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7/29/2019 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