In this presentation I will try to shed light on diagnosis of different pediatric epilepsy syndromes, non-epileptic paroxysmal events

1. Amr Hassan, M.D.,FEBN
Associate professor of Neurology
Cairo University
Non epileptic
Paroxysms in Childern

2. • These are common in practice
• Diagnosis is possible by history and exam alone
• Most kids will do well with early recognition and
management
• Costly investigations are avoidable
Why NPEs are important?

3. • Age of onset and course of events
• Description, frequency, awake/sleep/both, stereotypic
nature, triggering or relieving factors
• Consider pathophysiologic mechanisms based on
event description
• Epileptic & non-epileptic events may coexist
Clinical approach to NPEs are important?

4. Age/State Based Approach
4
Neonates (0-8 wks)
- Jittery
- Startle reflex
- Benign sleep myoclonus
Infants (2mth-2yr)
- Breath holding spells
Attacks
- Stereotypy (Mannerisms)
- Rhythmic movement disorder
(sleep) -
Childhood (2-12yr)
- Staring /day dreaming
- Tics/Movement disorder
- Migraine equivalents
- Masturbation
- Drugs (Antiemetics)
- Stereotypy (Mannerisms)
- Parasomnias (sleep)
Adolescents (>12 yr)
- Syncope/Cardiac
- Psychogenic seizures
Age/State
Based
Approach

5. Pathophysiological Approach
5
Cardiovascular
- Breath holding spell
- Syncope
- Arrhythmias
Movement Disorder
- Tics
- Dystonias
- Choreoathetosis
- Shuddering attacks
- Stereotypy
- Nonepileptic myoclonus
Neurobehavioral
- Migraine variants
- Sleep/arousal disorders
-- Masturbation
- Panic attacks
- Psychogenic seizures
Gastrointestinal
- Reflux
Patho-
physiological
Approach

6. NPE during sleep/ wake
Wake Sleep
Neonates Jitteriness Benign neonatal sleep myoclonus
<8wks Stiff baby/hyperexplexia
2 mo -2 ys Shuddering attacks
Spasmus Nutans
Stereotypies
Benign myoclonus of early
infancy
Hyperexplexia
Infants Breath holding spells Rhythmic movement disorder

7. Wake Sleep
children
2-12
years
• Stereotypies
• Syncope
• Migraine/variants (cyclic vomiting, benign
paroxysmal vertigo)
• Tics
• Paroxysmal choreoathetosis (kinesiogenic,
dystonic)
• Gastroesophageal reflux
• Pseudoseizures
• Head banging
• Parasomnias-
Sleep terrors,
sleep walking
• Hypnic jerks
NPE during sleep/ wake

8. NPE during sleep/ wake
Wake Sleep
Adolescents
>12 years
• Syncope
• Migraine and variants
• Paroxysmal
choreoathetosis
(kinesiogenic,
dystonic)
• Pseudoseizures
• Tremor
• Tics
• Transient global
amnesia
• Narcolepsy—steep
paralysis, hypnagogic
hallucinations
• Parasomnias

10. 10
Cyanotic Breath Holding Spell
• 6 months – 2 years (up to 5 years)
• Typically confused with tonic seizure
• Always a trigger: fear, injury, frustration
• Cry → breath holding (expiration) → stiff,
• Loss of awareness → clonic jerks
• Patho-physiology not understood
• Correction of anemia, counseling

11. • Parasympathetic dysregulation, pale and limp, with
asystole
• he most common stimulus is a painful event.
• The child turns pale (as opposed to blue) and loses
consciousness with little if any crying.
• The EEG is also normal, and again there is no post
ictal phase, nor incontinence.
• The child is usually alert within a minute or so.
Pallid type (Reflex asystole)

12. Breath-holding spells Epileptic seizures
Trigger Crying, injury Spontaneous, fever, sleep deprivation
Occurrence
during sleep
No May occur during sleep
Event Sequence  provocation —
apnea, cyanosis/ pallor,
limpness.
Associated with stiffening and jerking of
extremities
Postictal state Usually brief Maybe prolonged
Epileptiform
abnormalities on
EEG
Absent Usually present
Treatment Parental reassurance Anticonvulsant therapy
D.D. Breath Holding Spells

14. • Rapid, random, bilateral/asynchronous
• jerking, may be forceful and rhythmic
• Seconds-minutes or even hours in sleep
• All stages of sleep (Quiet sleep/NREM)
• Differential: Seizures and Jitteriness
• Disappear when infant is woken up
• Not seen during alert wakefulness
• Does not stop on passive flexion (jitter stops)
• EEG: Normal baseline and during events
• Mostly disappear by late infancy
Benign Neonatal Sleep Myoclonus

15. • Infants with sudden head nods/drops
• No fall or interruption of activity
• No change in facial expression/behavior
• No extremity movements
• Momentary, quick recovery
• Confused with infantile spasms
• History and Exam are benign
• Development, and EEG normal
• Management: Reassure
Head Drops
(Benign Infantile Myoclonus)

16. • Dystonic, abnormal movements of head, Neck, upper trunk
(Sandifer’s syndrome)
• Life-threatening events – apnea with Cyanosis and/or pallor
• Vomiting, failure to thrive
• More common in delayed/hypotonicpatients
• Management:
Confirm diagnosis, treatment of reflux
Gastro-esophageal Reflux

17. 17

18. • Onset 6mth – 10 yrs, gradually better
• Sudden tremulous contraction (shiver)
• Flexion of head and trunk
• Adduction and flexion of elbows
• Brief, up to 100/day, cluster
• Intervening several weeks of no spells
• Benign phenomenon
• No treatment, gradually disappears
• Specific stereotypy/mannerism
Shuddering Attacks

19. 19

20. • The movement resembles a tremor
• distinguished clinically from clonic seizures by
 No associated ocular movements
 No Autonomic phenomena
 Stimulus sensitivity ((eg, triggered by stimulation or easily
stopped with passive movement of the limb).
Jitteriness

21. Childhood Parasomnias
Features Nightmares Night terrors
Age of onset
Duration
Semiology
Stage sleep
Time
Recall
2-5 years
<1-2minute
Cling, verbalize
REM
early am
usually able
4-8 years
>5 minutes
vary/autonomic
NREM III & IV
first third of night
not able

23. • 1st decade, confused with myoclonic jerks
• Repetitive, Purposeless movements
• Sudden, brief, stereotypic, wax/wane
• Brief voluntary, inhibition possible
• Worsen with anxiety, Disappear in sleep
Motor Tics
• Simple: Face twitch, head shake, eye blink, sniff
• Complex: Facial distortion, jump, fiddle, sway
Vocal: cough, clear throat, words
Common: Tourette syndrome
Tics

24. Tics, hyperactive, aggressive behavior
• Clonidine
• Pimozide, Other dopamine antagonists
Inattention
• Methyl phenidate, D-amphetamine,
• Atomoxetine
Anxiety, depression, obsessive compulsive
• SSRI, Bupropion
• Behavior counseling, child guidance
Treatment of Tourette

25. 25

26. 26
D.D. of Tics

27. 27

28. Stereotypies Tics
Onset Younger School age
Pattern Patterned, predictable, identical Variable, wax and wane
Movements Flapping, waving arm/hand movements Blink, grimace, shrug
Rhythm Rhythmic Rapid, sudden , random
Duration Prolonged, continuous Brief, intermittent
Premonitory urge No Yes
Trigger Excitement, stress Excitement, stress
Suppression Distraction Brief (causes inner tension)
Family History Rarely positive Frequently positive
Treatment Poor response Neuroleptics
D.D. of Tics

29. • Infants/young children, min to hours
• Episodes of genital and self stimulation
• Paroxysmal, Stereotypic, rhythmic
• Tightening of thighs and rocking
• Pressure to pubic/supra-pubic areas
• Irregular breathing, flushing, diaphoresis
• Mimic complex partial seizures or pain
• Reassure and inform parents
Childhood Masturbation

30. • Benign condition, healthy toddlers Spells
• Sudden, few minutes (uncommonly ~hour) “As if
disequilibrium”
• Key: An alert child who is unable (refuses or frightened) to
walk
• 1-2/ week to 1 every 1-2 month
• Stable course, improve by 6 years age
• Family history of migraines
• No Rx., reassurance
• Rare: Anti vertiginous agents
Benign Paroxysmal Vertigo

31. Pre-syncopal symptoms (aura)
• Light head, nausea, visual blurring, distant
• Hearing, vertiginous
Syncope:
• Fall/slump, pale, loss of awareness, stiff, clonic
• jerking (confused with GTCS)
Three most common in children
• Vasovagal (Neuro-cardiogenic)
• Orthostatic (Drugs, autonomic dysfunction)
• Cardiogenic (Arrhythmia)
Benke et al., Eur Neurol 1997;37:28
Syncope (Convulsive Syncope)

32. • Treat cause if found
• Avoid precipitating factors, hydration,
• Adequate salt intake, compression stockings
Meds: Weak evidence in trials:
• Midodrine – alpha-1 agonist, 10mg tid
• Fludrocortisone (0.1mg/day to up to 1mg/d)
• Pseudoephedrine, Paroxetine, DDAVP
Treatment - Syncope

33. • 4-16 years, Duration: Secs. to few minutes
• Dystonic or Choreo-athetotic movements
• Induced by activity or emotion
• Involve extremities, face, head/neck, inability to speak or fall,
Intact sensorium
• Idiopathic sporadic, symptomatic, familial
• Stable or improving course
Treatment: If interferes with daily life
• Low doses of Carbamazepine or phenytoin
Paroxysmal Kinesigenic Choreoathetosis

34. 34
Paroxysmal Kinesigenic Choreoathetosis

36. • Body rocking
• Head banging
• Head rolling
• Other less common muscle movements include:
• Body rolling
• Leg rolling
• Leg banging
• A combination of the aforementioned symptoms
Rhythmic Movement Disorder

38. 38

40. • SN is an idiopathic disorder.
• Onset 4-12 months, Remit within 1 to 5 years of onset
• Triad of asymmetric and pendular nystagmus, head nodding,
and torticollis.
• Head nodding suppresses nystagmus through the vestibular-
ocular reflex and aids vision.
• D.D. optic pathway glioma (chiasmal)
Spasmus nutans

41. 41

42. • AHC is also extremely rare – approximately 1 in 1,000,000
people have this disorder.
• begin before 8 months of age
• 50% of AHC sufferers also suffer from EPILEPSY
• Closely related to AHC is FHM
• ATP1A3 as the likely genetic cause of this disorder (19q13.31).
• The most common drug used to treat AHC is FLUNARIZINE.
Alternating hemiplegia of childhood

43. 43

44. PseudoSeizures
Non-epileptic seizure Epileptic seizure
Duration Prolonged (several minutes) Usually less than 2-3 minutes
Clinical features • Fluctuating features
• Usually during wakefulness
• Preserved consciousness, avoidance
behavior
• Side to side head movements
• Out of phase extremity movements
• Forward pelvic thrusting
• Emotional vocalization
• Pupillary reflex retained
• Stereotypic features
• May occur in sleep
• Altered consciousness
• Head unilaterally turned
• In phase extremity movements
• Retropelvic thrusting
• Monotonous vocalization
• Pupillary reflex absent
Incontinence Rare Present
Tongue bite Occasional Common
Postictal changes None Usually present
Affect La Belle indifference Concerned

45. Bob Beamon
Mexico Olympics in
1968.
45

46. 46

47. • Cataplexy is a sudden and transient episode of muscle
weakness accompanied by full conscious awareness
• typically triggered by emotions such as laughing, crying, or terror.
• It is the cardinal symptom of narcolepsy with cataplexy affecting
roughly 70% of people who have narcolepsy
Cataplexy

48. D.D. of NPE
NPE Imitating epileptic condition/s
Syncopes Generalised Tonic Clonic Seizures, Focal seizures, Absences, Drop
attacks, myoclonic Epilepsies
Breath holding attacks Tonic Spasms
Cataplexy Atonic fits
Day dreams/Childhood
Preoccupation
Absence epilepsy
Tics Myoclonus, stereotypy
Sleep disorders Frontal lobe seizures, Benign rolandic epilepsy
Benign Sleep myoclonus Myoclonic Epilepsies, Focal Epilepsies
Migraines Occipital lobe seizures, temporal lobe seizures
Pseudo seizures Status epilepticus, tonic, tonic clonic seizures, absences, parietal lobe
sensory seizures, Status non-convulsicus

49. 49

50. Classification according to symptom of presentation
50
Apnoeas
•Gastro-oesophageal reflux
•Breath holding attacks
•Reflex anoxic seizures
•Sandifer syndrome
Staring or brief unresponsiveness
•Day dreams
•Gratification phenomena
Abnormal movements
•Restless leg syndrome
•Paroxysmal dyskinesias
•Tics
•Chorea
•Dystonia seen as part of cerebral palsy
•Benign infantile spasms
Tonic spasms
•Hyperekplexia
•Familial rectal pain syndrome
•Gratification phenomena
•Benign paroxysmal torticollis in infancy (BPTI)
•Raised intracranial pressure
•Shudders
•Benign paroxysmal tonic up gaze
•Breath holding attacks
Convulsive seizures
•Reflex anoxic seizure
•Syncope
•Long QT syndrome
•Heart blocks
•Jitteriness
•Hyperekplexia
•Gratification phenomena
•Sleep wake transition disorders
Myoclonic jerks
•Spinal myoclonus
•Benign myoclonic epilepsy of infancy (BMEI)
•Benign neonatal sleep myoclonus
•Non-epileptic myoclonus
•Benign non-epileptic infantile spasms
Psychic states
•Out of body experience
•Schizophrenia
•Panic attacks
Prolonged confusion/unresponsiveness
•Encephalitis
•Encephalopothies
•Drug intoxication
•Basilar artery migraine
Startle
•Hyperekplexia
•Sleep starts Sleep phenomena
•Narcolepsy
•Nightmares
•Night terrors
Weakness
•Hemiplegic migraine
•Periodic paralysis
•Alternating hemiplegia of childhood
Prolonged unresponsive states
•Sleep paralysis
•Pseudo seizures
•Encephalitis/ encephalopathy
•Head injuries
•Drug intoxication
Sensory symptoms
•Out of body experience
•Pseudo seizures
•Migraines
Ataxia:
•Episodic ataxias
•Benign paroxysmal vertigo of childhood
(BPVC)
Varying presentation
•Conversion disorder or pseudo
seizures
•Fabricated illness
•Night terrors
•Nightmares