosed
epilepsy
Newly diagnosed epilepsy

47%
First drug Seizure-free

13%
Second drug Seizure-free

40%

Refractory

Rational duotherapy Surgical assessment


Ten commandments in the
pharmacological treatment of epilepsy
• Choose the correct drug for the seizure
type or epilepsy syndrome
• Start at low dosage and increase
incrementally
• Titrate slowly to allow tolerance to central
nervous system side-effects
• Keep the regiment simple with once- or
twice-daily dosing, if possible
• Measure drug concentration when seizures
are controlled or if control is not readily
obtained (if possible)
• Counsel the patient early regarding the
implications of the diagnosis and the
prophylactic nature of drug therapy
• Try two reasonable monotherapy options
before adding a second drug
• When seizures persist, combine the best
tolerated first-line drug with one of the
newer agents depending on seizure type
and mechanism of action
• Simplify dose schedules and drug
regimens as much as possible in patients
receiving polypharmacy
• Aim for the best seizure control
consistent with the optimal quality of life
in patients with refractory epilepsy
Drug choice in newly diagnosed epilepsy in
adolescents and adults

Seizure type First line Second line


•Sodium valproate •Lamotrigine*
Tonic clonic •Carbamazepine •Oxcarbamazepine*
•Phenytoin
Absence •Sodium valproate •Ethosuximide
•Lamotrigine*
Myoclonic •Sodium valproate •Lamotrigine*
•Carbamazepine •Lamotrigine*
Partial •Phenytoin •Oxcarbamazepine*
•Sodium valproate
Unclassifiable •Sodium valproate •Lamotrigine*

*Lamotrigine and oxcarbamazepine are regarded as first-line drugs in some countries


Choice of antiepileptic drugs in children
Seizure type First line Second line Third line
Tonic-clonic Sodium valproate Lamotrigine* Phenytoin
Carbamazepine Oxcarbazepine*
Myoclonic Sodium valproate Lamotrigine* Clobazam
Phenobarbital
Tonic Sodium valproate Lamotrigine* Clobazam
Topiramate
Absence Sodium valproate Lamotrigine* Clobazam
Ethosuximide
Carbamazepine Sodium valproate Lamotrigine*
Partial Phenytoin Gabapentin Vigabatrin
Oxcarbazepine* Clobazam
Topiramate
Infantile spasms Vigabatrin Sodium valproate Lamotrigine*
Corticosteroids Nitrazepam
Lennox-Gastaut Sodium valproate Lamotrigine* Clobazam
Topiramate Felbamate
Status Epilepticus

• Life threatening medical defined as


frequent and / or prolonged epileptic
seizure
Some Reasons for Failure of Monotherapy
Wrong diagnosis
• Syncope, cardiac arrhythmia, etc.
• Malingering, pseudoseizures
• Underlying neoplasm
Wrong drug(s)
• Inappropriate for seizure type
• Kinetic / dynamic interactions
Wrong dose
• Too low (ignore target range)
• Side effects preventing dose increase
Wrong patient
• Poor compliance with medication
• Inappropriate lifestyle (e.g. alcohol or drug abuse)
When to stop medication

• After 2-3 years period of seizure’s free,


must be tappering off in six month
Prognosis
• Dependent with underlying syndrome and / or
its cause
• Patient’s compliance
• Reciprocal illness or medications
• 60-70% controlled by first-line drug of
epilepsy
• 10% of the rest controlled by new drugs
• The rest :
surgery
Institution
Special Problems of Epilepsy
• Behavioral problem :
-Label of epilepsy racial disadvantage
-Brain function, medication, type of seizure
-Attitudes of helpers and helped
• Education :
-Discussion between doctors, families,
schools teachers and the patient, steps
which might be taken to promote normal
education and personal development
• Employment :
-Personal and racial states as well as
financial reward
-Understanding of the employee of their illness in
the context of particular employment, safety for
their selves and environment
-People around in working hours need to know
what to do if the attack occurred
• The law
– Driving lisence
• Free of seizure after 6 months controlled epilepsy
• No permitting to drive if :
– Have suffered of epileptic attack at the age before
adolescent
– Medical condition caused driving a source of danger to
them selves and to the public
• Leisure :
– Swimming, water sport, cycling, horse riding in groups
with safety controlled
– Boxing, climbing, sport with body contact are prohibited
– Television and video games, avoid flickering of the
screen
• Marriage and pregnancy
• Health education
• Impairment, disability and handicap