non-epileptic paroxysmal events in childern
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In this presentation I will try to shed light on diagnosis of different pediatric epilepsy syndromes, non-epileptic paroxysmal events
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non-epileptic paroxysmal events in childern
- 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 ⢠Narcolepsyâsteep paralysis, hypnagogic hallucinations ⢠Parasomnias
- 10. 10 Cyanotic Breath Holding Spell ⢠6 months â 2 years (up to 5 years) ⢠Typically confused with tonic seizure ⢠Always a trigger: fear, injury, frustration ⢠Cry â breath holding (expiration) â stiff, ⢠Loss of awareness â 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 ď provocation â 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 (Sandiferâs syndrome) ⢠Life-threatening events â apnea with Cyanosis and/or pallor ⢠Vomiting, failure to thrive ⢠More common in delayed/hypotonicpatients ⢠Management: Confirm diagnosis, treatment of reflux Gastro-esophageal Reflux
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- 18. ⢠Onset 6mth â 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
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- 20. ⢠The movement resembles a tremor ⢠distinguished clinically from clonic seizures by ďź No associated ocular movements ďź No Autonomic phenomena ďź 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
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- 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) âAs if disequilibriumâ ⢠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 â 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
- 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
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- 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
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- 42. ⢠AHC is also extremely rare â 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
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- 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 â˘Gastro-oesophageal reflux â˘Breath holding attacks â˘Reflex anoxic seizures â˘Sandifer syndrome Staring or brief unresponsiveness â˘Day dreams â˘Gratification phenomena Abnormal movements â˘Restless leg syndrome â˘Paroxysmal dyskinesias â˘Tics â˘Chorea â˘Dystonia seen as part of cerebral palsy â˘Benign infantile spasms Tonic spasms â˘Hyperekplexia â˘Familial rectal pain syndrome â˘Gratification phenomena â˘Benign paroxysmal torticollis in infancy (BPTI) â˘Raised intracranial pressure â˘Shudders â˘Benign paroxysmal tonic up gaze â˘Breath holding attacks Convulsive seizures â˘Reflex anoxic seizure â˘Syncope â˘Long QT syndrome â˘Heart blocks â˘Jitteriness â˘Hyperekplexia â˘Gratification phenomena â˘Sleep wake transition disorders Myoclonic jerks â˘Spinal myoclonus â˘Benign myoclonic epilepsy of infancy (BMEI) â˘Benign neonatal sleep myoclonus â˘Non-epileptic myoclonus â˘Benign non-epileptic infantile spasms Psychic states â˘Out of body experience â˘Schizophrenia â˘Panic attacks Prolonged confusion/unresponsiveness â˘Encephalitis â˘Encephalopothies â˘Drug intoxication â˘Basilar artery migraine Startle â˘Hyperekplexia â˘Sleep starts Sleep phenomena â˘Narcolepsy â˘Nightmares â˘Night terrors Weakness â˘Hemiplegic migraine â˘Periodic paralysis â˘Alternating hemiplegia of childhood Prolonged unresponsive states â˘Sleep paralysis â˘Pseudo seizures â˘Encephalitis/ encephalopathy â˘Head injuries â˘Drug intoxication Sensory symptoms â˘Out of body experience â˘Pseudo seizures â˘Migraines Ataxia: â˘Episodic ataxias â˘Benign paroxysmal vertigo of childhood (BPVC) Varying presentation â˘Conversion disorder or pseudo seizures â˘Fabricated illness â˘Night terrors â˘Nightmares