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Epilepsy syndromes
• complex of signs and symptoms that define a
unique epileptic condition
Epileptic Syndromes
• Defined by:
– Clinical features
– Seizure type(s)
– Age of onset
– Family history
– EEG findings
– Diagnostic Investigations
– Treatment
– Prognosis
Electroclinical syndromes arranged by
age at onset
Neonatal period
Benign familial neonatal epilepsy (BFNE)
Early myoclonic encephalopathy (EME)
Ohtahara syndrome
Infancy
Epilepsy of infancy with migrating focal seizures
West syndrome
Myoclonic epilepsy in infancy (MEI)
Benign infantile epilepsy
Benign familial infantile epilepsy
Dravet syndrome
Myoclonic encephalopathy in nonprogressive disorders
• Childhood
• Febrile seizures plus (FS+) (can start in infancy)
• Panayiotopoulos syndrome
• Epilepsy with myoclonic atonic (previously astatic) seizures
• Benign epilepsy with centrotemporal spikes (BECTS)
• Autosomal-dominant nocturnal frontal lobe epilepsy
(ADNFLE)
• Late onset childhood occipital epilepsy (Gastaut type)
• Epilepsy with myoclonic absences
• Lennox-Gastaut syndrome
• Epileptic encephalopathy with continuous spike-and-wave
during sleep (CSWS)
• Landau-Kleffner syndrome (LKS)
• Childhood absence epilepsy (CAE)
• Adolescence – Adult
• Juvenile absence epilepsy (JAE)
• Juvenile myoclonic epilepsy (JME)
• Epilepsy with generalized tonic–clonic seizures
alone
• Progressive myoclonus epilepsies (PME)
• Autosomal dominant epilepsy with auditory
features (ADEAF)
• Other familial temporal lobe epilepsies
• Less specific age relationship
• Familial focal epilepsy with variable foci
(childhood to adult)
• Reflex epilepsies
Reflex epilepsy types
• Reflex epilepsies (REs) are identified as epileptic
seizures that are consistently induced by identifiable
and objective-specific triggers, which may be an
afferent stimulus or by the patient’s own activity. RE
may have different subtypes depending on the
stimulus characteristic.
• he stimuli can be external (light flashes, hot water),
internal (emotion, thinking), or both and should be
distinguished from triggering precipitants, which most
epileptic patients could report such as emotional
stress, sleep deprivation, alcohol, and menstrual cycle.
Refer:
https://www.ncbi.nlm.nih.gov/pmc/articles/P
MC5779309/table/t1-ndt-14-
327/?report=objectonly
Epilepsy syndromes
Epilepsy syndromes
Epilepsy syndromes

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Epilepsy syndromes

  • 2. • complex of signs and symptoms that define a unique epileptic condition
  • 3. Epileptic Syndromes • Defined by: – Clinical features – Seizure type(s) – Age of onset – Family history – EEG findings – Diagnostic Investigations – Treatment – Prognosis
  • 4. Electroclinical syndromes arranged by age at onset Neonatal period Benign familial neonatal epilepsy (BFNE) Early myoclonic encephalopathy (EME) Ohtahara syndrome Infancy Epilepsy of infancy with migrating focal seizures West syndrome Myoclonic epilepsy in infancy (MEI) Benign infantile epilepsy Benign familial infantile epilepsy Dravet syndrome Myoclonic encephalopathy in nonprogressive disorders
  • 5. • Childhood • Febrile seizures plus (FS+) (can start in infancy) • Panayiotopoulos syndrome • Epilepsy with myoclonic atonic (previously astatic) seizures • Benign epilepsy with centrotemporal spikes (BECTS) • Autosomal-dominant nocturnal frontal lobe epilepsy (ADNFLE) • Late onset childhood occipital epilepsy (Gastaut type) • Epilepsy with myoclonic absences • Lennox-Gastaut syndrome • Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) • Landau-Kleffner syndrome (LKS) • Childhood absence epilepsy (CAE)
  • 6. • Adolescence – Adult • Juvenile absence epilepsy (JAE) • Juvenile myoclonic epilepsy (JME) • Epilepsy with generalized tonic–clonic seizures alone • Progressive myoclonus epilepsies (PME) • Autosomal dominant epilepsy with auditory features (ADEAF) • Other familial temporal lobe epilepsies • Less specific age relationship • Familial focal epilepsy with variable foci (childhood to adult) • Reflex epilepsies
  • 7. Reflex epilepsy types • Reflex epilepsies (REs) are identified as epileptic seizures that are consistently induced by identifiable and objective-specific triggers, which may be an afferent stimulus or by the patient’s own activity. RE may have different subtypes depending on the stimulus characteristic. • he stimuli can be external (light flashes, hot water), internal (emotion, thinking), or both and should be distinguished from triggering precipitants, which most epileptic patients could report such as emotional stress, sleep deprivation, alcohol, and menstrual cycle.