Epilepsy

Dr. Kalpana MallaMD Pediatrics

Manipal Teaching Hospital

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Epilepsy

Definition:- Two or more unprovoked seizures (unrelated to fever or an acute cerebral insult)

occurring at an interval greater than 24 hrs apart

Exceptions –Multiple seizure within 24 hrs Episodes of status epilepticus** F. convulsion & neonatal seizures are excluded

Incidence of epilepsy 3%

Aetiology of Epilepsy

• Idiopathic 67.6%• Specific etiology - Identifiable in only 30% cases• Congenital……….20%

– HIE– Congenital brain anomalies

• Infection …………4.0%• Trauma………… 4.7%• Vascular …………1.5%• Neoplastic……….1.5%• Degenerative…….0.7%

ILAE- Epileptic seizures

• Partial seizures:1. Simple partial (consciousness retained) –

Motor, sensory, Autonomic, psychic2.Complex partial (consciousness impaired) *simple P followed by impaired conscious *Consciousness impaired at onset3. Partial seizures with secondary

generalization

( ILAE): using both clinical data and EEG

II. Generalized seizures A. Absence seizures (petit mal) B. Clonic seizures C. Tonic seizures D. Tonic-clonic seizures E. Atonic seizures F. Myoclonic seizuresI. Infantile spasms

III. Unclassified seizures which, due to inadequate data or classification

ILAE epilepsy & Epileptic syndromes

1. Localised - Simple Partial S Complex Partial S Syndromes:- a) Idiopathic age related -Benign childhood focal epilepsy with centrotemporal spikes (rolandic E)

b) Symptomatic- chronic progressive E Epilepsia partalis continua

ILAE epilepsy & Epileptic syndromes

2. Generalized – Same Syndromes : A) Idiopathic age related – childhood

absence, juvenile absence B) Cryptogenic -West syndrome (infantile spasms) - Lennox-Gastaut Syndrome - Myoclonic seizures

Simple Partial seizures

• Motor activity is the most common symptom

• Tend to involve the face, neck, extremities• Automatism do not occur, some c/o Aura• Persists -10-20 sec• Remain conscious can verbalize• No postictal phase• EEG-spikes or sharp waves uni or bilaterally

or multifocal spikes

Complex partial S • Aura may be +• Brief blank stare or sudden cessation/ pause

in activity• Period of altered consciousness may be brief

& infrequent• Automatisms are common 50-75%-• In infancy -lip smacking, chewing, swallowing

,excessive salivation (alimentary Automatisms)

Complex partial S • In older children-semipurposeful

uncoordinated and unplanned gestures-picking & pulling at

clothing or bed sheets, rubbing objects, walking or running in nondirective , repetitive , fearful fashion

• Secondary generalization may be +• Duration 1-2 min

Generalized seizures1.Absence S (petit-mal),simple, typical :- -Sudden cessation of motor activity with blank facial expression & flickering of the eyelids - Seizures are subtle - first clue to absence S

is often unexplained "daydreaming" and a decline in school performance recognized by a teacher

Absence S (petit-mal),simple, typical - Common age - 4 to 8 yrs or early adolescence, - Prevalent in girls - Incidence 15 to 20% of children - No aura, no postictal state - Duration 30 sec - Countless seizures in a day

Generalized seizures

Absence S (petit-mal),simple, typical…….. - Body tone normal but head may fall

slightly forward -Automatism frequently present -EEG-3/sec spike and generalized wave

discharge

Atypical Absence Seizures

• Longer duration • Seizure is accompanied by more obvious motor

signs consisting of myoclonic movements of face, fingers and extremities, focal or lateralizing features

• Mental retardation may be present• Less responsive to anticonvulsants • The EEG - shows a generalized, slow spike-and-wave

pattern with a frequency of =2- 2.5cycles/sec

2.Generalized tonic – clonic grand –mal)

Aura – Due to partial onset of seizure , lasts sec –min eg olfactory hallucination, epigastric discomfort, deja-vu, jerking of one limb

Tonic phase -rapid discharging of motor cortex cells cause tonic contraction of muscles throughout the body - skeletal muscle undergoes sustained spasm

Tonic phase….• Skeletal muscle undergoes sustained spasm - Laryngeal muscles ,muscles of expiration - forces air

out of the lungs through a partially closed glottis - produce "ictal cry." (shrill cry)

- Respirations are impaired, secretions pool in the oropharynx, and cyanosis develops

- Muscle rigidity: more in antigravity muscles uprolling of eyes or deviated to sides

Tonic phase….

• Contraction of the jaw muscles - biting of the tongue. • Lasts 10-30 sec

Generalized tonic – clonic grand –mal)

Clonic phase- 1-5 min - Gradually slowing discharge of cortical cells, rhythmic clonic contractions alternating with relaxation of muscle groups - Marked enhancement of sympathetic tone -

increases in heart rate, blood pressure, pupil dilated, Loss of sphincter control

Postictal phase• Characterized by :

- Semicomatose- Unresponsiveness - deep sleep for 30min to 2hrs

• Muscular flaccidity, loss of corneal reflex • Extensor plantar responses• Loss of sphincter control -incontinence , tongue biting • Lasts 30 min – 2 hrs

Generalized tonic – clonic grand –mal)

Post-ictal – 30 min – 2 hrs Deep unconsciousness, flaccid limbs, loss of corneal reflexes, extensor plantar responses, Excessive salivation cause stridorous

breathing and partial airway obstruction - Patients gradually regain consciousness

• Bi-frontal headache, confusion, aching muscles ,sometimes automatic behavior, occasional violence (2-21/2hours)

Postictal confusion

• Bifrontal headache• Confusion• Aching muscles• Sometimes automatic behavior • Occasinal violence• Lasts - 2-2 ½ hours

GTCS - Precipitated by

- Infections - Excessive fatique - Emotional stress - Various drugs -theophylline, anti-

psychotropic drugs, cipro, benzodiazepines, amphetamine, cocaine, alcohol, flumazenil

- Forgetting to take anticonvulsants

The EEGDuring the tonic phase - shows a - progressive increase in generalized low-voltage fast activity, followed by generalized high-amplitude, polyspike dischargesDuring clonic phase - the high-amplitude activity is typically interrupted by slow waves to create a spike-and-wave pattern

The postictal EEG - shows diffuse slowing that gradually recovers as the patient awakens

3.Myoclonic epilepsies of childhood• Repetitive seizures with brief ,symmetric

muscular contractions with loss of body tone and falling or slumping forward

• Subgroups:A. Benign myoclonus of infancy -confined to neck, trunk, extremities - EEG normal - Good prognosis - improves by age 2yrs - No medications needed

B. Typical myoclonic epilepsy of early childhood

-Normal birth history, normal milestones

-starts at 6 mo -4 yrs peak age-2 ½ yrs-Family history + in 1/3rd cases-EEG- fast spike waves-50% seizure free few yrs after

treatment-Learning language problem,

emotional, behavioral disorder- MR may occur in some

C. Complex myoclonic epilepsies

• Begin-1st yr of life, • Starts as generalized seizure associated

with URTI• Presents as status epilepticus-poor

prognosis• 1/3rd have delayed development,• H/o birth asphyxia ,microcephaly• No family h/o epilepsy• EEG- interictal slow spike waves• Refractory to anticonvulsants

D. Juvenile myoclonic epilepsy (janz syndrome)

• Begin at age 12-16 yrs• Initially hair combing, tooth brushing

become difficult• Few yrs later with myoclonus have GTC

seizure• EEG –abnormal• Responds to valproate required

lifelong

E. Progressive myoclonic epilepsies

• Neurologic abnormalities-cerebellar & extrapyramidal signs ++

• Mental deterioration is characteristic feature

• Genetic disorder –grave prognosis• EEG-abnormal• Valproic acid + clonazepam is effective

4.Infantile spasms

• 4-8 mo of life• Brief symmetrical contractions of

neck, trunk, extremities-sudden dropping of head & flexion of arms - ‘Salaam fit’

• EEG-Hypsarrhythmic pattern –diffuse high voltage slow spike and chaotic activity

4.Infantile spasms

• Types – • Flexor spasms• Extensor spasms-least common• Mixed infantile spasms-most

common

• More tendency when drowsy or immediately on awaking

4.Infantile spasms

Two groups1. Crytogenic -10-20% cases - Normal birth history - Normal development - Neurologic exam, CT,MRI head-

normal - No risk factors

4.Infantile spasms

2. Symtomatic- - 80-90% Related to several factors:- Antenatal-HIE, cong infections, inborn

errors of metabolism, neurocutaneous syndromes, Cong anomalies of brain

Post natal–CNS infections, head Trauma,HIE

-80-90% -MR

Lennox –Gastaut Syndrome

• Onset –late infancy or childhood• Mixed seizure- myoclonic, atypical

absence ,generalized tonic-clonic, partial• Prognosis unsatisfactory• Triad-1.Atypical absences, axial tonic seizures, drop

attacks2. Typical EEG pattern - Slow spike and waves

on awake EEG & 10Hz bursts during sleep3.Psychomotor retardation

Management

• 1st seizure: Blood sugar, electrolytes,calcium, EEG, CT,MRI,CSF-when indicated** Normal-Negative family history-No

treatment ,only close observation **Abnormal-treat underlying cause/AED if

necessary**Normal except EEG abnormal-Idiopathic

Epilepsy-treatment

Did the child had seizure ?

NO YES

SEIZURE MIMICS INITIAL RECURENTSEIZURE SIEZURE

RBS , electrolyte,EEG?, CT?, CSF?

ABNORMALSYMPTOMATIC SIEZURE

TREAT underlying causeAED if necessary

NORMAL

Isolated 1st sizureNo family historyNo TREATMENT Close observation

NORMAL EXCEPT EEGIDIOPATHIC EPILEPSY

Drug complianceImproper drugMetabolicicSolDegenerative CNSDrug interaction

NORMAL EXCEPT EEGIDIOPATHIC EPILEPSY

Classify seizure type

Good control Poor control Regular follow up HospitalizeAED drug levels EEG , video monitoringMonitor toxicity Readjust drugsEEG CT, MRI, FREQUENT

FOLLOW UPS

When To Initiate AED Therapy

(1) An abnormal neurologic examination(2) Seizures presenting as status epilepticus (3) Postictal Todd's paralysis (4) A strong family history of seizures (5) An abnormal EEG

• Antiepileptic drug therapy should be started in any patient with recurrent seizures of unknown etiology

Selection Of AED

• Carbamazepine, phenytoin, or lamotrigine is currently the initial drug of choice for partial seizures, including those that secondarily generalize

• Valproic acid is currently - best initial choice for primarily generalized, tonic-clonic seizures.

• Lamotrigine, followed by carbamazepine and phenytoin, are suitable alternatives.

• Valproic acid is effective in - absence, myoclonic, and atonic seizures - drug of choice for generalized epilepsy syndromes having mixed seizure types

Treatment

1.Establish diagnosis2. Decide about need for AED3. Choice of most suitable drug-age, type

of seizure, economic condition of patient4.Proper F/U**Go for monotherapy**start with small dose & gradually

increase to maintenance dose over few weeks

AED Choice Based on S. TypeSeizure type

First choice Others

Partial S CBZ Phenytoin, Valproic acid, Phenobarbital,

Tonic-clonic

Valproic acid Topiramate, Phenobarbital, Lamotrigine, Zonisamide, Felbamate,phenytoin ,carbamazepine

Atonic Valproic acid Topiramate, Lamotrigine, Zonisamide,Felbamate

AED Choice Based on S. Type

Seizure type

First choice Others

Childhood Absence epilepsy

Ethosuximide Lamotrigine,valproic acid, Zonisamide, clonazepamfelbamate

Juvenile Absence Epilepsy

Valproic acid Lamotrigine, topiramate, zonisamide

Infantile spasm

ACTH, Vigabatrin

Topiramate, Zonisamide ,prednisolone,valproic acid

AED Choice Based on S. Type

Seizure type

First choice Others

Partial S CBZ Phenytoin, Valproic acid, Phenobarbital,

Tonic-clonic

Valproic acid Topiramate, Phenobarbital, Lamotrigine, Zonisamide, Felbamate,phenytoin ,carbamazepine

Atonic Valproic acid Topiramate, Lamotrigine, Zonisamide,Felbamate

AED Choice Based on Epilepsy Type

Epilepsy type

First-choice Others

Juvenile Myoclonic Epilepsy

Valproic acid Lamotrigine, topiramate, zonisamide, felbamate ,phenobarbitone,clonazepam

Benign Rolandic Epilepsy

Carbamazepine Gabapentine,topiramate,lamotrigine,valproic

acid,phenytoin

Lennox-Gastaut

Valproic acid Topiramate,lamotrigine, zonisamide, felbamate,clonazepam

Adjunctive T when S poorly controlled

• Gabapentine, clobazam, lamotrigine, topiramate, vigabatrin

• Surgical Rx-Resection of corpus callosum, focal resection of parts of cerebral cortex involved as epileptic foci

WHEN TO DISCONTINUE THERAPY

Duration: should be invidualized as there is no unanimity in literature

(1) Complete medical control of seizures for 1 to 5 years

(2) In children seizures that are easily controlled and have no underlying structural lesions, no MR, no neurological deficits need 2-3 yrs seizure free course

(3) Normal EEG

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