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

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

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 ,can be 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.

Hysarrythmia in infantile syndrome

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

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 sclerosiscommon cause

Landau-kleffner syndrome (acquired epileptic aphasia)

Abrupt loss of previously acquired language.
Auditory agnosia Seizures in temporal lobe is responsible for language loss.

Rasmussen encephalitis
Onset-6-10 years Focal persistent motor seizure activity Hemiplegia and cognitive deterioration Hemispherectomy successful therapy seizure eradication prevent cognitive deterioration Permanent hemiparesis is an ineveitable consequence.

Ohtahara/W LennoxOhtahara/W est Gastaut est First months of life

CSWS

Malignant rolandic

Landau Kleffner

Age of onset

24 years

45 years Often nocturnal at the beginning; both partial and generalized

Before 6 years

38 years

Main seizure Epileptic type spasms

Atypical absences, myoclonic, tonic

Rare and Sensorimoto occasional, r focal, often often nocturnal nocturnal

EEG pattern

Burstsuppression /hypsarhyth mia Posterior

Slow SW, fast activities

ESES

ESES

ESES

Usual abnormal area

Anterior Symptomati c, about 30% cryptogenic

(Pre)frontal Cryptogenic, sometimes symptomati c

Central

Parietotemporal Cryptogenic in most of cases

Aetiology

Symptomati c in most cases

Cryptogenic

CSWS Cryptogenic (normal development before) or symptomatic (preexisting encephalopathy) Reduced attention span

Malignant rolandic epilepsy Cryptogenic (gliosis or dysplasia if cortical excision performed) Difficulties to maintain Difficulties in verbal learning and working memory

LandauKleffner syndrome

Aetiology

Rarely symptomatic

Attention

Deficits Residual impairment in verbal short-term memory Preserved at the beginning

Memory

Poor performance
Impaired temporospatial orientation Aggresiveness, hyperkinesia Expressive aphasia

Executive functions Behaviour

Language

Hyperactivity
Dysarthria or dysphasia

Excitability, hyperactivity
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, atypical absences, epileptic falls,

Eyelid myoclonia, eye blinking, atypical absences, head drops and atonic fits in upper limbs, automatisms,

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

31106000367# http://www.epilepsy.com/epilepsy/epilepsy_infantilespas ms http://emedicine.medscape.com/article/1176735overview Nelson-essentials of pediatrics,6th edition