1. Epilepsy Syndromes
Dr. Rumi Myedull Hossain
FCPS Part II student(Paediatric Neurology & Development)
National Institute of Neuroscience & Hospital
Dhaka.

2. Epilepsy Syndromes
• Epileptic syndrome is a group of signs and symptoms that share a
common pathogenesis, prognosis, and response to treatment.

3. Neonatal Onset
• Benign (idiopathic) neonatal seizures (fifth-day fits)
• Benign familial neonatal epilepsy
• Early infantile epileptic encephalopathy (Ohtahara syndrome)
• Early myoclonic encephalopathy

4. Infantile Onset
• Myoclonic epilepsy in infancy (benign Dravet variant)
• Benign epilepsy of infancy
• Benign familial infantile epilepsy
• Epilepsy of infancy with migrating focal seizures
• Hemiconvulsion-hemiplegia-epilepsy syndrome
• West syndrome (infantile spasms, hypsarrhythmia; to be distinguished from
benign myoclonus of early infancy, which is not an epilepsy)
• Severe myoclonic epilepsy of infancy (classic Dravet syndrome)
• Myoclonic encephalopathies in nonprogressive disorders

5. Childhood
• Lennox-Gastaut syndrome
• Panayiotopoulos syndrome (early-onset childhood occipital epilepsy)
• Myoclonic-atonic (formerly astatic) epilepsy (Doose syndrome)
• Benign epilepsy with centrotemporal spikes (benign rolandic epilepsy)
• Late-onset childhood occipital epilepsy (Gastaut type)
• Epilepsy with myoclonic absences (Tassinari syndrome)
• Genetic epilepsy with febrile seizures plus (can begin in infancy)

6. • Epileptic encephalopathy with continuous spike and wave in slow
sleep
• Acquired epileptic aphasia (Landau-Kleffner syndrome)
• Childhood absence epilepsy (pyknolepsy)
• Generalized epilepsy with eyelid myoclonia (Jeavons syndrome)

7. Adolescence to Adult Onset
• Juvenile absence epilepsy
• Juvenile myoclonic epilepsy
• Epilepsy with generalized tonic-clonic seizures alone
• Progressive myoclonus epilepsies
• Mesial temporal lobe epilepsy with hippocampal sclerosis
• Autosomal dominant focal epilepsy with auditory features
• Autosomal dominant nocturnal frontal lobe epilepsy

8. Syndromes With Less Specific Age Relationship
• Familial focal epilepsy with variable foci (childhood to adult)
• Reflex epilepsies

9. Panayiotopoulos Syndrome
• Syndrome onset is usually between 3 and 6 years of age, although a
wide range, from 1 to as late as 14 years, has been described.
• The most prominent feature is an autonomic component, and the
classic scenario is a seizure with recurrent vomiting with onset during
sleep.
• The events can be prolonged (ie, at least 5 minutes and sometimes
even hours)
• Nausea, vomiting, and eye deviation with preserved consciousness

10. • Other autonomic symptoms include bowel or bladder incontinence,
pallor, pupillary changes, and syncope.

11. Interictal EEG
• Normal background with high-amplitude sharp- and slow-wave
complexes, similar in morphology to those seen in benign childhood
epilepsy with centrotemporal spikes.
• There is great variability in location. Occipital localization is the most
common, but all other brain regions may be involved.
• EEG abnormalities in PS are accentuated by sleep.

14. Childhood Occipital Epilepsy of Gastaut
• The seizures of COE of Gastaut are always of occipital lobe onset.
• primarily manifest with visual seizures, which are the most typical and
usually the first ictal symptoms.
• The main types of seizures: visual manifestations, motor seizures,
migraine-like symptoms, and less frequent autonomic manifestations.
• Visual seizures: elementary and complex visual hallucinations, visual
illusions, blindness or partial visual loss, and sensory hallucinations.

15. • Motor seizures: Deviation of the eyes, head version, forced eyelid
closure and blinking.
• Migraine like symptoms: Ictal or postictal headache occurs in 30%–50%
of patients.

16. EEG findings
• The EEG shows occipital paroxysms occur when the eyes are closed
and disappear or attenuate upon eye opening.
• EEGs with random occipital spikes, sometimes occurring only during
sleep, are frequent.
• A small number of patients with COE of Gastaut have rare occipital
spikes and consistently normal EEGs.
• Centrotemporal, frontal, and giant somatosensory spikes are much less
frequent than in PS

18. Severe Myoclonic Epilepsy of Infancy
(Dravet Syndrome)
• Seizures typically begin in the first year of life in a normal infant
without pathological antecedents.
• A positive family history is frequently present.
• Seizure accompanied by mild hyperthermia; clonic, generalized,
unilateral or predominating on one side of the body, and change from
one seizure to the next.
• In some patients, isolated episodes of focal myoclonia are observed
before the appearance of the first convulsive seizure.
• The first convulsive seizure can be afebrile, after a vaccination, a bath,
or during a cold.

20. Interictal EEG features
• The interictal EEG is usually normal at the onset.
• It may display a diffuse or unilateral slow background if recorded after
a prolonged seizure.
• In some patients, generalized spike-wave discharges are elicited by
intermittent photic stimulation.
• Rhythmic 4–5-Hz theta activity may be present in the centroparietal
areas and the vertex.

22. Benign Rolandic epilepsy
• Benign Epilepsy With Centrotemporal Spikes (BECTS)
• Nearly 25% of childhood epilepsy
• The usual age of onset is 4 to 11 years, with a peak at age 7 to 8 and
male predominance.
• The timing of seizures is usually shortly after sleep onset or just before
awakening, although about one-fourth of patients may have seizures
only during the awake state.
• Seizures tend to be infrequent, and up to 20% of patients will have
only a single seizure.

23. • The symptomatology: begin with paresthesia on one side of the tongue
or mouth, followed by dysarthria or gagging-type noises, jerking of
the ipsilateral face, and excessive drooling.
• Secondary generalization may occur.
• Cognition remain within normal range.

24. EEG findings
• The spike may have a phase reversal in the centro-temporal or parietal
regions but less commonly in the frontal or the vertex areas.
• A more posterior predominance is often observed in the youngest
subjects.
• The most striking finding of the centro-temporal spikes is their
significant increase in frequency during light NREM sleep.

27. Childhood Absence Epilepsy (Pyknolepsy)
• This syndrome comprises approximately 15% of the childhood-onset
epilepsies.
• Typical age of onset between 4 and 10 years with a peak between 5
and 7 years.
• Affects girls more frequently than boys.
• Clinically characterized by the typical absence seizure, meaning
abrupt impairment of consciousness, often with associated
behavioral arrest, staring, eye fluttering, or automatisms.

28. • The duration is usually 10 seconds but longer episodes may occur.
• Hyperventilation at the bedside is especially valuable in diagnosis
and, to some extent, for follow-up for medication effectiveness.

29. EEG features
• The classic EEG signature of typical absence seizures in childhood
absence epilepsy is the generalized bilaterally synchronous and
symmetric regular 3 Hz spike-and-wave paroxysms of abrupt onset
and offset.
• The discharges tend to be frontal predominant as opposed to truly
generalized.