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Practical Approach To An Epileptic Patient

Diagnosis of epilepsy

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Practical Approach

To An Epileptic

Patient

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Amr Hasan, M.D. Associate professor of Neurology - Cairo University

Diagnosis of Epilepsy

3

History taking..

• Physical examination in most

cases is relatively unrevealing

• History – taking from the patient

and observer is the most

important step for reaching the

diagnosis.

Diagnosis of Epilepsy

4

Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy

Axis 2: • Identify type of seizure (seizure semiology)

International classification of epileptic seizures.

Axis 3: • Investigations aiming at confirmation of the

diagnosis & searching for an aetiology

Axis 4: • Identify epileptic syndrome International

classification of epilepsy & epileptic syndromes

Diagnosis of Epilepsy

5

AXIS 1: D.D. OF EPILEPSY

6

A) Episodic impairment of consciousness

1. Impaired cerebral perfusion e.g. TIAs

2. Syncope.

3. Metabolic disturbances (e.g.,hypoglycemia).

4. Sudden increase in intracranial pressure.

5. Sleep disorders.

6. Movement disorders.

7. Psychologically – related phenomena.

7

Red flags:

8

-Young adult female, low socioeconomic and educational state. -Nontraditional triggers

- Events in the waiting room…audience - Longer duration >2min - Historionic behavior, comorbid psych depression - Rapid cognitive recovery - Symptomatizing lengthy dis: fatigue, fibromyalgia - Refractory to AED - High frequency not affected by AED - History of abuse - Pt recalls events during period he was unresponsive

AXIS 1: D.D. OF EPILEPSY

9

B) Drop Attacks

• Falling spells without warning signs, without loss of

consciousness or post-ictal symptoms.

• Identifiable mechanisms OTHER THAN epilepsy include;

1. Brainstem ischemia, or rarely if both ACA arise from the

same stenosed ICA.

2. Hydrocephalus.

AXIS 1: D.D. OF EPILEPSY

10

B) Drop Attacks

3. Otolithic catastrophe” of Meniere disease.

4. Cataplexy.

5. orthostatic hypotension.

6. EPS.

AXIS 1: D.D. OF EPILEPSY

11

C) Transient Focal Neurologic Manifestions

1. Migraine with aura.

2. Transient global amnesia.

3. Transient ischemic attacks.

4. Structural intracranial lesions (AVM, chronic subdural

hematoma).

AXIS 1: D.D. OF EPILEPSY

12

C) Transient Focal Neurologic Manifestions

5. Metabolic disorders (hyperglycemia “NKHO”,

hypoglycemia, hypercalcemia, hyponatremia).

6. Labyrinthine disorders (e.g. Meniere’s and BPV).

7. Paroxysmal abnormal involuntary movements e.g.

Paroxysmal dystonia & tics.

8. Psychological.

AXIS 1: D.D. OF EPILEPSY

13

D) Non-Epileptic Paroxysmal Attacks in Pediatric Age Group

• Breath-holding attacks: • Cyanotic spells

• Pallid spells “reflex anoxic seizures”

• Movement disorders:

• Benign non-epileptic myoclonus of early infancy

Paroxysmal choreoathetosis

• Shuddering attacks,

• hyperekplexia, paroxysmal torticollis and tics

Diagnosis of Epilepsy

14

Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy

Axis 2: • Identify type of seizure (seizure semiology)

International classification of epileptic seizures.

Axis 3: • Investigations aiming at confirmation of the

diagnosis & searching for an aetiology

Axis 4: • Identify epileptic syndrome International

classification of epilepsy & epileptic syndromes

Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy

Diagnosis of Epilepsy

15

International Classification of Epilepsies

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I. Partial (focal, local) seizures

A. Simple partial seizures (consciousness not impaired)

1. With motor signs

2. With sensory symptoms

3. With autonomic symptoms or signs

4. With psychic symptoms

International Classification of Epilepsies

17

I. Partial (focal, local) seizures B. Complex partial seizures (temporal lobe or psychomotor

seizures; consciousness impaired)

1. Simple partial onset, followed by impairment of

consciousness

a. With simple partial features (A.1-A.4), followed by impaired

consciousness

b. With automatisms

2. With impairment of consciousness at onset

a. With impairment of consciousness only

b. With automatisms

International Classification of Epilepsies

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I. Partial (focal, local) seizures

C. Partial seizures evolving to secondarily generalized seizures

(tonic-clonic, tonic or clonic)

1. Simple partial seizures (A) evolving to generalized seizures

2. Complex partial seizures (B) evolving to generalized seizures

3. Simple partial seizures evolving to complex partial seizures,

evolving to generalized seizures

International Classification of Epilepsies

19

II. Generalized seizures (convulsive or nonconvulsive)

1. Simple partial seizures (A) evolving to generalized seizures

2. Complex partial seizures (B) evolving to generalized seizures

3. Simple partial seizures evolving to complex partial seizures,

evolving to generalized seizures

III. Unclassified epileptic seizures (caused by incomplete

data)

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

20

I. Partial (focal, local) seizures

A. Simple partial seizures

• Are caused by focal cortical discharge that results in seizure

phenomenon appropriate to the function of the discharging

area of the brain without impairment of consciousness

• SPS may consist of motor , sensory, autonomic or psychic

symptoms and signs

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

21

I. Partial (focal, local) seizures

• The symptoms are, at least initially, always strictly contralateral to the

hemispheric focus and may represent the expression of excitatory

(positive—irritative) phenomena, inhibitory (negative—suppressive or

paralytic) phenomena, or a combination of the two.

A. Simple partial seizures

1. Simple partial seizures with motor signs:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

22

I. Partial (focal, local) seizures

• The simplest form of simple partial seizure with motor signs is

clonus, which consists of rhythmic alternating contraction and

relaxation of muscle groups controlled by the precentral gyrus

A. Simple partial seizures

1. Simple partial seizures with motor signs:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

23

I. Partial (focal, local) seizures

• The episodes may be self-limited (clonic focal seizure), recurrent (focal

motor status epilepticus), or continuous (epilepsia partialis continua).

• Spread of the discharge along contiguous areas of the precentral gyrus

gives rise to the characteristic march of spreading involvement of muscle

groups in jacksonian seizures.

A. Simple partial seizures

1. Simple partial seizures with motor signs:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

24

I. Partial (focal, local) seizures

• Somatosensory seizures are usually described as"numbness,"

"tingling," "pins and needles," or "like a weak electric shock" and

may arise from the postcentral (most often) or precentral areas.

A. Simple partial seizures

2. Simple partial seizures with sensory symptoms:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

25

I. Partial (focal, local) seizures

• The focal discharge may spread to the adjacent sensory cortex, producing

a jacksonian march of sensory phenomena.

• The focal discharge also may spread to the adjacent motor cortex,

producing motor symptoms.

A. Simple partial seizures

2. Simple partial seizures with sensory symptoms:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

26

I. Partial (focal, local) seizures

• The focal discharge may spread to the adjacent sensory cortex, producing

a jacksonian march of sensory phenomena.

• The focal discharge also may spread to the adjacent motor cortex,

producing motor symptoms.

A. Simple partial seizures

3. Simple partial seizures with autonomic symptoms or signs:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

27

I. Partial (focal, local) seizures

• Autonomic symptoms accompanying simple partial seizures may consist of

epigastric sensations, flushing or pallor, sweating, pupil dilation,

diaphoresis, piloerection, nausea, vomiting, borborygmi, or incontinence.

A. Simple partial seizures

3. Simple partial seizures with autonomic symptoms or signs:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

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I. Partial (focal, local) seizures

• Psychic symptoms of simple partial seizures may include dysphasia,

dysmnesia, cognitive symptoms, affective symptoms, illusions, or

structured hallucinations.

• Dysphasic symptoms may take the form of speech arrest, vocalization, or

palilalia (involuntary repetition of a syllable or phrase).

A. Simple partial seizures

4. Simple partial seizures with psychic symptoms.

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

29

I. Partial (focal, local) seizures

• Dysmnesic symptoms, distortions of memory, may take the form of a temporal

disorientation, a dreamy state, a flashback, the sensation that an experience has

occurred before (deja vu, if, visual; deja entendu, if auditory), or the sensation

that a familiar sensation is new (jamais vu, if visual; jamais entendu, if auditory).

• Occasionally, a patient may experience a rapid recollection of episodes from the

past (panoramic vision).

A. Simple partial seizures

4. Simple partial seizures with psychic symptoms.

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

30

I. Partial (focal, local) seizures

• The central feature of CPSs is impairment of consciousness,

which is defined as the inability to respond normally to exogenous

stimuli by virtue of altered awareness or responsiveness.

B. Complex Partial Seizures (Psychomotor or Temporal

Lobe Seizures)

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

31

I. Partial (focal, local) seizures

• Responsiveness refers to the ability of the patient to carry out

simple commands or willed movement.

• Awareness refers to the patient's contact with events during the

period in question and its recall.

B. Complex Partial Seizures (Psychomotor or Temporal

Lobe Seizures)

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

32

I. Partial (focal, local) seizures

• The period of impairment of consciousness may or may not be

preceded by symptoms or signs of a simple partial seizure.

B. Complex Partial Seizures (Psychomotor or Temporal

Lobe Seizures)

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

33

I. Partial (focal, local) seizures

• Other manifestations may appear during the period of impaired

consciousness, or automatisms (i.e., nonreflex actions performed

"automatically," without conscious volition, and for which the patient

has no recollection.

B. Complex Partial Seizures (Psychomotor or Temporal

Lobe Seizures)

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

34

II. Generalized seizures

• Absence seizures consist of sudden onset and cessation of

impaired responsiveness, accompanied by a unique 3-Hz spike and

wave EEG pattern.

1. Absence Seizures:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

35

II. Generalized seizures

•No aura is present, and little or no postictal symptomatology occurs.

• The majority of absence seizures last 10 seconds or less and may

be accompanied by mild clonic components, atonic or tonic

components, automatisms, or autonomic components.

1. Absence (Petit Mal) Seizures:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

36

II. Generalized seizures

• Absence seizures usually first manifest between the ages of 5 and

12 years and often stop spontaneously in the teens.

1. Absence Seizures:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

37

II. Generalized seizures

•Myoclonic seizures consist of brief, sudden muscle contractions that may be generalized or localized, symmetric or asymmetric, synchronous or asynchronous.

• No loss of consciousness is usually detectable

2. Myoclonic Seizures:

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

38

II. Generalized seizures

•Tonic seizures consist of a sudden increase in muscle tone in the axial or extremity muscles, or both, producing a number of characteristic postures.

•Consciousness is usually partially or completely lost

3. Tonic Seizures

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

39

II. Generalized seizures

•Prominent autonomic phenomena occur.

•Postictal alteration of consciousness is usually brief, but it may last several minutes.

•Tonic seizures are relatively rare and usually begin between 1 and 7

years of age

3. Tonic Seizures

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

40

II. Generalized seizures

•Atonic seizures consist of sudden loss of muscle tone.

•The loss of muscle tone may be confined to a group of muscles, such as the neck, resulting in a head drop.

•Alternatively, atonic seizures may involve all trunk muscles, leading

to a fall to the ground .

4. Atonic Seizures

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

41

II. Generalized seizures

•Clonic seizures occur almost exclusively in early childhood.

•The attack begins with loss or impairment of consciousness associated with sudden hypotonia or a brief, generalized tonic spasm.

•This is followed by 1 minute to several minutes of bilateral jerks,

which are often asymmetric and may appear predominately in one limb

5. Clonic Seizures

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

42

II. Generalized seizures

•During the attack, the amplitude, frequency, and spatial distribution of these jerks may vary greatly from moment to moment.

•In other children, particularly those aged 1 to 3 years, the jerks

remain bilateral and synchronous throughout the attack.

•Postictally, recovery may be rapid, or a prolonged period of confusion or coma may ensue.

5. Clonic Seizures

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

43

II. Generalized seizures

• Before the tonic phase of a tonic-clonic seizure, bilateral jerks of the

extremities or focal seizure activity may occur.

• The onset of the seizure is marked by loss of consciousness and increased

muscle tone (tonic phase), which usually results in a rigid, flexed posture at

first, and then a rigid, extended posture.

6. Tonic-Clonic (Grand Mal) Seizures

AXIS 2: IDENTIFY TYPE OF SEIZURE

TYPES OF SEIZURES

44

II. Generalized seizures

• This is followed by bilateral rhythmic jerks that become further apart (clonic

phase).

• Prominent autonomic phenomena are observable during the tonic and

clonic phases. Incontinence may occur.

• The patient awakens by passing through stages of coma, confusional state

and drowsiness

6. Tonic-Clonic (Grand Mal) Seizures

Diagnosis of Epilepsy

45

Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy

Axis 2: • Identify type of seizure (seizure semiology)

International classification of epileptic seizures.

Axis 3: • Investigations aiming at confirmation of the

diagnosis & searching for an aetiology

Axis 4: • Identify epileptic syndrome International

classification of epilepsy & epileptic syndromes

Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy

Axis 2: • Identify type of seizure (seizure semiology)

International classification of epileptic seizures.

Diagnosis of Epilepsy

46

AXIS 3: INVESTIGATIONS

Labs

47

Should be tailored according to the case;

• Drug screen

• Electrolytes

• KFT

• LFT

• CSF examination

• Lactic acid

AXIS 3: INVESTIGATIONS

EEG

48

Conventional Electroencephalography

• Used in establishing the diagnosis and focality of

an epileptic disorder

• Normal EEG doesnot exclude presence of

epilepsy thus the yield of EEG can be increased

by Sleep deprivation(< 4 h sleep )

• Invasive techniques are used in some cases

which include: • Sphenoidal leads,

• Subdural

• Epidural electrodes

• Depth electrodes.

AXIS 3: INVESTIGATIONS

EEG

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Multiple Electroencephalography (EEG) exams

• Records interictal and ictal events, sometimes with sleep

deprivation or other activation techniques may help in

bringing out the abnormality.

EEG monitoring with video (video-EEG)

• Used widely in the evaluation of epileptic fits , exclusion

of non-epileptic attacks and to define the electro-clinical

characteristics of the seizures.

AXIS 3: INVESTIGATIONS

NEUROIMAGING

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A) MRI

• MRI Has become necessary in

delineating anatomic abnormalities

that may be the etiology of the

seizure focus such as:

• Focal cerebral pathologies e.g.

neoplastic lesions ,infarction,

vascular malformations.

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AXIS 3: INVESTIGATIONS

NEUROIMAGING

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A) MRI

• Diffuse cerebral

pathologies e.g.

cytoarchitectural lesions,

such as neuronal

heterotopias, gyral

anomalies, and other

abnormalities.

AXIS 3: INVESTIGATIONS

NEUROIMAGING

58

B) Functional Neuroimaging

• When anatomical imaging fails to identify a lesion,

positron emission tomography (PET) and single photon

emission tomography (SPECT) are used.

• PET measures regional cerebral metabolism and blood

flow. It has been quite successful in identifying the focus

as an area of hypometabolism between attacks.

AXIS 3: INVESTIGATIONS

NEUROIMAGING

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B) Functional Neuroimaging

• SPECT demonstrates regional cerebral blood flow, which

is linked to cerebral metabolism and can therefore be

used to identify the epileptic focus and can be used to

study both ictal and inter-ictal states.

AXIS 3: INVESTIGATIONS

NEUROIMAGING

60

C) Others

1. Proton MRS

2. Magnetoencephalograpy/magnetic source imaging

• However, PET ,SPECT ,Proton MRS &

Magnetoencephalograpy/magnetic source imaging

should be only done when surgical resection is planned

Diagnosis of Epilepsy

61

Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy

Axis 2: • Identify type of seizure (seizure semiology)

International classification of epileptic seizures.

Axis 3: • Investigations aiming at confirmation of the

diagnosis & searching for an aetiology

Axis 4: • Identify epileptic syndrome International

classification of epilepsy & epileptic syndromes

Axis 1: • Is it epileptic seizure or not? D.D. Of epilepsy

Axis 2: • Identify type of seizure (seizure semiology)

International classification of epileptic seizures.

Axis 3: • Investigations aiming at confirmation of the

diagnosis & searching for an aetiology

Diagnosis of Epilepsy

62

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AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

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EPILEPTIC SYNDROME IS DEFINED BY

1. Type or types of seizure e.g. Absence ,GTC.

2. Age of seizure onset

3. Aetiology

4. Degree of neurologic and intellectual deficit

5. Clinical evolution of the epilepsy

6. EEG pattern

7. Neuroimaging abnormality

Important epileptic

syndromes

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AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

66

I. LOCALIZATION RELATED (IDIOPATHIC)

Benign epilepsy of childhood with centrotemporal spikes

• The syndrome is termed "rolandic" epilepsy

because of the characteristic features of partial

seizures involving the region around the lower

portion of the central gyrus of Rolando.

AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

67

I. LOCALIZATION RELATED (IDIOPATHIC)

Benign epilepsy of childhood with centrotemporal spikes

• Common characteristic features includes :Unilateral

somatosensory involvement, mostly of the tongue

(occasionally of the inner cheeks, lips, gums, or even a

single tooth) ,Speech arrest ,Preservation of

consciousness in most cases ,Pooling of saliva &Tonic

or tonic-clonic spread to face.

AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

68

I. LOCALIZATION RELATED (IDIOPATHIC)

Benign epilepsy of childhood with centrotemporal spikes

• The typical interictal EEG shows centrotemporal spikes

or SW, which are either unifocal or bifocal.

• Carbamazepine is often the first medication to be tried,

and seizures usually are well controlled.

• Excellent prognosis.

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AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

71

I. LOCALIZATION RELATED (IDIOPATHIC)

Childhood epilepsy with occipital paroxysms:

• Early onset type :

• Young childern 5 ys

• Ictal vomiting , deviation of the eye ,impairment of the

consciousness sometimes progress into GTCs

• Two thirds of seizures occur during sleep

AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

72

I. LOCALIZATION RELATED (IDIOPATHIC)

Childhood epilepsy with occipital paroxysms:

• Late onset (Gastaut) type :

• Older childern 9 ys

• Formed visual hallucinations or amaurosis followed

by hemiclonic convulsions with post-ictal migrain

• EEG: occipital spikes

• Excellent prognosis

AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

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II. GENERALIZED(IDIOPATHIC):

Childhood absence epilepsy

• Seizure semiology:

• Absence:Typical absence

• High frequency hundreds / day

• + Myoclonus

• + GTCS

• 3-12 years

• EEG changes: 3-Hz spike and wave

• Treatment : VPA, EXM

• Favorable outcome( long term remission)

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AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

77

II. GENERALIZED(IDIOPATHIC):

Juvenile myoclonic epilepsy

• Genetic disorder

• Females>Males

• Age 8-26 Y, but 79% mostly12-18 Y

• Symmetric myoclonus (upper limbs)

• GTCs: common

• Absence (associated or independently)

• Photosensitivity

• EEG : polyspike and spike wave(rapid)

• Respond to medical ttt: VPA,LEV

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AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

85

II. GENERALIZED (CRYPTOGENIC):

West syndrome (infantile spasms):

• Triad of :

1. Seizures

2. Psychomotor retardation or regression

3. Specific EEG changes : hypsarrythmia.

• 1st year of life

• Related to prenatal ,natal or postnatal insult

AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

86

II. GENERALIZED (CRYPTOGENIC):

West syndrome (infantile spasms):

• Seizures

1. Flexor spasms

2. Extensor spasms

3. Mixed flexor & extensor spasms

• Resistant to treatment

• Poor prognosis

AXIS 4: IDENTIFY EPILEPTIC SYNDROME

EPILEPTIC SYNDROMES

87

II. GENERALIZED (CRYPTOGENIC):

Lennox Gastaut syndrome

• Mixed seizure disorder:

• (Tonic, TC, Myoclonic, Atypical absence & drop attacks )

• Mental retradation

• EEG:

Slow spike & wave

Sharp & slow wave complex

Slow abnormal background

• Treatment : commonly need Polytherapy

• Poor prognosis & cure rarely achieved

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