1. Pediatric Epilepsies
Ali Alwadei, MD
R4-Peds Neurology

2. Outline Definitions Classifications General Approach to Epilepsies
Etiologies / DDx (Sz Imitators)
Common Pediatric Epilepsy Syndromes

3. Definitions
Seizure
Convulsion
Epilepsy
Epilepsy Syndrome

4. Definitions Seizure/Convulsion
Seizure: Abnormal Electrical Activity in the brain, its manifestations depend on where the abnormal electrical activity takes place in the brain (i.e. localization: Motor/convulsive, sensory/visual, autonomic).
abbreviated sz.
Convulsion: motor seizure
Epilepsy
2 or more (RECURRENT) unprovoked seizures.

5. Definitions Epilepsy Syndrome
refer to ‘‘a complex of symptoms and signs that define a unique epileptic condition.’’
denotes specific constellations of
clinical seizure type(s)
EEG findings
other characteristic clinical features, such as
age at onset
course of epilepsy
associated neurologic and neuropsychological findings
underlying pathophysiologic or genetic mechanisms.

6. Clinical Classification
Partial
Simple (without impairment of consciousness)
Complex (with impairment of consciousness)
Gelastic
Reflex
Generalized
Absence/Atypical Absence
Myoclonic/spasms/-ve myoclonus
GTC
Tonic/Atonic
Clonic with or without tonic features
Partial with 2ry Generalization

7. Precipitating stimuli for reflex seizures
Visual stimuli
Flickering light -color to be specified when possible
Patterns
Other visual stimuli
Thinking
Music
Eating
Praxis
Somatosensory
Proprioceptive
Reading
Hot water
Startle
ILAE,
Benjamin G. Zifkin and Frederick Andermann

8. Clinical Classification Status Epilepticus
Generalized status epilepticus
Absence status epilepticus
Myoclonic status epilepticus
GTC status epilepticus
Tonic status epilepticus
Clonic status epilepticus
Focal status epilepticus
Epilepsia Partialis Continua of Kojenikov (Kojewnikow syndrome)
Hemiconvulsive status with Hemiparesis
Aura Continua
Limbic status epilepticus (psychomotor status)

9. Etiological Classification
Idiopathic presumed genentic
Symptomatic
Cryptogenic probably symptomatic

10. Classification Etiology Dev MRI Unknown ? genetic NL Known
Idiopathic presumed genentic
Unknown
? genetic NL
Symptomatic
Known
Usually structural
AbN
Cryptogenic probably symptomatic
?abN-3T

16. General Approach to Epilepsies
Aim to classify the patient under specific:
Clinical class ( SPC, CPS, Gen…etc)
Etiologic class (idio, symp, crypto), more difficult
Syndrome if possible. Much more difficult Hx Ex
EEG
MRI

17. General Approach to Epilepsies
Identification of a specific syndrome is important to define the best treatment and accurately prognosticate long-term outcome.
Most syndromes recognized in epilepsy are genetic and developmental disorders that begin in the pediatric years.

18. Hx Age and sex FHx: if +ve ? genetic Perinatal Hx: e.g. HIE
Developmental: helpful to differentiate idiopathic vs Sympto /Cryptogenic
Vaccination and relation to sz onset
PHx
Meds (current and previous)
Allergies

19. Hx HPI: Age at 1st sz ( onset) if known epileptic Aura
Sz types (semiology) Partial vs Generalized.
Sz Frequency
Sz Duration
Post-Ictal
Urinary/Fecal incontinence
Tongue Biting
R/O Sz imitators ( see DDx)
Previous work up

22. Exam Full neuro exam including: May help localize
Head C.
Neuro-Cutaneous Stigmata
May help localize
Usually not helpful in epilepsy

23. W/up Consider: EEG ( sensitivity, SD, prolonged) blood and urine: CSF
SMA10
S. glucose
LFTs
Metabolic, Ammonia
CGH micro-array
Toxic screening
CSF
MRI, 3-Tesla magnet.

25. MRI
Performing an MRI is important particularly when partial seizures are suspected.
MRI has been shown to be superior to CT as the overall resolution for potential epileptogenic lesion detection is superior.
many patients with epilepsy do not have identifiable lesions on MRI

26. Common examples include:
mesial temporal sclerosis (MTS)
primary brain neoplasms
remote trauma
stroke
developmental abnormalities

31. Common Pediatric Epilepsy Syndromes
IDIOPATHIC GENERALIZED EPILEPSY (IGE) SYNDROMES
Infantile Onset:
Benign Neonatal Convulsions (BNC/BNS)
Benign Familial Neonatal Sz (BFNS)
Benign Ideopathic Neonatal Sz (BINS)
Benign Myoclonic Epilepsy of Infancy (BMEI)
Childhood Onset:
Generalized Epilepsy With Febrile Seizures Plus (GEFS+)
Myoclonic Astatic Epilepsy of Doose (MAE)
Childhood Absence Epilepsy (CAE)

32. Common Pediatric Epilepsy Syndromes
IDIOPATHIC GENERALIZED EPILEPSY (IGE) SYNDROMES
Adolescence Onset:
Juvenile Absence Epilepsy (JAE)
Juvenile Myoclonic Epilepsy (JME)
Generalized Tonic-Clonic Sz (GTCS) upon awakening

33. Common Pediatric Epilepsy Syndromes
IDIOPATHIC PARTIAL EPILEPSY SYNDROMES
Benign Epilepsy of Childhood With Rolandic Spikes (BECRS)
Benign Occipital Epilepsy Early and Late (BOE)
Autosomal Dominant frontal lobe epilepsy ( ADNFLE)
Familial Temporal lobe epilepsy ( FTLE)

34. Common Pediatric Epilepsy Syndromes
SYMPTOMATIC EPILEPSY SYNDROMES
Infantile Onset:
Early Myoclonic Encephalopathy (EME)
Early Infantile Epileptic Encephalopathy (EIEE) or Ohtahara Syndrome (OS)
West Syndrome (WS)
Dravet Syndrome (SMEI)
Childhood Onset:
Lennox-Gastaut Sydrome (LGS)
Landau-Kleffner Syndrome (LKS)
Continuous Spike-Wave in sleep (CSW-SWS)
Rasmussen Encephalitis
Varying Age:
Progressive Myoclonic Epilepsy (PMEs)

35. Benign Familial Neonatal Sz (BFNS)

36. Benign Ideopathic Neonatal Sz (BINS)

37. Benign Myoclonic Epilepsy of Infancy (BMEI)

38. Generalized Epilepsy With Febrile Seizures Plus (GEFS+)

39. Myoclonic Astatic Epilepsy of Doose (MAE)

40. Childhood Absence Epilepsy (CAE)

41. Juvenile Absence Epilepsy (JAE)

42. Juvenile Myoclonic Epilepsy (JME)

43. Generalized Tonic-Clonic Sz (GTCS) upon awakening

44. Benign Epilepsy of Childhood With Rolandic Spikes (BECRS)

45. Benign Occipital Epilepsy Early and Late (BOE)

46. Benign Occipital Epilepsy Early and Late (BOE)

47. Autosomal Dominant frontal lobe epilepsy ( ADNFLE)

48. Familial Temporal lobe epilepsy ( FTLE)

49. Early Myoclonic Encephalopathy (EME)

50. Early Infantile Epileptic Encephalopathy (EIEE) or Ohtahara Syndrome (OS)

51. West Syndrome (WS)

52. Dravet Syndrome (SMEI)

53. Lennox-Gastaut Sydrome (LGS)

54. Landau-Kleffner Syndrome (LKS)

55. Continuous Spike-Wave in sleep
(CSW-SWS)

56. Rasmussen Encephalitis

57. Progressive Myoclonic Epilepsy (PMEs)

58. Progressive Myoclonic Epilepsy (PMEs)

59. Thank You