Not all seizures are. Non-Epileptic Events in Children. but some are.

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1 Non-Epileptic Events in Children How to tell what is real, what is not, and helpful hints. Not all seizures are seizures but some are. 17 th Annual Pediatric Emergency Medicine Update 16 April 2015 Michael Strunc MD Child Neurology and Sleep Medicine First Seizure(better, say spell or episode). Hx- seizure or not? Next- IF it IS seizure Provoked Treat underlying cause(na 112), +/- MRI, EEG, etc. Unprovoked Treat if necessary, time, and maybe.. Get studies as dictated- EEG, imaging. Diagnosis History Do not call it a seizure, it is a spell or event (unless you know it IS a seizure). You need details, the play by play for each event. Before- what was going on? Trigger- yes or no? Event- beginning, middle and end. Seizures usually have thisex. Stiff tonic posturing, then rhythmic tonic-clonic that builds in tempo, then slows and stops, then sleepy, tired.. Color change or not? Awake or asleep or both. Can you alter the event? if so how, if not- what did you do to attempt to stop it? Is there a way you can predict this event? Is there a pattern to how the event(s) occur? Family history Development and any change- regression, irritable, ataxia, etc.. Breathholding Differential Diagnosis Myoclonus- benign neonatal Hyperventilation Self-Gratification Vasovagal/syncope TIA Narcolepsy, Night Terrors, Parasomnias Migraine and variants Psychogenic Rhythmic movement disorder Tics/Tourette ADHD Sandifer(reflux) Tremor Spasmus nutans Cases 2 week old with sudden, brief, multifocal myoclonic seizures. 4 month old with tonic seizures. 10 month old with reflux. 15 month old with spells. 6 year old with staring seizures. 9 year old with atonic seizures/spells. 10 year old with brief focal motor seizures. 1

2 2 week old, healthy, multifocal seizures.. Thriving infant, born without complications Parent notes sudden brief jerks, multifocal, that continue for seconds, to minutes. This has occurred many times, they cannot make it stop. No trigger, not ill. What else? Benign Neonatal Sleep Myoclonus Breastfeeding, sudden multifocal twitching, sustained, cannot suppress but infant is actually falling asleep at breast IF we wake infant, and it stops, but you may need to chase this down EEG normal- and reasonable to get it Done this since? Days of life. Is NOT ill, and is thriving. Myoclonic seizures are bad, and infants with them are encephalopathic usually Tetany, electrolytes, jittery, hypoglycemia 4 month old tonic sz Term, normal development but slow with weight gain Several seizures with remarkable stiffening, infant seems stuck, and is clearly irritable Cannot stop it Duration- seconds, minutes, but recurs often Other questions? Reflux(Sandifer) Our infant arches, stiffens and is irritable- typical posturing-atnr posture? Related to eating- hard to tell Treating presumptively- can help Eyes are closed- usually open in true seizure Fussy eater, spits a ton? Tonic Seizures In infancy, sudden partial or complete stiffening but less often ATNR, usually brief(seconds) Eyes open? Consciousness is often preserved Encephalopathy common- consider severe syndromes such as Ohtahara In utero? Metabolic, or anatomic( major CNS malformations) EEG diagnostic 10 month old reflux? Irritable Wakes and does this thing.. Not myoclonus, but need description Can be extensor, flexor, both Often does it several times in a row or as a cluster, but can occur randomly Irritable, and more fussy lately EEG diagnostic Etiology, treatment? Neurocutaneous Disorder 2

3 Infantile Spasms 6-18 months most common around 1 yr. Cluster spasms with waking Brief( not myoclonus) Irritability,? Regression Idiopathic, Symptomatic TS EEG- Hypsarrhythmia, Electrodecremental Treat- ACTH, Vigabatrim(TS) Badness, Lennox-Gastaut, Delays severe 10 month old tonic/staring seizure? She does it a lot, an awful lot. Gets stuck/stiff, with rhythmic movements Cannot seem to stop it. It almost always happens in carseat, highchair, or lying in crib.. Stiffens, adducts legs, red in face, and seems zoned out. Stares off.. This is freaking mom out Self-Gratification Infancy to toddler most often Girls Rock, grunt, sweat, stiff, stare off tonic seizure, or staring spell Often positional- sitting, supine Duration seconds to minutes, and difficult to interrupt Benign, outgrown, CAN become extreme 6 yr old staring spells At school, or watching TV Lasts? minutes, usually Cannot get him to pay attention to me.?how do you attempt to interrupt him? Does NOT interrupt him during speaking, eating, etc. HV normal- you need to do this!! Grades seem to be falling. He is not hyper! ADHD, or staring seizure How to tell the difference? Absence vs Daydreamer Absence yrs. Peak 6-8 yrs. Girls >Boys Dozens to Hundreds a day, seconds long Interrupts speech, activity Hyperventilation provokes GTC in some Remit usually EEG with 3 Hz s/w Daydreamer Same age Less frequent Longer duration Will not interrupt Boredom Hyperventilation will not provoke EEG nl. NMCP MStrunc

4 9 yr old atonic seizures or syncope He will simply sag, slump or actually fall EKG is normal No color change, pallor, etc. Prodrome- nope. Well, it does seem to occur when he is really upset. Most often- laughter Partial often, consciousness preserved. History! Narcolepsy Co-morbid features to be aware of Sleepy- not tired, but powerful sleep pressure Weird, vivid dreams( nightmares?) Sleep Paralysis Cataplexy- Often misinterpreted, but look for ANY strong emotion resulting in change in tone, voice, posture, balance Funny thing in young child- look like this Sleepy sleepy guy 10 yr old brief focal motor sz He is talking to me, and see this sudden recurring twitch, jerk of face. He cannot stop it, and it is stereotypic, short duration, recurs often Can tell it will happen, and it almost better to let it happen He does blink eyes a lot Sniffles, all the time. Allergy shots not helpful Tics, tourette Very common in toddler to gradeschooler Face, neck Funny stereotypies can also occur Triggers- situations where it is bigger, smaller CAN suppress, briefly Wax, wane, come and go OCD, anxiety, attention, type A phenotype Teen with seizure The teenager who passes out or has a first convulsive seizure. MOST common- one of 3 diagnoses in the otherwise healthy teen. Syncope Seizure Non-epileptic Event Teen with Seizure Syncope- Early in day, prodrome, pallor, collapse- sudden, or not? Recovery- wake in seconds, ANS amped up ANS- tachycardia, sweaty, but alert Coherent enough rapidly- I helped get myself to wheelchair, drank water in nurse office.. Seizure- Aura(prodrome)- not often Sudden, not time of day related, not positional Go down, convulse immediately, (urinate, bite? maybe_) Wake? In ambulance, on gurney. Not able to do much for longer minutes? Next slide---key question to ask if it sounds like THIS? Non-epileptic Events 4

5 Juvenile Myoclonic Epilepsy Teenager Convulsive seizure Morning myoclonus Photoconvulsive Can have atypical absence as well EEG- Generalized 4-6 polyspike/wave Lifelong Tx-VPA, Lamictal, ZNG, Keppra etc. NOT Na channel drugs Juvenile Myoclonic Epilepsy Convulsive seizure Morning myoclonus ASK, pt, parent. Photoconvulsive Can have atypical absence as well EEG- Generalized 4-6 polyspike/wave Lifelong Tx-VPA, Lamictal, ZNG, Keppra etc. NOT Na channel drugs Seizure, or not 14 yr. Old girl presents to ER for her second seizure. She was at school when her friends state she suddenly fell to the floor, began shaking all over for minutes before the ambulance came and took her to the hospital. She is in the trauma room where you go to see her only to have another seizure begin. She is visibly stiff, arching her back, and has jerking of her arms and legs, independently. This lasts several minutes, then slowly stops. She is then unresponsive, even to a painful stimulus. More Her exam is generally unremarkable. Vitals normal. Neurologic-No focal findings, pupils brisk and equal. She does not fix, follow, but when you go to open her eyes you notice her forced eyelid closure. Labs are normal including chem panel( bicarbonate), glucose, prolactin pending. CPK normal. You suspect nonepileptic seizure. What do you want to know? What do you do now? If she seizes again? History Epilepsy? Her, or friend, family member. Normal in most. Exam Prior events, DETAILS. Injury(head,bite tongue), incontinence, post-ictal state. Duration(seconds versus many minutes). Situation-rarely alone(they are usually observed during event). Not during sleep. Weakness that is subjective. Wax/wane, gone when not observed. Hoover sign Defy UMN pattern Bizarre movements seen during event such as.. Psychosocial history(abuse, sexual/physical). 5

6 Herky-Jerks Pelvic thrusting, wild flailing movements. Shifting lateralization Opisthotonos Out-of-phase clonic activity Stopping and starting repeatedly Slow onset and cessation. Avoidance behavior(hand on face, mirror if eyes open, withdrawal from pain, cotton in nose), forced eye closure. Examiner can modify Moaning and Groaning Not seen on exam. Autonomic changes Incontinence Salivation,drooling. Post-ictal state Nonepileptic seizures Psychogenically determined clinical events that resemble epileptic attacks but are not associated with physiologic CNS dysfunction. This represents a conversion disorder where spells are a nonvolitional expression of hidden negative feelings/conflicts, reduced in severity via the spell, and thus keeping the conflict out of conscious awareness. What else could it be? IF not a seizure(and this is possible). Malingering-secondary gain and volitional. Munchausen by proxy Factitious disorder(goal is not clear secondary gain, but to assume the sick role). Depression, mood disorder? Nonepileptic seizures Incidence-20-30% referrals to epilepsy centers Often can coexist with epilepsy Abuse often exists(adult study 32-67%) Outcome better for kids-over 80% cured at 3 years versus 40% adults. Diagnosis-the real McCoy Typical seizure-gtc, CPS, less often stare spells Sudden onset, abrupt ending. Duration short, less than 90 seconds. Rythmic, synchronized movements that are tonic, and/or clonic- and have a tempo Post-ictal period Incontinence, injury, bite. Occur during sleep, when not observed, not in a stereotypic environment. 6

7 Nonepileptic Observed, not during sleep(in bed? asleep). Vary from spell to spell Bizarre movements(see earlier). Prolonged-minutes to hours. No post-ictal period. Can be provoked often. Pain prodrome, vocalizations. Amnesia variable- often out of proportion Epilepsy- can be comorbid diagnosis. BUT Frontal lobe seizures can be.. Unusual motor, although unilateral. Preserved consciousness No postictal period. IF suspect it?? Video-EEG, provoke?? Not in ER, admit? Psychiatry needs to be involved. DO NOT say faker, faker, you re a belly acher. Support diagnosis with labs? Prolactin- minutes to 1 hour post. Higher than 20 mg/ml, and twice baseline. Acidosis Not perfect, false positives and negatives occur. Ataxia and Weakness Functional gaits and non-organic ataxia are uncommon presentations Important in case of weakness, ataxia- MUST demonstrate what is real on exam Start slow, and keep moving Getting ready to demonstrate gait, strength Testing on gurney History 15 year old presents with weakness, and inability to walk that began 3 days ago, while at school. Was in backseat of car the day prior, when they were rear-ended at low speed. No injuries, all went home fine. Now weakness of her legs, difficulty with balance. No bowel bladder symptoms, or sensory loss. No back pain. Home from school with this issues, and now worse over last 2 days. History 1. Abrupt onset 2. Static course or on and off 3. Spontaneous remission 4. Precipitated by minor trauma, not congruent 5. Psychiatric comorbidities 6. Multiple somatization, or additive 7. Employed in health profession(adult, or family) 8. Presence of secondary gain 9. Witnessed, not alone and no injury 10. Young age (female>male) Inconsistent character of movement (amplitude, frequency, distribution, selective ability) 7

8 Clinical 1. Paroxysmal movement disorder 2. Movements increase with attention or decrease with distraction(helpful colleagues in ER) 3. Ability to trigger or relieve the abnormal movements with unusual or non-physiological interventions (e.g.trigger points on the body, tuning fork) 4. False weakness-rolling chair, distraction, observation Hoover sign, Drop test Collapsing weakness, give-way or push-pull -( test patient) 5. False sensory complaints 6. Astasia-Abasia, walking on ice(magnetic), or 7. Deliberate slowness of movements, OR entraining movement 8. Functional disability out of proportion to exam findings 9. Movement abnormality that is bizarre, multiple or difficult to classify Normal reflex examination Objective normal strength Clinical Lack of additional cerebellar findings with hysterical gait Normal sensation or non-anatomic loss Tuning fork sensory test of forehead-hemi loss Hemisensory, or circumferential Hoover s sign. J Stone et al. J Neurol Neurosurg Psychiatry 2005;76:i2-i12 Summary Seizures, spells, weakness and gait abnormalities can be sorted out with good histories And, proving historical complaints with a neurologic examination that is complete, regardless of the subjective complaint Don t NOT test, due to subjective complaints Be able to use distraction, repeated examination and specific testing maneuvers to prove findings EEG, imaging has a role, but the money is on your history and the exam Neurologists are around to help 2005 by BMJ Publishing Group Ltd 8