Definition- Epilepsy
•The word “epilepsy” comes from the Greek
and means to be taken, seized or
attacked.
•Epilepsy is a condition characterized by
repeated seizures due to a disorder of the
brain cells.
Epilepsy definition cont’d
•Recurrent
•Abnormally excessive and/or
hypersynchronous discharge of neurons
•Clinically expressed as recurrent seizure
of various forms
These discharges could arise from
cerebral cortex, sub cortical structures,
brainstem or spinal cord
SEIZURE
•A seizure is a result of excessive nerve-cell
discharges in the brain. It is seen as a sudden
abnormal function of the body, often with loss
of consciousness, an excess of muscular
activity, or sometimes a loss of it, or an
abnormal sensation
•A seizure is also referred to as a convulsion, fit,
or attack. However, the words “convulsion” or
“fit” are usually used to refer to seizures with
tonic-clonic muscle movements.
Epidemiology
•Prevalence
~10/1000 globally(?)
5-8/1000 in Ethiopia
•Incidence
50/100000, developed countries
100-190/100000, developing countries
•Prognosis
~70% full control with medication
5-10% refractory seizure
•Mortality higher than the general population
.
•Epilepsy – overall mortality 2-3 times that
of general population
•SUDEP: 2-18% of deaths in patients with
epilepsy
•Risk of sudden unexpected death is 24 –
40 times that in the general population
High mortality in Epilepsy
1) Underlying condition e.g. tumor
2) Frequent or uncontrolled seizure
3) Accidents e.g. drowning
4) Increased risk of suicide
High prevalence of Epilepsy in
Developing Countries
1.Poverty - inaccessibility to medical care,
low health awareness, poor sanitation
2.Higher rate of brain injuries - birth trauma,
traffic accidents
3.Higher rate of infectious diseases
Cause of Epilepsy
Perinatal brain damage
Head injuries
Cerebrovascular diseases
Neoplasm
Central nervous system infections
Congenital or metabolic disorders
Degenerative neurologic disorders
Chronic alcohol abuse
Genetics
Aggravating and precipitating factors:
•Sleep deprivation
•Starvation or hypoglycemia
•Emotional disturbance
•Drugs – psychotropic medications
•Alcohol
•Physical systemic illnesses
•Stress
•Flashing light
•Over hydration
•Hyperventilation
•Hormonal change, e.g., during menses
Medical illnesses With seizure
1) Bacterial meningitis
2) Uremia
3) Hyponatremia, hypoglycemia
4) Thyrotoxicosis
5) Cardiac arrest- hypoxic encephalopathy
6) Lead, mercury, and CO poisoning
7) Alcohol withdrawal
8) Pyridoxine deficiency
.
Attitudes Towards Epilepsy
1.Epilepsy is considered to be a form of
insanity
2.Epilepsy is attributed to supernatural
force possessions by evil spirits
3.Epilepsy is believed to be a
contagious disease
Consequences of Negative
attitude
1) Stigmatization
a)Social isolation
b)Marital break down or inability to get
married
c)Children are not allowed to play with other,
not send to school
2) Delay in medical treatment
THE CHALLENGES OF EPILLEPSY TREATMENT IN
ETHIOPIA – CAN WE BRIDGE THE TREATMENT GAP?
Question
Answer
Yes (%) No (%)
Cause-evil sprits 30.0% 70.0%
Is epilepsy contagious? 44.6% 55.4%
Would you employ an epileptic?
25.0% 75.0%
Have an epileptic as a friend? 41.7% 58.3%
Would you allow someone in
your family to marry an
epileptic?
27.6% 72.4%
How should society take care of
the persons with epilepsy-give
alms
34.2% 65.8%
International classification of epilepsy
1-Generalized seizures
–·Myoclonicseizures
–Petit mal epilepsy
–·Grand mal seizures
–·a tonic (astatic)
–·West syndrome
–·Lennox-Gastautsyndrome
2-Partial seizures (focal, local)
–·Simple partial seizures there is no impairment of
consciousness
–·Complex partial seizures -there is impairment of
consciousness
–·Partial seizure with secondary generalization e.g.
secondarily generalized
Unclassifiable –special epileptic syndrome
Grandmal Seizure
1.Prodromal subjective phenomena: for
minutes, hours or days
2.Aura: occur seconds to minutes
3.The Seizure: three phases.
oTonic phase- tonic contraction
oClonic phase - clonic jerks
oTerminal phase - remain unconscious
4.Post ictal phase
.
Complication of grand mal
Seizures
a.Burn accidents
b.Injuries - head injury, subdural hematoma,
vertebral fractures etc.
c.Transient neurologic deficit - paralysis of
extremities, aphasia, hemianopsia, etc.
Tab.2: Psychogenic seizure Vs Grand mal epilepsy
Characteristics Psychogenic seizure Grand mal seizure
Onset Gradual, prolonged with warning period Sudden
Tongue bite Very rare Frequent
Incontinence Rare Frequent
Nocturnal occurrence Rare (need the presence of an audience) Common
Postictal confusion, lethargy,
sleepiness
May be laughing or smiling Prominent
Injuries as a result of the
attack
Rare Common
Recollecting the events
during the attack
Sometimes detailed None
Neurologic signs None; forceful closure of the eyes, resistance
to open it
pupil non reactive to light,
Babinski sign present
Tab. 3: Syncope Vs Grand mal epilepsy
Characteristic Syncope Grand mal seizure
Position of the patient Rarely when patient is
recumbent
Day or night regardless of the
position of the patient
Onset More deliberate, prolonged Sudden in onset
Skin color
Pallor at the onset No color change at onset;
cyanotic at later stage
Injuries as a result of the attack None / rare Frequent injury from falling
Muscle contractions Rarely- clonic contraction of
isolated muscle groups
Generalized tonic -clonic
contractions
Urine incontinence Rare Frequent
Tongue bite Rare Frequent
Period of unconsciousness Shorter Longer
Post ictal stage Mentally clear, physically
weak
Post ictal confusion
Petit mal Epilepsy (Absence)
Characteristic epilepsy of childhood
Usually begins in the middle of the first decade
of life.
Clinical manifestations
1.No aura symptom
2.Sudden interruption of consciousness - patients
become motionless, stop talking, stare blankly,
cease to respond
3.Clonic movements- eye lids, facial muscles,
fingers
4.Simple automatism - lip smacking, chewing, etc.
Petit mal Epilepsy cont’d
Very short in duration – less than 11
seconds
Occur many times a day – even up to 100
times
Can be elicited by hyperventilation
–3 minutes of hyperventilation in the
examination room
Outcome of Petit mal Epilepsy
1.Disappear in adolescence
2.Give way to grand mal seizures
3.Petit mal persist into adulthood
4.Accompanied by additional grand mal
seizures
Myoclonic epilepsy
Myoclonic epilepsy of early childhood
oBetween 6months - 4 years
Juvenile myoclonic epilepsy
oBetween 12 years – 14 years
Clinical presentation
oFrequent upper extremity and upper trunk
myoclonic jerks
Flexor muscles of the neck and shoulders
If forceful jerk –sudden drop to the ground
oAdditional grand mal seizures or absence seizures
are possible
Focal motor seizure
•Caused by a lesion in the opposite frontal
lobe, the motor cortex.
Clinical manifestations
1.Turning movements of the head and the eyes
to the opposite side of the lesion
2.Unilateral tonic contraction of the trunk and
extremities
3.No loss of consciousness, no falling down
4.No generalized convulsion and no
incontinence
Complex partial seizure
Aura
Alterations in psychic function- perceptual
distortions , Hallucinations, illusions
Seizure
Impaired consciousness
Motor disturbance – simple automatism like lip
smacking , sucking
Complex automatic behavior – e.g. laughing, running,
picking , undressing, aggressive sexual automatisms
Post ictal phase – amnesia, deep sleep, headache
•Can occur in clusters
Complex partial seizure cont’d
Not peculiar to any age group
Past history of febrile seizure present in
~30%of patients
~60% will have additional grand mal
seizure
DIAGNOSIS
Questions raised when patient presents
with a seizure
•Seizure or not?
•Focal onset?
•Evidence of interictal CNS dysfunction?
•Metabolic precipitant?
•Seizure type? Syndrome type?
•Studies?
•Start AED?
Diagnostic Work-up
History taking
1.Complete description of the seizure itself
2.Specific precipitant factor
3.Familial predisposition
4.Perinatal and developmental history
5.Past medial history – head injury, CNS infections, etc.
Physical examination –Neurological, renal,
cardiovascular, etc
Ancillary investigation – laboratory, EEG, Skull X-ray,
CT-Scan
Diagnostic Work-up cont'd
In our set up- EEG, CT scan, MRI – required
if;
Acute onset 3-6 months
First onset after age 40
Interval sign and symptom of neurologic or
medical condition
Organic causes of epilepsy
Brain tumor- primary/secondary
Intracranial bleeding
•Thrombotic/hypertensive stroke
•Rupture of aneurysm
•Post head injury
Intracranial inflammation
•Abscess/mass – tb, meningitis, syphilis
•Cyst- neurocysticercosis
•HIV AIDS – toxoplasmosis, sarcoma
Common clinical features
Apart from the epileptic seizures, they have
interval symptoms, most commonly
1.Focalizing neurologic symptoms
Paralysis of one part of the body
Aphasia, double vision
Sensory loss e.g. anesthesia, analgesia
Major Components of Treatment
A)Non-drug measures
B)Drug treatment
C)Surgery
General non-drug measures
1. Physical and mental hygiene
• Regular hours of sleep
• Avoid substances – alcohol, hashish, cigarettes
• Avoid dangerous situations
• Moderate physical exercise
2.Removal of precipitating factors-e.g.. TV watching
3.Supportive psychotherapy – education about the
illness, avoid overprotection in children
Management during acute grand
mal seizure SHOULD DO
1.Move patient away from water, fire, traffic,
2.Take away any object that could harm the
patient
3.Loosen tight cloths, remove eye-glasses
4.Put something soft under the head
5.Turn patient to his side
6.Remain with the patient until he regains
consciousness
Management during acute grand mal
attack cont’d SHOULD NOT DO
1.Donot put anything( e.g. tongue plate) into
the mouth
2.Donot light matches
3.Donot give anything to drink
4.Donot try to stop the convulsion
5.Donot give diazepam - except during status
epilepticus or series of seizure attacks
Principles of drug treatment
A. General remark
ANTIEPILEPTIC DRUGS - AEDs
Decrease the frequency/severity of seizures
in patients with epilepsy
Treat the symptoms, not the underlying
condition
Goal: maximize quality of life by minimizing
seizures and adverse drug effects
Principles of drug treatment cont’d
B. Steps of drug treatment
1.Start drug treatment with one drug
2.Select the appropriate drug
3.Start drug treatment with a small dose
4.Gradually increase dosage until complete
control of seizure
5. Aim to achieve lowest maintenance dose
Principles of drug treatment cont’d
6. Watch for presence of marked side
effects
7. If initial drug is not well tolerated,
substitute with another
8. If initial drug cannot control seizure,
substitute with another
9.Gradually withdraw first drug while
gradually introducing the new drug
How Efficacious Are Anticonvulsants?
50% RESPOND TO 1st AED
20% RESPOND TO 2nd AED
5% RESPOND TO 3rd AED
5% RESPOND TO POLYTX
~20% OF
PATIENTS
REFRACTORY TO
AED THERAPY
Tab. 4 Drug of choice
Type of seizure Drugs
Simple partial epilepsy,
complex partial epilepsy
Carbamazepine, phenytoin,
Phenobarbital
Grandmal epilepsy Carbamazepine, phenytoin,
Phenobarbital, sodium
valproate
Petit mal epilepsy Sodium valproate,
ethosuximide
Myoclonic epilepsy Clonazepam, Valproate
Tab. 5 Drug dosage and side
effects
Drugs
Childhood
dosage,
mg/kg/day
Adult
dosage
mg/day
Common side effects
Phenobarbital 2-3 60-200 Mental dullness, sedation, drowsiness, skin
rash, hyperactivity in children
Phenytoin 4-7 100-400 Gum hypertrophy, skin rash, hirsutism,
neuropathy, ataxia, slurred speech
Carbamazepine 10-20 400-1600 Skin rash, leucopenia, elevated liver
enzymes
Sodium valproate20-40 600-1200 Liver toxicity, alopecia
Ethosuximide 20-30 750-1500 Drowsiness, hyperactivity, sleep
disturbance
Duration of drug treatment
•Grand mal epilepsy, partial epilepsy( SPS, CPS ): 4 (2-5)
years
•Petit mal epilepsy: 2 years
•Juvenile myoclonic epilepsy: for life
Indications for surgery
1.Intractable to medical therapy
2.Partial or secondarily generalized seizure
3.Absence of progressive diffuse cerebral
disease
4.Good surgical risk
SPECIAL ISSUES IN
EPILEPSY
1. STATUS EPILEPTICUS
2. FEBRILE CONVULSIONS
3. HEAD INJURY AND EPILEPSY
4. WOMEN AND EPILEPSY
5. TROPICAL DISEASES AND EPILEPSY
6. EPILEPSY AND PSYCHIATRIC DISORDERS
7. HIV/AIDS AND EPILEPSY
Status epilepticus
Definition
Status epilepticus is diagnosed when two or more
seizure occurs in close succession with out
consciousness being regained
OR a single seizure lasting 30 minutes or longer
Subtypes
Convulsive status epilepticus
GM epilepsy, Focal motor seizure (Epilepsia
partialis continua)
Non-convulsive status epilepticus
PM epilepsy, complex partial epilepsy
Causes of Status Epilepticus
Sudden discontinuation of antiepileptic drugs
Alcohol withdrawal in chronic alcoholics
Intracranial infection
Head trauma
Management of Status Epilepticus
1.General supportive measures for the
unconscious
Side positioning
Institute IV line
Control input-output
Frequent check-up of vital signs
2.Drug treatment
•Diazepam 10 mg IV slowly stat , if no response anther 10
mg IV slowly; for children 0.2-0.5 mg/kg body weight .
•If no response refer to ICU for further management
Febrile convulsions
Age related seizure disorder occurring during
febrile illnesses
Common between 6 months and 6 years of age
Seizure occur in association with high fever- usually
above 39
0
C
Commonly grand mal seizure, partial with
secondary generalization
Common child hood illnesses- URTI, otitis,
gastroenteritis
Some children develop epilepsy later
Febrile convulsions
Management of febrile convulsion
Identify and treat the primary illness
Control fever - antipyretics, cold sponges
Terminate seizure – diazepam 0.2-0.5mg/kg IV
slowly; rectal diazepam 0.5mg/kg
Head injury and epilepsy
•High risk of epilepsy after
–Open head injury
–Penetrating head injury
–Head injuries with long hours of loss of consciousness
•Genetic predisposition increases the risk
•Mainly partial seizure – simple or complex partial
seizure with or with out secondary generalization
•80% of post traumatic seizures occur with in the first 2
years of the injury
Women with epilepsy
Contraception
Fertility
Seizure during pregnancy
Obstetrical risk
Teratogenecity
Breast feeding
Women and Epilepsy
Seizure tend to frequent in the premenstrual
period
Some anti-epileptic drugs lead to contraceptive
failure – phenobarbital, phenytoin, carbamazepine
Oral contraceptives do not increase seizure risk
Fertility is lower in epileptic women
.
Possible causes of low fertility
•Direct effect of seizures on the hypothalamus,
disrupting ovulation as well as causing the
elevation of prolactin
•AEDs
–Interfere with the hypothalamic-pituitary axis,
leading to menstrual irregularities, anovulatory
cycles, and polycystic ovary disease (PCOD)
–Some AEDs induce hepatic metabolism and
decrease in the unbound fraction of the sex
hormones
•Diminished libido
Women and Epilepsy cont’d
•~ 30% of pregnant epileptic women have
increased seizure frequency
•There is increased obstetric complications
obleeding tendency during delivery
oHaemorrhagic disease of the infant
oLow infant birth weight, preterm delivery, still births
oLabor induction, operative delivery-cesarean section,
forceps
Perinatal mortality is increased in pregnancies
complicated by epilepsy
•There is increased risk of congenital
malformation in children born to epileptic women
( ~ twice as high as the general population)
Women and Epilepsy cont’d
•Generalized tonic-clonic seizures, with their
accompanying acidosis and hypoxemia, are
detrimental to the fetus, but the effects of partial
seizures on the fetus are currently unknown
•The occurrence of seizures in the first trimester
poses the greatest risk of congenital
malformation and developmental delay in the
offspring.
Management –epileptic women
If contraceptive failure
ohigher estradiol containing contraceptive (50 ug)
oLoop; levonorgestrel implant not recommended
oChange drug – e.g. valproate, gabapentine
Never stop medication during pregnancy, labor
or breast-feeding
Avoid polydrug therapy during pregnancy
Do not use carbamazepine and sodium
valproate during pregnancy
Management –epileptic women, cont’d
•Give fefol tab (4mg folic acid/day) during the whole
pregnancy
•Delivery should be in a health institution
•Give vitamin K for the mother – 10mg/day orally,
from the 36
th
week of gestation until delivery
•Give vitamin K for the new born - 1mg IM stat at
birth
•Weaning should be gradual to avoid withdrawal
symptom in the baby
Epilepsy and Tropical diseases- Pathomechanisms
3.5% of epilepsy in the tropics – due to protozoan
illnesses, helminthiasis and bacterial illnesses
Pathomechanism
•Migration of intermediate stage of parasite into the brain
tissue e.g. ascaris
•Primary inflammatory reaction of brain tissue e.g. amoebic
meningoencephalitis
•Forming space occupying space in brain e.g. cysticercosis-
cyst
•By merely increasing the incidence of febrile seizure e.g.
malaria
Epilepsy and psychiatric
disorders
.
Causes of Psychopathology in
Epilepsy
1.Social stigma of the disease- chronic
stressor
2.Long term anticonvulsant medication-
neurotoxicity
3.Repeated or recurrent seizure affecting
brain function
4.Underlying brain lesion causing both
epilepsy and psychiatric disturbance
Perictal symptoms
•Prodromal –Days or hours before seizure
Irritability, tension, restlessness, depression
•Aura –Few seconds before seizure
Perceptual disturbances, depersonalization
•Ictal – During the seizure- part of the seizure manifestation
Altered level of consciousness, variable duration
Automatism, fugue state, amnesia
•Post ictal - Just following the seizure attack
Confusional state, restlessness, aggresivity, Paranoid
hallucinatory symptom
Inter- ictal psychiatric illnesses in Epileptics
Personality disorders
Episodic dyscontrol syndrome
Cognitive disturbances
Behavioral and emotional disturbances in epileptic children
Mood disorder – unipolar depression, bipolar disorder , dysthymia
Schizophrenia-like psychosis
Episodic dyscontrol syndrome
Intermittent aggressive out burst
No or minimal provocation
No amnesia for the incident
Depression
–Commonest psychiatrist manifestation of epilepsy
–20-60% of epileptic patients suffer from
depression,
–Twice as common than other chronic disabling
illnesses
–Mainly unipolar depression – dysthymia, major
depressive disorder
–Suicide risk is 5 times that of the general
population
Schizophrenia-like psychosis
1.Transient Schizophrenia-like psychosis
•Short lasting - day to few weeks
•Remissions are complete
•Symptoms comprise of delusions,
hallucinations, and disorganized behavior
2.Chronic schizophrenia- like psychosis
•After many years of seizure disorder
•Persists for years, no full remission
•Characteristic features include….
…Chronic schizophrenia-like psychosis
Frequent religious/mystical delusions
Passivity phenomena
Paranoid delusions are common
Affect is warmer and better retained ; more benign
course, less personality and social deterioration
Catatonic and hebephrenic features are rare
No genetic loading for schizophrenia
More common in left sided or bilateral temporal lobe
lesion
Management
Some antipsychotics and antidepressants
exacerbate seizure
•Chlorpromazine and clozapine are worst
antipsychotics
•Cloimipramine is the worst antidepressant
Management – cont…
For schizophrenia-like psychosis
Donot use chlorpromazine
Donot use clozapine
Risperidone, thioridazine, modecate, haloperidol,
and molindone -preferred drugs
For unipolar depression
Donot use cloimipramine, bupropion, amoxapine,
maprotiline
Trimipramine, desipramine, imipramine, SSRI’s –
preferred drugs
Management cont…
For bipolar disorder
Carbamazepine and sodium valproate are
the preferred drugs
Lamotrigine –both antiepileptic and mood
stabilizer
ECT – for refractory cases of depression and
mania
Management cont…
Avoid polypharmacy, single drug from
each group
Start with the least seizure provoking
drug
Start low and go slow with the
antipsychotic/antidepressant medication
Increase dose of anticonvulsant drug
only if seizure recurs
Monitor side effects and toxicity
HIV/AIDS and Epilepsy
• .
Cause of seizure
Directly due to the HIV
Secondary to HIV related tumors, hemorrhages
Secondary to opportunistic infections
Drug toxicity – ART and drugs used to treat
other related illnesses
Time of occurrence “new-onset seizure”
Occurs at any stage of the illness
From seroconversion to late stage
In 3-18% the first presenting symptom
Strong association with focal lesion
Type of seizure
1.~70% generalized – grand mal
2.Simple partial
3.Complex partial
Status epilepticus could also occur
Epilepsia partialis continua
Generalized convulsive status epilepticus
Treatment of epilepsy in HIV/AIDS
•High risk of seizure recurrence
Maintenance treatment after first-
onset seizure
•Drug toxicity
Compromised brain
Highly sensitive to drugs
Treatment of epilepsy in HIV/AIDS cont’d
•No anticonvulsant has absolute contraindication
Valproate –hepatotoxicity, AZT toxicity
Carbamazepine – bone marrow depression,
leucopenia
Phenobarbital - over sedation
Phenytoin –neurotoxicty
•Special attention for those taking protease
inhibitors- stronger drug-drug interactions
EEG
.
•Harmless and painless investigation
•Measuring of brain waves
–Electrodes are put on the scalp
–Potential difference between various sites measured
•Takes ~20-30 minutes
•Some provocation method used
–Hyperventilation
–Photic stimulation
–Sleep deprivation-sleep EEG
,
Types of waves
Alpha
Beta
Theta
Delta
Normal variations
–Age
–Sleep
Epileptiform discharges
Sharp waves
Spikes
Spike-wave complexes
Importance of EEG
1.Confirming the diagnosis of epilepsy
2.Classifying seizure correctly – for some types
of epilepsy
3.Localizing epileptic focus
4.Identifying the presence or absence of
underlying lesion e.g. tumor
5.Controlling the effectiveness of
anticonvulsant medication
.
EEG findings in epilepsy patients
1. Normal EEG
2. Non-specific abnormalities
– background slowing
– regional slowing
– generalized slowing
3. Epileptiform discharges
– interictal epileptiform discharges
– ictal epileptiform discharges
a normal EEG does not exclude epilepsy
a pathologic EEG cannot prove epilepsy
•
AWARENESS CREATION WITHIN THE GENERAL POPULATION ON EPILEPSY AND ITS
MANAGEMENT.
•
EDUCATE HEALTH PERSONNEL AT EVERY LEVEL ON EPILEPSY TREATMENT.
•
GENERAL PRACTITIONERS AND INTERNISTS SHOULD BE ENCOURAGED TO TREAT AND
FOLLOW UP UNCOMPLICATED EPILEPSY CASES PARTICULARLY IN RURAL HOSPITALS.
•
UPGRADE THE TRAINING OF PSYCHIATRY NURSES ON EPILEPSY MANAGEMENT AND
DEPLOY THEM TO RUN EPILEPSY CLINICS UNTIL SUCH TIME THAT WE HAVE ADEQUATE
NEUROLOGISTS.
•
MAKE PHENOBARB AND PHENYTOIN READILY AVAILABLE AT ALL HOSPITALS (? HEALTH
CENTERS). CONTINUOUS SUPPLY SHOULD BE GUARANTEED. OTHER ANTICONVULSANTS
TO BE CONTINUOUSLY AVAILABLE WHEREVER NEUROLOGISTS PRACTICE.
•
THE LOCAL PHARMACEUTICAL FACTORY SHOULD BE REQUIRED TO PRODUCE PHENOBARB
AND PHENYTOIN AND SELL THEM AT SUBSIDIZED PRICE.
•
MEDICAL SCHOOLS AT JIMMA, HWASSA, GONDAR AND MEKELE SHOULD HAVE AT LEAST
ONE NEUROLOGIST AS A MEMBER OF THE TEACHING STAFF.
•
NEUROLOGY DEPARTMENTS AT TERTIARY CENTERS SHOULD BE FULLY EQUIPPED WITH
NEUROPHYSIOLOGICAL LABORATORIES TO INVESTIGATE COMPLICATED EPILEPSY CASES.
SUGGESTIONS ON BRIDGING THE TREATMENT GAP