Dr.

Zuraini, sps

What Is the Difference Between Epilepsy & Seizures?

A seizure is a brief, temporary disturbance in the electrical activity of the brain Epilepsy is a disorder characterized by recurring seizures (also known as seizure disorder)

A seizure is a symptom of epilepsy

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Seizure: time-limited paroxysmal events that result from abnormal, involuntary, rhythmic neuronal discharges in the brain Unpredictable (can occur at inconvenient, embarrassing, or even dangerous times) Convulsion: the muscle contractions that can occur during a seizure.

A seizure is as a sudden, disorderly discharge of cerebral neurons.

Seizures involve a transient alteration in brain function involving motor, sensory, autonomic, or psychic clinical manifestations

The occurrence of repeated unprovoked seizures* Unprovoked seizure: events occurring in absence of a recognized etiological or risk factor (idiopathic and cryptogenic) and events occurring in patients with antecedent stable (non-progressive) CNS insults (remote symptomatic seizure). A chronic disorder characterized by recurrent (more than 2) unprovoked seizures.

*ILAE, Epilepsia1993;34:592-6

Aura: A partial seizure experience before the onset of a generalized seizure; often described as a funny feeling Prodroma: Early clinical manifestations that may occur hours to a few days before the onset of a seizure (malaise, headache, or depression) Tonic phase: A state of muscle contraction with increased muscle tone (associated with loss of consciousness)

Clonic phase: Alternating contraction and relaxation of muscles Postictal phase: Time period immediately after the end of seizure activity

About 2.3 million Americans have epilepsy (0.5-1% of the population) Roughly 181,000 new cases of seizures and epilepsy occur each year 50% of people with epilepsy develop seizures by the age of 25; however, anyone can get epilepsy at any time Now there are as many people with epilepsy who are 60 or older as children aged 10 or younger
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 All brain functions -- including feeling, seeing, thinking, and moving muscles -- depend on electrical signals passed between nerve cells in the brain
A seizure occurs when too many nerve cells in the brain fire too quickly causing an electrical storm

In about 70% of people with epilepsy, the cause is not known In 30%, most common causes are:
- Head trauma - Infection of brain tissue - Brain tumor and stroke - Heredity - Prenatal disturbance of brain development

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1.

2.

3.

Is this a seizure? Is this a provoked or unprovoked seizure? What is the probability of recurrence of seizures (to establish a diagnosis of epilepsy)?

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Primary epilepsy Secondary epilepsy

Prenatal and perinatal factors 2. Trauma and surgery 3. Metabolic cuases 4. Toxic causes
1.

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5. Infectious and inflammatory causes 6. Cerebral vascular diesease 7. Intracranial tumors 8. Hypoxia 9. Degenerative disease

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Electrical discharges between neurons are usually restricted, and produce the normal rhythm recorded on the EEG (electroencephalogram). When a seizure occurs, large groups of neurons are activated repetitively and hypersynchronously, with dysfunction of the inhibitory synaptic contact between neurons. This produces the high-voltage spike-andwave activity on the EEG.
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The onset of the epileptic discharge may include the whole cortex (primary generalized), may be confined to one area of the cortex(partial), or may start focally and then spread to involve the whole cortex (secondary generalization of a partial seizure).

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Psychogenic nonepileptic spell (pseudoseizure) Syncope Migraine Transient Ischemic attack Sleep behaviors and disorders Movement disorders

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Characteristics

Prolonged duration of event (10-30 min) Preservation of consciousness despite whole body jerking Bizarre and asynchronous motor movements Pelvic thrusting movements Not stereotypical

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Metabolic abnormalities

Hypo/Hyperglycemia Hyponatremia Hypocalcemia

Alcohol withdrawal Acute neurological insult

Infection (meningitis, encephalitis) Stroke (ischemic, hemorrhagic) Head trauma

Illicit drug intoxication the lowers seizure threshold (i.e. Theophylline, tricyclic antidepressant) High fever in children

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Seizure Loss of consciousness at the onset? yes No

Generalized seizures

Partial seizures Alteration of consciousness

Yes
??? Complex partial

No
Simple Partial

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Focal seizures account for

80% of adult epilepsies Simple partial seizures Complex partial seizures Partial seizures secondarilly generalised

Generalised seizures Unclassified seizures

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I. Partial (focal, local) seizures

A. Simple partial seizures (consciousness not impaired) B. Complex partial seizures (with impairment of consciousness) C.partial seizures evolving to generalized seizures

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Simple partial seizures Motor, sensory, vegetative or psychic symptomatology Typically consciousness is preserved

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Complex partial seizures (= psychomotor seizures) Initial subjective feeling (aura), loss of consciousness, abnormal behavior (perioral and hand automatisms)

Usually originates in TL

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Partial seizures evolving to tonic/clonic convulsions secondary generalised tonic/clonic seizures (sGTCS)

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II. Generalized seizures

A. Absence seizures
1. Absence seizures 2. Atypical absence seizures

B. Myoclonic seizures C. Clonic seizures D. Tonic seizures E. Tonic-clonic seizures F. Atonic seizures (astatic seizures)

III. Unclassified seizures

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Absences Myoclonic seizures Clonic seizures Tonic seizures Atonic seizures

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I. Localization-related (focal, local, partial) epilepsies

and syndromes A. Idiopathic (with age-related onset). At present, two syndromes are established: 1. Benign childhood epilepsy with centro temporal spikes 2.Childhood epilepsy with occipital paroxysms B. Symptomatic. This category comprises syndromes of great individual variability.
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Temporal lobe epilepsy Frontal lobe epilepsy Parietal lobe epilepsy Occipital lobe epilepsy Absence epilepsy Myoclonic epilepsy Syndrome
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Medial basal

Aura: epigastric discomfort, autonomic sign eg. Pallor, flushing, fearful, olfactory and gustatory

Lateral temporal

Auditory/visual disturbance/speech

Lasting > 1 min, amnesia, post-ictal confusion with gradual recovery to normal

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Brief duration Complex partial with little or no postictal confusion Rapid secondary generalization Prominent tonic or postural movement Frequent complex gestural automatism at onset Frequent falling when discharges are bilateral.

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Sensory at the onset Tactile, tingling, electricity, crawling, stiffness, cold, or pain, or unpleasant dysaesthesias. Post-ictal transient deficit eg. Cortical sensory deficit

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Eye movement, head turning and/or visual hallucinations Hallucination:

Posteriorly: unstructured light Anteriorly: structured image or visual distortions such as macropsia or micropsia

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Epilepsy syndrome

Localization-Related Epilepsy Idiopathic

Generalized Epilepsy Idiopathic

Symptomatic
Cryptogenic

Symptomatic
Cryptogenic

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Idiopathic epilepsy
Localization-related BFEC (Benign Rolandic Epilepsy of Childhood) Benign Occipital Epilepsy of childhood Autosomal Dominant Nocturnal Frontal Lobe Epilepsy Primary reading epilepsy Generalized Benign neonatal convulsion Benign myoclonic epilepsy in infancy CAE JAE JME Epilepsy with generalized tonic-clonic seizures on awakening Some reflex epilepsy
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Symptomatic epilepsy
Localization-related Temporal lobe Frontal lobe Parietal lobe Occipital lobe Rasmussen encephalitis Most reflex epilepsies Generalized Early myoclonic encephalopathies Early infantile epileptic with suppression burst (Ohtahar syndrome) Cortical abnormalities dysplasias Metabolic abnormalities West syndrome Lennox-Gastaut syndrome

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Cryptogenic epilepsy Localization-related Generalized

Any occurrence of partial seizure without obvious pathologic cause

Epilepsy with myoclonicastatic seizures

Epilepsy with myoclonic absence seizures

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II. Generalized epilepsies and syndromes

A. Idiopathic (with age-related onset, in order of age appearance) 1. Benign neonatal familial convulsions 2. Benign neonatal convulsions 3. Benign myoclonic epilepsy in infancy 4. Childhood absence epilepsy (pyknolepsy, petit mal) 5. Juvenile absence epilepsy 6. Juvenile myoclonic epilepsy (impulsive petit mal) 7. Epilepsy with grand mal seizures on awakening

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II. Generalized epilepsies and syndromes B. Idiopathic, symptomatic, or both (in order of age of appearance) 1. Infantile Spasms 2. Lennox Gastaux 3. Epilepsy with myoclonic-astatic seizures 4. Epilepsy with myoclonic absences

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C. Symptomatic 1. Nonspecific cause, early myoclonic encephalopathy 2. Specific syndromes. Epileptic seizures may complicate many disease states. Under this heading are included those diseases in which seizures are a presenting or predominant feature.

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III. Epilepsies and syndromes undetermined as to whether they are focal or generalized A. With both generalized and focal seizures 1.Neonatal seizures 2. Severe myoclonic epilepsy in infancy 3. Epilepsy with continuous spikes and waves during slow-wave sleep 4. Acquired epileptic aphasia (LandauKleffner syndrome) B. Without unequivocal generalized or focal features

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IV. Special syndromes A. Situation-related seizures 1. Febrile convulsions 2.Seizures related to other identifiable situations, such as stress, hormones, drugs, alcohol, or sleep deprivation B. Isolated, apparently unprovoked epileptic events C. Epilepsies characterized by the specific modes of seizures precipitated D. Chronic progressive epilepsia partialis continua of childhood

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Stress, emotion Sleep/sleep deprivation Hyperventilation Fever Medications, metabolic disturbance Reflex epilepsy

Photic stimuli: TV, flashing lights, visual patterns Startle, music, reading, eating

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EEG MRI brain 1st Unexplained seizure does not necessitate AED treatment except:

Recognized epileptic syndrome with high probability of recurrence. Focal brain lesion.

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Absence Epilepsy Juvenile Myoclonic Epilepsy Benign Rolandic Epilepsy Infantile Spasms Lennox Gastaux

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age of onset 3-8 years abrupt cessation of activity with change of facial expression and blank gaze duration short usually < 15 seconds child returns to normal and no postictal period automatisms sometimes activated by hyperventilation characteristic EEG 3 Hz spike & wave treat with AEDs (Ethosuxsimide, Valproate, Topamax, and Lamictal) patients usually grow out of seizures by teen years

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3 Hz Spike & Wave

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Also called Janz syndrome First described in 1867 Triad includes myoclonic jerks, absence, & tonic clonic seizures Normal development Normal imaging

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A common epilepsy syndrome: 10-15% of all epilepsies Age of onset 12-18 years F=M Accounts for 25% of patients with idiopathic generalized epilepsies. Most have myoclonic jerks, 85% have GTCs, and 15-38% have absence

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EEG with 3-6 Hz multispike and wave Photosensitivity in 27%-41% Focal EEG abnormalities in up to 55% Triggers: AM wakening, lack of sleep, fatigue, ETOH, and fasting Requires life-long treatment Little data on effective treatment

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autonomic dominant onset 3-13yrs with peak 6-8 years usually nocturnal or during sleep infrequent episodes that awake the child with drooling, speech arrest, ipsilateral facial twitching or twisted to one side that are only minutes in duration can sometimes generalize development and exam are normal characteristic EEG that shows Midtemporal (T3,T4) and Central (C3,C4) spikes Treatment usually not indicated if infrequent but can treat with AEDs usually outgrown by 14 years

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onset 15mos-15years, usually 4-8 years initial seizure manifestations include visual hallucinations (flashing lights), blindness, amaurosis, micropsia, metamorphopsia, loss of consciousness can occur can have migraine and nausea afterward different seizure types (GTC, CPS, unilateral clonic) and occur mostly when transitioning from wakefulness to sleep EEG shows occipital spike & wave 1.5-2.5 Hz and eye opening enhances and sleep inhibits

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specific type of seizure seen in infancy and early childhood onset is predominantly in the first year of life, typically < 1 year characteristic EEG called hypsarrhythmia typical pattern is a sudden bending forward and stiffening of the body, arms, and legs.

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Spasms tend to begin soon after arousal from sleep. Individual spasms typically last for 1 to 5 seconds and occur in clusters, ranging from 2 to 100 spasms at a time. Infantile spasms usually stop by age 5, but are often replaced by other seizure types. West Syndrome is characterized by infantile spasms, hypsarrhythmia, and mental retardation.

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Etiology

Cerebral malformations 35%, Perinatal insult 15%, Metabolic 15%, Tuberous Sclerosis 10% Treatment usually starts with AEDs, steroids, ACTH, Vigabatrin, B6, Surgery (if lesions) Prognosis depends on etiology. Worse prognosis with symptomatic as many, 50%, go on to have other types of seizures Many develop mental retardation or delayed development.

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Childhood epileptic encephalopathy (Lennox-Gastaut syndrome [LGS]) is a devastating pediatric epilepsy syndrome constituting 1-4% of childhood epilepsies triad characterized by multiple types of seizures, mental retardation or regression, and characteristic EEG abnormal EEG with generalized slow spike-and-wave discharges (1.5-2 Hz)

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most common seizure types are tonic-axial, atonic, and absence seizures, but myoclonic, generalized tonic-clonic, and partial seizures can be observed. Seizures often are resistant to therapy. mean age at epilepsy onset is 3-5 years (range, 1 d to 14 y) 60% have underlying cause (TS, NF, perinatal insult) and 20% have history of Infantile Spasms diagnosis by history, PE, and EEG treatment is difficult
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Landau-Kleffner Syndrome onset 2-12 years acquired aphasia, verbal auditory agnosia, decreased spontaneous speech difficulty understanding speech and child stops talking several seizure types (GTC, Myoclonic, Absence) neuropsychological disturbances in >50% but intelligence is not affected
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sometimes the child is diagnosed with Autism or being deaf EEG is normal during wakefulness but during sleep there is spike & wave mostly in parietal and temporal lobes, sometimes electrical status of sleep treatment with AEDs and steroids shows good control recovery of language is variable and if onset is before 6 years there is better outcome less than 50% live independent lives

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LandauKleffner EEG Shows S&W

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The concern of the clinician is that epilepsy may be symptomatic of a treatable cerebral lesion. Routine investigation: Haematology, biochemistry (electrolytes, urea and calcium), chest X-ray, electroencephalogram (EEG). Neuroimaging (CT/MRI) should be performed in all persons aged 25 or more presenting with first seizure and in those pts. with focal epilepsy irrespective of age. Specialised neurophysiological investigations: Sleep deprived EEG, video-EEG monitoring. Advanced investigations (in pts. with intractable focal epilepsy where surgery is considered): Neuropsychology, Semiinvasive or invasive EEG recordings, MR Spectroscopy, Positron emission tomography (PET) and ictal Single photon emission computed tomography (SPECT)

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