Epilepsy

Dr Omar Alrawashdeh
Done by: Majd AL-Hayek

1
 Definition
Epilepsy is the name for occasional, sudden, excessive, rapid and local discharges of
grey matter.

Clinically, epilepsy is a paroxysmal disorder in which cerebral cortical neuronal

discharges result in intermittent, stereotyped attacks of altered consciousness, motor
or sensory function, behavior or emotion.

A distinction must be drawn between an isolated seizure and the recurring tendency to
seizures which is epilepsy

2
 Classification
• Epileptic seizures are broadly classified by whether their onset is :

focal (partial) generalized.

Simple partial
seizures, where Complex partial seizures,
consciousness is where consciousness is
retained impaired at any stage.
throughout the
attack; Partial seizures may become secondarily generalized, the patient
losing consciousness with clinical evidence of spread across the
cerebral cortex, e.g. bilateral convulsive movements.

3
 Epilepsy

• idiopathic (most symptomatic

patients)
• Idiopathic epilepsy
frequently shows a
strong inherited
predisposition

4
 Classification
• Generalized seizures

• Focal or partial seizures:

1. Focal seizures without loss of consciousness
2. Focal seizures with loss of consciousness
3. Focal seizures with secondary generalisation

5
Focal seizures

6
 Focal seizures presentation
• Cognitive symptoms:
Seizures can start with aura in the form of :
1. automatism (hand or oral)
2. it can be reactive (drinking from a cup in the hand)
3. perseverative ( continuation of complex act). This form is usually localised to
the temporal lobe

• This can be followed by staring, fearful or sad expression.

• This is usually localised to the amygdala or anterior cingulate {Gelastic or
dacrystic automatisms } hypothalamic hamartomas

7
 Focal seizures presentation
Mesial temporal lobe epilepsy
 In these seizures, an aura or warning of the attack may consist of psychic
symptoms (e.g., fear or a sensation of dej ´ a vu ` ), hallucinations
(olfactory, gustatory or formed visual images)
 Typically begins with aura consisting of epigastric rising, fear, staring,
oroalimantary automatism.
 Patients may become confused and anxious, and exhibit organized,
stereotyped movements (automatism). These include chewing and lip
smacking, but may be more complex and sometimes aggressive and
violent
 The most common cause is hippocampal sclerosis

8
 Jacksonian epilepsy
 These focal motor attacks typically begin in the corner of the mouth,
the thumb and index finger or the great toe.
 Movements rapidly spread across the face or ascend the limb
(Jacksonian march).
 Jacksonian epilepsy is generally associated with underlying organic
brain disease, e.g. tumour in the region of the motor cortex.
 After such an attack, the affected limb(s) may remain temporarily weak
(Todd’s paralysis)

9
 Neocortical temporal cortex epilepsy

 comprises a heterogeneous group of epilepsies with focal

seizures characterized by auditory, somatosensory, or psychic auras
followed by motionless staring, early contralateral clonic activity often
secondarily generalizing

 Auditory hallucination, vertigo, language disturbances, musicogenic

seizures

10
 Focal seizures
• Frontal seziures bizarre motor automatism, hyper motor activity, salivation,
spitting
• Cingulate gyrus focus may causes expression of a particular affect such as
laughter
• Orbitofrontal cortex: complex motor automatism and olfactory
hallucinations.
• Clonic, or Tonic clonic movement in primary motor area according to the
motor homunculus involved {An example of partial seizures
that affect the primary motor cortex
is Jacksonian march}
11
 Focal seizures
• Fencers posture:
asymmetric bilateral movement of the upper limbs: supplementary motor cortex.
This can be bilateral without loss of consciousness (there is extension of the upper
limb (at the elbow) contralateral to the hemisphere of seizure onset (often with a
clenched fist and flexion at the wrist) and flexion of the ipsilateral upper limb at the
elbow )
• Unilateral forced gaze and head deviation (versive seizures): premotor cortex
and frontal eyes field.
a forced and involuntary turning of the head and eyes in one direction with an
associated neck extension resulting in a sustained unnatural position of both.
• Arm and facial seizures, with laryngeal movement, tachycardia, : contralateral
insula
12
Generalized seizure

13
 Tonic clonic seizures (grand
mal)
• convulsive activity typically lasts < 1 minute.
• Mood changes can precede seizures by days
• Immediately pretonic-clonic phase: a few myoclonic jerks or brief clonic
seizure activity; occasionally begins with forced eye and head deviation
• Tonic phase: contracture of the axial musculature with upward eye
deviation, pupillary dilation, and forced expiration of air {epileptic cry};
usually involves some decerebrate posturing. Tongue and jaw muscle
tonus causes perioral injury, typically lateral tongue biting.
Frequently, patient becomes cyanotic, tachycardic, and hypertensive

14
 Tonic clonic seizures
• Clonic phase: starts as low-amplitude, high-frequency (~ 8 Hz) convulsive
movements of the extremities > the thorax and abdomen that progresses to
high-amplitude, low-frequency (~ 4 Hz) movements. Development of atonia
breaks the seizure and causes incontinence

• Postictal phase: patient is poorly responsive and hypotonic; confusion and

memory impairment may last a few minutes to hours, occasionally followed by
psychiatric changes (depression, psychosis, anxiety, irritability) that can persist
for about a day
Postictal phase involves generalized fatigue, soreness, and migrainous
headaches
15
 Tonic clonic seizures

tonic phase: clonic phase : Postictal phase:

• generalized muscle spasms there are sharp repetitive here is usually a
occur, lasting only a few muscular jerks. Tongue headache and stiffness
second biting, incontinence of urine or injury from the fall.
• upward eye deviation, pupillary and salivation may occur. Back pain is common
dilation, and forced expiration
patient is poorly
of air {epileptic cry}
responsive and
• Frequently, patient becomes
hypotonic; confusion
cyanotic, tachycardic, and
and memory
hypertensive
impairment may last a
few minutes to hours

16
 Tonic seizures
 diffuse contraction of the axial musculature, sometimes involving the proximal
limbs or entire limbs;
 It can be in the form of startle response that can follow a stimulus of any type

17
 Atonic seizures
 Sudden loss of consciousness and muscle tone especially of the head This
can results in a drop attacks
 These are usually preceded by myoclonic jerks
 EEG may demonstrate polyspikes and waves during the ictal period and
followed by generalised slowing of the central area

18
 Myoclonic seizures
• Shock like movement of one muscle or a group of muscles.
• Irregular and can be singular or repetitive.
• It affects the eyelid, facial muscles, upper limbs ad lower limbs
• EEG: Polyspikes and waves

19
 Epilepsy syndromes
Generalised epilepsy syndromes
• Infantile spasms
• Lennox – Gastaut syndrome
• Juvenile myoclonic epilepsy
• Absence epilepsy

20
 Epilepsy syndromes
• Focal epilepsy syndromes
• Landau Kleffner syndrome
• AD nocturnal frontal lobe epilpesy
• Benign occipital epilepsies
• Benign rolandic epilepsy
• Rasmussen syndrome

21
 West syndrome (Infantile
spasms)
• Seizures: classically manifest as clusters of symmetric extensor and/ or flexor
movements, followed by tonic contractions lasting < 10 seconds
• seizures most commonly occur after awakening (i) Abdominal flexions are often
mistaken for colic (b) Autism (c) Progressive mental retardation (d) Microcephaly
• These consist of a triad of:
● brief spasms beginning within the first few months of life, characteristically shock-like
flexion of arms, head and neck with drawing up of the knees (salaam attack),
● progressive learning difficulties,
● characteristic EEG abnormality (hypsarrhythmia).
In a minority of patients the condition is idiopathic but usually a cause can be identified,
e.g. perinatal asphyxia, encephalitis, metabolic disorders and cerebral malformations.
The treatment of choice is often with corticosteroids.

22
 West Syndrome
• In75 % of cases there is an underlying structural lesion which
can be idiopathic or acquired cerebral injury ,intracranial
hemorrhage or infection, or genetic abnormality such as Down
syndrome
• EEG: Hypsarrhythmia
• Treatment: ethosuximide or Na valproate

23
 West Syndrome
• EEG: Hypsarrhythmias

24
 Lennocx- Gastaut Syndrome
• Symptoms: onset between 3– 5 years of age with
• Multiple seizure types :
1. Tonic seizures (90%).
2. Atypical absence seizures (65%) that involve repetitive facial motions
followed by loss of tone in head and face.
3. Myoclonic seizures (20%)

25
 Lennox – Gastaut Syndrome
• Interictal EEG: exhibit 2– 2.5-Hz slow spike-and-wave complexes with
frontotemporal predominance; paroxysmal fast activity > 10 Hz is apparent
while sleeping
• Ictal EEG: Atypical absence seizures exhibit 2– 2.5-Hz spike-and-wave
similar to interictal pattern

26
Lennox – Gastaut Syndrome

27
 Lennox – Gastaut Syndrome
• Pathophysiology:
60% of cases are related to structural brain abnormalities; 20% of cases develop
from infantile spasms
• medications (usually fail) Ketogenic diet or Vagal nerve stimulator; corpus
callosotomy

28
 Juvenile Myoclonic epilepsy
• Symptoms:
seizures involve bilateral but asymmetric flexor movements of the upper extremities or rarely of
the lower extremities that develop after awakening
• usually no loss of consciousness
• Other types of seizures commonly exist such as absence and tonic clonic
• Photic stimulation provokes discharges
• This is increasingly recognized as a common form of primary generalized epilepsy; age of
onset is typically in the teens.
Patients have the clinical triad of:
 infrequent generalized seizures, often on waking
 daytime absences,
 sudden, shock-like, involuntary jerking movements (myoclonus), usually in the morning
29
 Juvenile Myoclonic epilepsy
• interictal EEG demonstrates bursts of bilateral, symmetric 3.5– 6-Hz spike-
and-wave and polyspike discharges; ictal EEG exhibits diffuse polyspike
activity followed by 1– 3-Hz slow waves
• Pathophysiology: genetic, but usually with complex inheritance pattern
• Treatment: valproate > lamotrigine, levetiracetam, topiramate, zonisamide
Treatment with sodium valproate is often successful, but recurrence is likely if
medication is stopped.

30
 Absence epilepsy (petit mal)
• Symptoms:
5– 10-second long unresponsive staring spells ± rhythmic facial movements or
picking behaviours; does not have an aura or postictal state
• The attacks (typical absences) occur without warning. The child stares blankly
into space and stops talking. The eyes may flutter or roll up under the lids.
Recovery occurs within seconds and there may be many attacks daily
• Childhood-onset form is usually self-limited, whereas the juvenile-onset form is
more likely to persist into adulthood
• autosomal dominant inheritance
• slow-wave discharges on EEG may relate to cyclic activity of T-type calcium
channels and repolarizing potassium currents in the reticular thalamic nucleus

31
 Absence epilpsy
• Occur on a daily basis in the childhood-onset form, more infrequently in the
juvenile-onset form
• 50% also have generalized tonic-clonic seizures, more commonly in the
juvenile-onset form; these are infrequent and can be well controlled with
medication
• Diagnostic testing: EEG demonstrates bilateral spike-and-wave complexes at
3 Hz that are reliably activated by hyperventilation.

32
• Treatment:
1. ethosuximide, valproate, acetazolamide
2. Avoid carbamazepine, phenytoin, and gabapentin, which have
all been found to increase seizure frequency and may induce
absence status epilepticus

33
Absence epilepsy

34
 Landau-Kleffner syndrome
• is a rare, childhood neurological disorder characterized by the
1. sudden or gradual development of aphasia (the inability to understand or express language)
2. an abnormal electro-encephalogram (EEG) , LKS affects the parts of the brain that control
comprehension and speech, typically affecting understanding rather than expression.
• receptive aphasia progressing to global aphasia over a period of several days .
• Progression of aphasia is not related to frequency of seizures and is fluctuant
• Auditory agnosia may occur with involvement of the non-dominant hemisphere
• Clinical seizures (70%) usually occur during sleep and may be focal or generalized motor or hemi-
clonic status epilepticus
• The disorder usually occurs in children between the ages of 5 and 7 years. Typically, children with
LKS develop normally but then lose their language skills for no apparent reason

35
 Landau-Kleffner syndrome
• Behavioral changes: hyperactivity, aggression
• Transient motor impairments
• Treatment :
1. Glucocorticoids often improve seizures and aphasia.
2. Antiepileptic drugs control seizures but have an unclear effect on aphasia
3. In severe cases, subpial transection to isolate seizure focus or surgical
resection of seizure focus
• Prognosis: seizures remit by age 15; aphasia may persist into early
adulthood before improving
36
 Benign occipital epilepsy
• Pathophysiology: unknown; frequently occur in patients with migraine, but
not associated with structural lesions
• Symptoms: seizures involve unformed visual hallucinations, visual illusions
(micropsia, macropsia, metamorphopsia), vision loss (either complete or
hemianopic), vomiting and drooling, and/ or forced blinking and forced gaze
deviation; some patients will have seizures followed by a loss of consciousness
lasting several hours
• Seizures usually develop after a migraine headache, and migraine headaches
typically develop in patients who lose consciousness after the seizure once
they regain consciousness

37
 Benign occipital epilepsy
• Seizures usually occur during sleep onset
• Diagnostic testing: interictal EEG demonstrates unilateral or bilateral high-
amplitude occipital spike-and-wave discharges at 1.5– 2.5 Hz that are
inhibited by eye opening; ictal EEG is similar except that the spike-and-wave
discharges are at higher frequencies
• Epileptiform activity is reliably induced by photic stimulation

38
 Benign rolandic epilepsy
• Symptoms: perioral paraesthesia followed by twitching of face
and mouth with speech arrest and drooling but without
impairment of consciousness.
• convulsions may develop in an upper extremity or generalize,
usually in patients < 5 years of age
• Diagnostic testing: EEG demonstrates typical high-amplitude,
diphasic spike with prominent after going slow wave,
maximum in the centrotemporal regions

39
 Benign rolandic epilepsy
• Treatment: only if the seizures are frequent; carbamazepine
controls seizures in 65%
• Prognosis: spontaneous resolution of seizures by age 14;
withdrawal of antiepileptic medication after 2 years leaves 80%
seizure-free

40
 Rasmussen syndrome
 focal motor seizures that spread to contiguous muscle groups; 60% develop
focal status epilepticus {epilepsia partialis continua} before the disease remits
after 2– 10 years
 inflammatory degeneration of the cortex and supratentorial subcortical
structures inflammatory degeneration (its a very rare, chronic inflammatory
neurological disease that usually affects only one hemisphere (half) of the
brain) , It does not involve the contralateral hemisphere or posterior fossa
structures

41
 Rasmussen syndrome
• EEG demonstrates continuous focal spike discharges that spread
to contiguous areas of cortex and eventually to mirror foci on
the contralateral hemisphere
• Neuroimaging: may be normal initially, but hemispheric atrophy
and ipsilateral hydrocephalus ex vacuo develop within 6
months
• Treatment: refractory to antiepileptic medications; limited
benefit from immunosuppressive agents; early
hemispherectomy can be curative
42
 Antiepileptic drugs
• Predictive factors for successful seizure remission
1. A single type of seizure
2. Had no seizures for > 2 years
3. A normal neurological exam and IQ
4. A normalized EEG on antiepileptic treatment
• Reasons for refractory epilepsy include:
1. non-concordance with medication,
2. pseudoseizures or non-epileptic attacks (either alone or in combination with
genuine seizures),
3. associated structural brain disease, e.g. developmental anomalies, which may or
may not be amenable to surgery
4. alcohol and lifestyle 43
• The decision to stop treatment in an adult will be determined by:
1. duration of remission,
2. type of epilepsy,
3. effect of seizure recurrence on driving and employment
4. side effects of treatment.
5. Surgical treatment Patients with intractable epilepsy, refractory to optimal
doses of anti-epilepsy drugs, are increasingly being considered for
neurosurgical procedures.

44
 Epilpesy treatment
• Management of medication failure:
1. If maximal doses of an antiepileptic drug (AED) fail to control
the seizures, there is only a 10% likelihood of developing
control over seizures with a second AED
2. There is < 5% likelihood of developing control over seizures
with a third AED or by using multiple AEDs after failing a
second AED
3. 25% of patients with chronic refractory seizures are
eventually found to have the wrong diagnosis
45
 AED prophylaxis
• Prophylactic AED use in patients with another neurological disorder, but
without seizures, is usually not recommended :
1. Brain tumor: prophylaxis does not seem to reduce development of
seizures in patients with primary or metastatic tumors
2. Stroke: not indicated for ischemic stroke, although AEDs are routinely
used short-term in subarachnoid hemorrhage patients because of the
fear of increased intracranial pressure that accompanies seizures
3. Severe head trauma : (prolonged loss of consciousness, amnesia,
depressed skull fracture, contusion, or hematoma) can be treated with
acute prophylaxis limited to 1 week
46
47
48
49
50