3.1.1.8 - kejang (epilepsi)
DESCRIPTION
kesehatanTRANSCRIPT
EPILEPSY
Dr. Hj. Meiti Frida, Sp.S
Bagian Neurologi FK-Unand
RS. DR. M. Djamil Padang
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 EPILEPTOGENESISAsynchronous 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
Hypoxia
Hypoglycaemia
Hypocalcaemia
Febrile
Seizures
Intracranial
Infections
Birth trauma
Intracranial
haemorrhage
Congenital anomalies
Tuberous sclerosis
Storage diseases
1 5 100 20
Head Injuries
Drugs and
alcohol
Genetic epilepsies Cerebral tumours
60
Cerebrovascular degenerations
Age (years)
Factors lowering seizure threshold
Common Occasional•Sleep deprivation•Alcohol withdrawal•Television flicker•Epileptogenic drugs•Systemic infection•Head trauma•Recreational drugs•AED non-compliance•Menstruation
•Barbiturate withdrawal•Dehydration•Benzodiazepine
withdrawal•Hyperventilation•Flashing lights•Diet and missed meals•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 seizuresClonic seizuresTonic seizuresTonic clonic seizuresAtonic 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 epilepsyNon-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 featuresSpecial syndromesSituation-related seizures
- febrile convulsions- seizures related to other identifiable
situations, such as stress, hormonal changes, drugs, alcohol withdrawal or sleep deprivationIsolated, apparently unprovoked epileptic eventsEpilepsies characterized by specific modes of seizure precipitationChronic progressive epilepsia partialis continua of childhood
DiagnosisInterviews with patients or witness
• Circumstances surrounding the attacks idiopathic and generalized
No seizure worningNo underlying brain lesionsAssociated with a family history
• Symptomatic and localization relatedAuraSpecific site of onsetIdentifiable cause
• Recurrent episodes of seizures• Symptoms occured during and after seizures
Recording symptomatic events with videocamera and continous ambulatory EEG monitoring
E E G
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
ScanScan 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 spectroscopyFunctional 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
ManagementMedical 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 surgeryIntractable 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
First drug
Second drug
Refractory
Rational duotherapy Surgical assessment
Seizure-free
Seizure-free
47%
13%
40%
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
• 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
Tonic clonic
•Sodium valproate
•Carbamazepine
•Phenytoin
•Lamotrigine*
•Oxcarbamazepine*
Absence •Sodium valproate •Ethosuximide
•Lamotrigine*
Myoclonic •Sodium valproate •Lamotrigine*
Partial
•Carbamazepine
•Phenytoin
•Lamotrigine*
•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
Carbamazepine
Lamotrigine*
Oxcarbazepine*
Phenytoin
Myoclonic Sodium valproate Lamotrigine* Clobazam
Phenobarbital
Tonic Sodium valproate Lamotrigine* Clobazam
Topiramate
Absence Sodium valproate Lamotrigine*
Ethosuximide
Clobazam
Partial
Carbamazepine
Phenytoin
Sodium valproate
Gabapentin
Oxcarbazepine*
Lamotrigine*
Vigabatrin
Clobazam
Topiramate
Infantile spasms Vigabatrin
Corticosteroids
Sodium valproate
Nitrazepam
Lamotrigine*
Lennox-Gastaut Sodium valproate Lamotrigine*
Topiramate
Clobazam
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
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 :
• neurosurgery department
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