Epileptic Syndromes in paediatrics

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    EPILEPTIC SYNDROMES Benign focal epilepsy

    Benign neonatal convulsions

    Juvenile myoclonic epilepsy (of janz)

    Infantile spasms (west syndrome)

    Lennox-Gastaut syndrome

    Atonic seizures

    Acquired epileptic aphasia (landau kleffner syndrome)

    Rasmussen encephalitis

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    Benign focal epilepsy Also called as the rolandic epilepsy

    Age-5-10years.

    Occurs during sleep or on awakening

    Focal motor seizures mostly in face and arms

    Common symtoms were drooling and rythmic guttural

    sounds

    Responses to anticonvulsants therapy, epilepsy resolves

    after puberty

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    Infantile spasms

    Peak age is 3-8months Occurs when the child is awakening from or going to

    sleep.

    Occurs in clusters a day, can be of variable duration.

    When flexion of the thighs and crying are prominent ,canbe mistaken for colic.

    If not treated,can lead to autistic behaviour.

    Tuberculous sclerosis is the commonest cause

    Treatment- ACTH, oral corticosteroids, benzodiazepines,

    valproic acid and vigabatrin.

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    Hysarrythmia in infantile syndrome

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    Lennox-Gastaut syndrome

    Occurs less than 5 years of age Multiple seizure types

    Children have underlying brain injury and malformations

    Good response to valproic acid

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    Astatic-akinetic(atonic seizure)

    Onset-1-3 years of age Duration-1-4 seconds

    Tend to fall to ground and

    dropping of head. Tonic

    component

    Repeated head injury can be

    prevented by using a safety

    helmet.

    50 or more daily seizures.

    Developmental delay.

    Tuebrculous sclerosis-

    common cause

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    Landau-kleffner syndrome

    (acquired epileptic aphasia) Abrupt loss of previously acquired language.

    Auditory agnosia

    Seizures in temporal lobe is responsible for language loss.

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    Rasmussen encephalitisOnset-6-10 years

    Focal persistent motor seizure activity

    Hemiplegia and cognitive deteriorationHemispherectomysuccessful therapy

    seizure eradication

    prevent cognitive deterioration

    Permanent hemiparesis is an ineveitable consequence.

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    Ohtahara/W

    est

    Ohtahara/W

    est

    Lennox-

    GastautCSWS

    Malignant

    rolandic

    Landau

    Kleffner

    Age of onsetFirst months

    of life24 years 45 years

    Before 6

    years38 years

    Main seizure

    type

    Epileptic

    spasms

    Atypical

    absences,

    myoclonic,

    tonic

    Often

    nocturnal at

    the

    beginning;

    both partial

    andgeneralized

    Sensorimoto

    r focal, often

    nocturnal

    Rare and

    occasional,

    often

    nocturnal

    EEG pattern

    Burst-

    suppression

    /hypsarhyth

    mia

    Slow SW,

    fast

    activities

    ESES ESES ESES

    Usual

    abnormal

    area

    Posterior Anterior (Pre)frontal CentralParieto-

    temporal

    Aetiology

    Symptomati

    c in most

    cases

    Symptomati

    c, about

    30%cryptogenic

    Cryptogenic,

    sometimes

    symptomatic

    Cryptogenic

    Cryptogenic

    in most of

    cases

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    CSWSMalignant rolandic

    epilepsy

    LandauKleffner

    syndrome

    Aetiology

    Cryptogenic (normal

    development before) or

    symptomatic (pre-

    existing encephalopathy)

    Cryptogenic (gliosis or

    dysplasia if cortical

    excision performed)

    Rarely symptomatic

    Attention Reduced attention span Difficulties to maintain Deficits

    Memory Poor performance

    Difficulties in verbal

    learning and working

    memory

    Residual impairment in

    verbal short-term

    memory

    Executive functionsImpaired temporospatial

    orientation

    Preserved at the

    beginning

    BehaviourAggresiveness,

    hyperkinesiaHyperactivity Excitability, hyperactivity

    Language Expressive aphasia Dysarthria or dysphasia Auditory agnosia,acquired aphasia

    Seizures

    Often nocturnal seizures

    at the beginning; partial/

    generalized: unilateral or

    bilateral clonic,

    generalized; never tonic

    Somatomotor often

    nocturnal at the

    beginning; then negative

    myoclonus during

    wakefulness, atypicalabsences, epileptic falls,

    Eyelid myoclonia, eye

    blinking, atypical

    absences, head drops

    and atonic fits in upper

    limbs, automatisms,

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    References http://www.sciencedirect.com/science/article/pii/S10591

    31106000367#

    http://www.epilepsy.com/epilepsy/epilepsy_infantilespas

    ms

    http://emedicine.medscape.com/article/1176735-

    overview

    Nelson-essentials of pediatrics,6th edition

    http://www.sciencedirect.com/science/article/pii/S1059131106000367http://www.sciencedirect.com/science/article/pii/S1059131106000367http://www.epilepsy.com/epilepsy/epilepsy_infantilespasmshttp://www.epilepsy.com/epilepsy/epilepsy_infantilespasmshttp://emedicine.medscape.com/article/1176735-overviewhttp://emedicine.medscape.com/article/1176735-overviewhttp://emedicine.medscape.com/article/1176735-overviewhttp://emedicine.medscape.com/article/1176735-overviewhttp://emedicine.medscape.com/article/1176735-overviewhttp://www.epilepsy.com/epilepsy/epilepsy_infantilespasmshttp://www.epilepsy.com/epilepsy/epilepsy_infantilespasmshttp://www.sciencedirect.com/science/article/pii/S1059131106000367http://www.sciencedirect.com/science/article/pii/S1059131106000367