"Non-Epileptic Paroxysmal Events (NEPE) Erick Sell, M.D Neurology Division Children s Hospital of Eastern Ontario

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1 "Non-Epileptic Paroxysmal Events (NEPE) Erick Sell, M.D Neurology Division Children s Hospital of Eastern Ontario

Objectives Learn the clinical presentation of some

in childhood Understand the prognostic and

NEPE Learn a general diagnostic approach and

2 Objectives Learn the clinical presentation of some examples of non-epileptic paroxysmal events (NEPE) in childhood Understand the prognostic and therapeutic implications of effectively diagnosing NEPE Learn a general diagnostic approach and indications for further investigations when diagnosing children with NEPE.

3 Outline Definition and Grouping General approach to NEPE Case examples

4 Definition and Grouping

5 Definition and Grouping

7 General Approach to Paroxysmal events Features of the spell Circumstances: When, where, how often? Premonitory signs and triggering factors Consciousness Movements (rhythm and distribution) Associated features (skin color, incontinence) Post-event period (lethargy, paralysis)

11 Shuddering attacks 7/666 NEPES series Start by 4months-3 years, end before 10yrs Multiple daily Rapid shivering, adduction of knees, flexion of head, elbows, knees and extension of the neck essential tremor in an immature brain?

13 Breath Holding spells There is a disease.in children from anger or grief, when the spirits are much stirred and run from the heart to the diaphrams forceably, and hinder or stop the breath.but when passion ceaseth, the symptom ceaseth Nicholas Culpepper

15 Breath Holding spells High Diaphragm on fluoroscopy Small periodic movements of the diaphragm spasm of the glottis Arterial O2 decreased by 20mmHg in 20 seconds LOC in 30 seconds Excessively complaint ribcage allowing collapse stretching airways simulation of maximum lung volumes inhibition of inspiration

17 Pallid breath-holding spell Better: reflex syncope Provoked by sudden fright of pain May gasp and cry very briefly Quiet, looses consciousness, and pale Limpness and diaphoresis, mild cyanosis Clonic movements, incontinence May sleep for several hours after the episode

19 Childhood periodic Syndromes Benign paroxysmal Vertigo Cyclic vomiting Abdominal migriane Benign paroxysmal torticollis

21 Synkoptein" (Greek for: "to cut short ) 1-3% of emergency department visits 30 to 50% at least one episode by adolescence 33-51% recurrence The incidence peaks in year olds

22 Cardiogenic VS Neurogenic Cardiovascular syncope is less frequent in children Higher mortality Check for: History of cardiac disease Absence of pre-syncope premonitory symptoms Abrupt LOC during exercise Causes Ao stenosis, cardiomyopathy, long Q-T, 1ry pulmonary Hypertension..

23 Neurocardiogenic Syncope (Vasodepressor: Vasovagal: Neurally mediated syncope). Prodromal phase: Light-headedness Blurred vision Epigastric discomfort Nausea Pallor Diaphoresis Environmental factors

24 What leads to Syncope? Peripheral venous pooling decreased venous return decreased ventricular filling mechanoreceptors dorsal vagal nucleus (or sympathetic withdrawal?) bradicardia, vasodilation, and hypotension Cerebral Hypoperfusion for 8-10 seconds LOC, abnormal tone (Syncope)

Tilt-table testing Head up right 60-80 degrees for 15-60 minutes Isoproteneral increase HR 20%; no added

25 Tilt-table testing Head up right degrees for minutes Isoproteneral increase HR 20%; no added benefit Pediatric data Sensitivity of 75% and specificity of 90% 11.6% had convulsive syncope 65% tonic 23% myclonic

26 Another Case 16 year old male healthy Fainting episodes Spells are reproducible by stretching both arms

27 Psychogenic syncope 1- Episodes extremely frequent 2-Not associated with injury or usual precipitating factors 3-Onset in supine position 4-Fails to regain consciousness rapidly even in supine position 5-Remarkable indifference to their syncope

29 Seizure, Reflux, Dystonia?

30 Investigations: Prolactin Specificity 96% Sensitivity generalized tonic clonic seizures 60% Complex partial seizures 46% 10 to 20 minutes after a suspected event Elevation after tilt-test induced syncope Circadian fluctuations

Paroxysmal Kinesogenic dyskinesias Paroxysmal kinesogenic dystonia (PKD), or choreoathetosis PKC Aura: Tightness, paresthesias + epilepsy family history

32 Paroxysmal Kinesogenic dyskinesias Paroxysmal kinesogenic dystonia (PKD), or choreoathetosis PKC Aura: Tightness, paresthesias + epilepsy family history Precipitation by initiation or acceleration of movement, a duration of usually <1 min and always <5 min Multiple episodes per day benefit from anticonvulsants : CBZ, PHT

36 Gratification phenomena (Infantile masturbation) (1) onset after the age of 3 months and before 3 years (2) stereotyped episodes of variable duration (3) vocalizations with quiet grunting (4) facial flushing with diaphoresis (5) pressure on the perineum with characteristic posturing of the lower extremities Yang et al. Pediatrics Dec, 2005

37 Gratification phenomena (Infantile masturbation) (6) no alteration of consciousness (7) cessation with distraction (8) normal examination (9) normal laboratory studies Direct observation of the events is crucial, and the video camera is a useful tool that may help in the identification of masturbatory behavior. Yang et al. Pediatrics Dec, 2005

38 Nocturnal Frontal Lobe Epilepsy vs. Parasomnia Apparent full wakefulness after event Sudden, clear offset Hyperventilation, grunting respirations Dystonic, stiffening posture Settling Behaviors after episode (adjusting position, etc.) Rubbing nose or face Periods of motionless staring Crying, sobbing, coherent speech Derry et al; Sleep, 2009

40 Ambulatory & Video EEG monitoring

Khoo Teik Beng Paediatric Institute Hospital Kuala Lumpur

PAROXYSMAL NON-EPILEPTIC EVENTS Khoo Teik Beng Paediatric Institute Hospital Kuala Lumpur Epileptic seizures Transient clinical events that result from the abnormal, excessive activity of a set of cerebral