1. Introduction To Epilepsy Semiology diagnosis Treatment
M. Scott Perry, M.D.
Emory University
April 18, 2007
September 18, 2006

2. Objectives Recognize different types of seizures.
Discuss workup for new onset seizures
Learn classification of epilepsy types based on history, seizure type, MRI, and EEG findings
Review common treatments used in epilepsy
Learn prognosis based on epilepsy type
Briefly review some frequently asked questions

3. Spells Seizure Symptomatic Electrolytes Trauma Ingestion Recurrent
Seizure Equivalents
GERD
Breath Holding
Infantile Masturbation
Syncope
Benign Sleep Myoclonus
Symptomatic
Electrolytes
Trauma
Ingestion
Recurrent
(Epilepsy)

4. Case 1

6. Seizure Imitators Benign Neonatal Sleep Myoclonus
Myoclonic jerks are focal, multifocal, unilateral or bilateral
1-5 hz, distal>proximal
Begins in first weeks, diminishes by 2nd month, generally gone by 6 months
Episodes may be exacerbated by benzos

7. Seizure Imitators Breath Holding Spells
Incidence: 4.6% (population study, N=4980)
Onset: 6-18 months
90% resolve by age 6y
cyanotic and pallid

8. CYANOTIC BREATH-HOLDING SPELLS
60 % are cyanotic
stimulus triggered (anger, frustration)
short cry
breathing interrupted in expiration
cyanotic, limp, LOC
+/- sleep

9. COMPLICATED BREATH-HOLDING SPELLS
Breath-holding spells + seizure-like activity
usually more prolonged
15% have complicated features
clonic activity follows LOC
stiffening

10. Seizures: What information is useful?
What was the patient doing when it started? Unresponsive?...are you sure? Asleep or awake?
Tell us exactly what you saw:
E.R.B.S.A.O?
Does it make anatomical sense? Same side, both sides, just arms, etc.
How long did it last?

11. Clinical Characteristics of Seizures in Neonates (Scher, et al 1989)
No accepted classification for neonatal seizures
80 neonates with suspicious movements. Only 8 had electrographic seizures.
Focal/multifocal clonic: 44% epileptic
“Subtle seizures”-roving eye movements, arrest of behavior, lip smacking, autonomic-30%
Tonic(focal or generalized) 8%
Myoclonic 7%

12. Clinical Characteristics of Seizures in Neonates (Scher, et al. 1993)
92 neonates with electrographic seizures (345 EEG recordings)
48% had electroclinical event
Subtle 71%, clonic 41%, myoclonic 20%, tonic 9%
34% with only electrographic events
17/90 (19%) of paralyzed neonates had electrographic events

13. Clinical Seizures in Neonates
Gen. Tonic Clonic seizures don’t happen in neonates.
69 infants, 101 seizures, only 4 resembled GTCS, none truly were. (Nordli, et al.)
Duration- average duration 2.25 minutes. Usually shorter, rarely longer. Intertictal recovery 8 minutes (Clancy and Legido, 1987)
Status Epilepticus- clinical SE is rare, electrographic may not be
487 seizures, only 2 SE (Clancy, et al)
33% FT infants, 9% PT (Scher, et al)

14. Seizure Semiology of Neonates
Focal/Multifocal Clonic
Tremors
Subtle
Tonic

15. Seizure Types Partial Generalized Simple Partial Complex Partial
Partial Secondarily
Generalized

16. Simple partial Preserved consciousness Isolated motor/sensory
Complex partial involves loss of consciousness

17. Partial Secondarily Generalized
Starts partial, rapidly spreads
You have to ask the questions to get the answers

18. Partial Seizure Clues Contralateral
Head Deviation, Eye Deviation, Dystonic Posturing, Unilateral Clonic Activity, Postictal Paralysis
Ipsilateral
Automatisms, Eye Blinking, Nose Wiping

19. Differentiating Seizure Types - Semiology
Head and Eye Deviation
Partial Seizures

20. What do you see? 2. Automatism 3. Eye Deviation
1. Head Deviation
2. Automatism
3. Eye Deviation
4. Unilateral Dystonic/Clonic Activity

21. Generalized Tonic Clonic
Seizure Types
Partial
Generalized
Generalized Tonic Clonic
Tonic
Clonic
Atonic
Myoclonic
Absence
Simple Partial
Complex Partial
Partial Secondarily
Generalized

22. Generalized Seizure Myoclonic
Characterized by quick, arrhythmic, and symmetric/asymmetric movements
Often not reported by patients.
Ask about sudden falls or dropping objects

23. Myoclonic-fast, jerking motion
See the Difference?
Myoclonic-fast, jerking motion
Clonic-rhythmic

24. Typical Absence Seizures
Semiology
Typical Absence Seizures
Characterized by brief, abrupt impairment of consciousness associated with EEG demonstrating 3 Hz spike and slow wave complexes with normal interictal background
May also demonstrate:
mild clonic, tonic, or atonic components
automatisms
autonomic components
Panayiotopoulos “The Epilepsies: Seizures, Syndromes, and Manangement

25. Generalized Seizure Semiology
Infantile Spasms

26. Review So Far Common seizure imitators in pediatrics
Seizures come in two basic types. You have to ask the right questions to distinguish them
Now...how do you diagnose epilepsy (i.e. when is EEG/MRI necessary) and why do we care?

27. Epilepsy Types Partial Generalized Cryptogenic Cryptogenic Idiopathic
Benign Rolandic Epilepsy
Benign Occipital Epilepsy
Cryptogenic
Idiopathic
Symptomatic
Childhood Absence
Juvenile Absence
Juvenile Myoclonic
Grand Mal Upon Awakening
West Syndrome
Lennox-Gastaut
Symptomatic
Primary = Idiopathic = presumed genetic
Secondary = Symptomatic=underlying cerebral cause (i.e. injury, dyplasia, etc.)

28. Idiopathic Partial Epilepsy
Benign Rolandic Epilepsy
(Benign Childhood Epilepsy with Centro-Temporal Spikes)
Onset 1-14 years, 75% between 7-10 years of age
Prevalence is 15% of children with seizures
Characterized by infrequent, often single, focal seizures consisting of unilateral facial sensorimotor symptoms, oropharyngolaryngeal manifestations, speech arrest, or hypersalivation lasting 1-2 minutes
1/3 -2/3 will have secondarily generalized seizures
75% are nocturnal
MRI normal
Typical EEG

30. Benign Rolandic Epilepsy
Prognosis/Treatment
2-3% school age children have CT spikes with <10% having BRE
Remission usually within 2-4 years from onset and before the age of 16 years
Less than 2% will develop infrequent generalized seizures in adulthood
Treat or not to treat

31. Benign Occipital Epilepsy
Gastaut Type
Onset 3-15 years
Manifest as visual hallucinations, blindness, or both-lasting seconds to <3 minutes
Rarely terminate with hemiconvulsions or generalized convulsion
50% have postictal headache
Similar manifestation to seizures from occipital lesions - MRI needed
Typical EEG

32. Benign Occipital Epilepsy
Fixation-Off EEG

33. Benign Occipital Epilepsy
Prognosis/Treatment
Remission occurs 2-4 years from onset for 50-60% of patients
Dramatic response to carbamazepine in >90%
15% association with celiac disease

34. Symptomatic Partial Epilepsy
Abnormal MRI (stroke, dyplasia, etc.) or abnormal EEG without classic pattern
History not consistent with primary partial epilepsy
Prognosis varies

35. Secondary Partial Epilepsy - MRI
Heterotopia
Mesial Temporal Sclerosis

36. Idiopathic Generalized Epilepsy Childhood Absence Epilepsy
Onset 2-10 years, peak 5-6 years
2/3 are females
Abrupt cessation of activity or speech last 4-20 seconds followed by return to baseline
Normal MRI
Typical EEG with 3Hz SW often provoked with HV

37. Idiopathic Generalized Epilepsy Childhood Absence Epilepsy

38. Idiopathic Generalized Epilepsy Childhood Absence Epilepsy
Prognosis/Treatment
Remission often occurs before 12 years of age
Less than 10% develop infrequent generalized tonic clonic seizures in adolescence or adult life
Rarely will patients continue to have absence seizures as adults
Treatment with valproic acid, ethosuximide, or lamotrigine will control absences in >80%
Possible role for topiramate and levetiracetam

39. Idiopathic Generalized Epilepsy Juvenile Absence Epilepsy
Age of onset 9-13 years
80% suffer from GTCS and 15-25% have Myoclonic seizures with onset 1-10 years after absences
Frequent/severe absences
Absence status in 20%
Prognosis: % will be controlled, though this is a lifelong disorder
20% may have intractable absences and GTCS

40. Idiopathic Generalized Epilepsy Juvenile Myoclonic Epilepsy
Characterized by myoclonic jerks upon awakening starting in adolescence
GTCS (>90%) may begin a few months later, occasionally earlier
Absence seizures (33%), if present, begin between 5-16 years
M:F equal
Seizure precipitants: Sleep deprivation, alcohol, stress, video games

41. Idiopathic Generalized Epilepsy Juvenile Myoclonic Epilepsy
EEG: irregular generalized 3-6hz spike/polyspike-slow wave discharges and generalized fragments. 33% have photoparoxysmal responses

42. Idiopathic Generalized Epilepsy Juvenile Myoclonic Epilepsy
Treatment/Prognosis
Valproic Acid, levetiracetam most commonly used monotherapy treatment
Lamotrigine, clonazepam
Prognosis: Seizures well controlled in up to 90% of patients. Treatment is lifelong, as 80% relapse after drug withdrawal
Carbamazepine, oxcarbazepine, phenytoin, gabapentin, tiagabine, and vigabatrin are contraindicated
Lifestyle management with regards to alcohol use, sleep deprivation, etc.

43. Symptomatic Generalized Epilepsy
Infantile Spasms
West Syndrome
“...these bobbings...they come on whether sitting or lying; just before they come on he is all alive and in motion...and then all of a sudden down goes his head and upwards his knees; he then appears frightened and screams out. --W.J. West (1841)

44. Symptomatic Generalized Epilepsy
Infantile Spasms
West Syndrome
Onset between 3-12 months, peak at 5 months
Incidence: 3-5/10,000
Spasms are flexor, extensor, or combined
Clusters with seizures per day, occurring most often on awakening or prior to sleep
Developmental delay preceeds spasms in 2/3
Classified as symptomatic, probably symptomatic, and cryptogenic

45. Symptomatic Generalized Epilepsy
Infantile Spasms
West Syndrome
80% symptomatic with most caused by pre-, peri-, or post-natal insults (i.e. HIE, ICH, dysplasias, trauma)
50% of patients with TS have spasms
3% of patients with Trisomy 21
Aicardi’s syndrome (spasms, agenesis of the corpus callosum, and retinal lacunes
EEG demonstrates hypsarrhythmia
High voltage, chaotic, arrhythmic and asynchronous which becomes more synchronous in NREM sleep
Multifocal independent spike wave discharges
Periods of electrodecrement

46. Symptomatic Generalized Epilepsy
Infantile Spasms
West Syndrome

47. Symptomatic Generalized Epilepsy
Infantile Spasms
Prognosis
Spasms typically will remit, even without treatment, by 18 months of age
60% of patients develop other seizure types, CPS and Lennox-Gastaut syndrome are most common
90% of patients have developmental delay, 66% are severely cognitively impaired

48. Symptomatic Generalized Epilepsy
Infantile Spasms
Treatment
ACTH - 50% remission, all or none. No proven dosing regimen, no clear reason why it works
Topiramate - similar efficacy usually in high doses (25-30mg/kg/d)
Vigabatrin - especially useful in TS (90%), beware of irreversible visual field defects
Pyridoxine, valproate, zonisamide, levetiracetam, lamotrigine, felbatol, keto diet
Surgery

49. Symptomatic Generalized Epilepsy Lennox-Gastaut Syndrome
Three criteria
Multiple intractable seizures including tonic (80-100%), atypical absence (66%), and atonic (50%)
cognitive and behavioral abnormalities
Slow (<2.5 Hz) generalized spike wave
Onset 1-7 years, peak 3-5
10-30% develop from West syndrome or other epileptic encephalopathies

50. Symptomatic Generalized Epilepsy Lennox-Gastaut Syndrome

51. Symptomatic Generalized Epilepsy Lennox-Gastaut Syndrome
Prognosis/Treatment
5% die, 80-90% have seizures as adults, and approximately 90% have severely impaired cognition and behavior
Treatment includes almost every AED with polypharmacy common.
Ketogenic diet, VNS, corpus callosotomy

52. Choosing an AED
Type of epilepsy

53. Treatment of Epilepsy: AEDs
Partial
Generalized
Phenytoin
Phenobarbital
Valproic Acid
Carbamazepine
Oxcarbazepine
Gabatril
Gabapentin
Topiramate
Lamotrigine
Zonisamide
Levetiracetam
Valproic Acid
Topiramate
Zonisamide
Lamotrigine
Levetiracetam
Ethosuximide
Felbamate

54. Choosing an AED Type of epilepsy
Type of formulation (IV, capsule, sprinkle, etc.)

55. Choosing an AED Formulation
IV: Benzos, phenytoin, phenobarbital, valproic acid, levetiracetam
Sprinkles: valproate, topiramate
Liquids: carbazepine, oxcarb, levetiracetam, valproate, dilantin. zonegran,lamictal,topiramate will dissolve in H20
Extended release: valproate, carbamazepine

56. Choosing an AED Type of epilepsy
Type of formulation (IV, capsule, sprinkle, etc.)
Time to onset

57. Choosing an AED Time To Onset
Rapid onset: Any IV form
Onset in 24 hours: Levetiracetam
Onset in Days: carbamazepine, oxcarb, dilantin, valproate, zarontin.
Slow titration: Topiramate, zonisamide
Really slow: Lamictal

58. Choosing an AED Type of epilepsy
Type of formulation (IV, capsule, sprinkle, etc.)
Time to onset
Side Effects

59. Choosing An AED Side Effects
Somnolence
All
Rash
Lamictal
Phenytoin
Phenobarb
Renal Stones
Topiramate
Zonisamide
Hyponatremia
Carbamazepine
Oxcarbazepine
Cognitive
Phenobarb
Topiramate
Parasthesia
Topiramate
Zonisamide
Behavior
Levetiracetam
Labs draws
Carbamazepine
Valproic Acid
Phenytoin
Levels
All

60. Choosing an AED Type of epilepsy
Type of formulation (IV, capsule, sprinkle, etc.)
Time to onset
Side Effects
Dosing Schedule

61. Choosing An AED Dosing Schedule
QD: Depakote ER, Zonisamide
TID: Depakene, Neurontin, Tegretol, Phenytoin (neonates)
BID: Everything else

62. Febrile Seizure 3 types (simple, complex, status) treatment
NIH consensus: Febrile seizure is an event in infancy or childhood, usually 3m-5 years, associated with fever but without evidence of intracranial infection or defined cause. Seizures with fever in children who have suffered a previous nonfebrile seizure are excluded.
incidence- 4%: absolute risk increased with family hx (1 relative 10%, 2-32%), daycare (7%), dev delay (10%)
Risk of recurrence: 1 in 24
risk of future epilepsy: 2-10%
workup - MRI/EEG does not predict recurrence
treatment

63. Practice Parameter Febrile Seizures
Current Recommendations AAP [Pediatrics 97(5), May 1996, ]
Age 6-12 months with febrile seizure should strongly consider LP
Age months should consider
>18 months may use physical exam, associated symptoms to drive need
Based recommendations on 4 studies reporting 13-15% of children will present with seizures as the initial manifestation of seizures with 30-35% having no meningeal signs.
More recent reviews have suggested the presence of meningitis in the absence of associated signs is rare (1/200), with a large percentage of such patients with normal CSF at presentation. The introduction of the H.Flu vaccine has significantly altered the epidemiology of infantile bacterial meningitis making present treatment different from that 30 years ago (which the AAP based their recommendations).
Most physicians would agree that LP in children outside the range of febrile convulsions is necessary, as well as children within the range with sign or symptoms of CNS infection, such as nuchal rigidity, altered mental status, etc.

64. Practice Parameters First Unprovoked Seizure
Laboratory investigations (CBC, CMP, tox screens) should be considered based on historic and clinical findings
LP is of limited value in first unprovoked afebrile seizure
EEG is recommended to dx epilepsy syndromes and provide for prognosis
MRI is preferred modality and should be considered in children with cognitive/motor impairment that is unexplained, focal onset seizures, or in children < 1y.
Emergenat imaging should be performed in children with prolonged todd’s, or prolonged (several hours) postictal state.
Treatment: 46% have recurrence in 10years, 19% > 4 seizures, and 10%>10 seizures

65. FAQ (the ED)
I have a 14 month old with a febrile seizure and a “raging otitis,” do I need to do a LP?
(3a.m.) Hey, how are you? I have a kid here with known epilepsy that had a breakthrough seizure (like he does once every 3 months or so), do you want to increase his medicine?
Do I need to CT this kid?
I have a patient of Dr. Flamini’s here, how do you want to treat him?

66. FAQ (the parents) Why does my child have seizures?
Will my child be stupid?
How long does my child need treatment?
What do I do when my child has a seizure?

67. How long will she need medicine?
Case
16 year old female with first unprovoked seizure, described as generalized tonic clonic
Focal signs at onset?
Myoclonic or absence type episodes?
Time of day?
Previous workup?
Treatment choices: Depakote, Keppra, Trileptal
How long will she need medicine?

68. Case
8 y/o with frequent episodes of staring, at times associated with lip smacking
Focal signs at onset? Can they be stopped?
Myoclonic or GTC episodes?
Duration?
Time of day?
Previous workup?
Treatment choices: Depakote, Keppra, Trileptal