Nonepileptic Paroxysmal Events in Children

Educational Objectives

The goal of this program is to improve management of common pediatric nonseizure activity in children. After hearing and assimilating this program, the clinician will be better able to:

1. Describe common types of nonepileptic neurologic events in children.
2. Identify conditions that can provoke vertigo in children.
3. Determine when treatment is required for tics, parasomnias, and other neurologic events in children.

Summary

Seizure activity: typically occurs at regular intervals (jerking alternates with relaxing); unilateral seizure most common presentation of small stroke in newborn; irregular shaking while falling asleep likely benign neonatal myoclonus (usually occurs 3 wk to 3 mo of age; resolves)

Benign positional vertigo (BPV): differential diagnosis — partial epilepsy; medulloblastoma (symptoms constant); Ménière disease (features hearing loss, occurs in older children); perilymphatic fistula (subacute, persists over weeks); BPV — on migraine spectrum; resolves spontaneously; brief episodes of vertigo typically occur in toddlers; children appear frightened and pale and may complain of dizziness; nystagmus apparent; vomiting prominent symptom; no loss of consciousness; treatment supportive

Sandifer syndrome: children drool and posture; caused by gastroesophageal reflux disease; typically occurs ≤10 yr of age (most common in infants and toddlers)

Neurologic episodes: electrocardiography (ECG) more important than electroencephalography (EEG), as arrhythmias much more likely fatal than seizures; consider psychiatric disorders and masturbation; may record video of activity on phone and send to neurologist; true seizure — eyes open; EEG rarely normal with daily seizures; seizures tend to produce more positive than negative phenomena (latter include paleness, bradycardia; former include tachycardia); not represented by directed acts of violence (automatism purposeless); conversion disorder and malingering rare <10 yr of age

Evaluation: obtain history and perform physical examination; hyperventilation can produce some types of epilepsy; full laboratory analysis not very useful, but toxicology screen important; consider pregnancy test in teenage girls; obtain computed tomography of head in emergency, but avoid excessive radiation exposure; ECG important; pH probe currently out of favor; EEG used with increasing frequency

Breath-holding spells: typically in toddlers; lusty crying followed by cessation of air movement; children frightened and apprehensive; gaze deviation possible; hypoxic jerks similar in appearance to seizures; child may be “winded” or appear postictal; duration <1 min; runs in families; tends to recur; formerly called cyanotic pallid spells (paleness likely related to arrhythmia; consult cardiologist for reflex asystole); treatment — supportive care; iron therapy

Dizziness and vertigo: syncope — inadequate blood flow to brain causes fainting; provoked by Valsalva maneuver; classically occurs in underhydrated or thin patients; dizziness — vague complaints; vertigo — sense of motion with illusion of rotation; feeling of spinning inside own head, floating, or being out of body typically indicates psychiatric issue; diagnosing dizziness — wide differential diagnosis includes hypotension, arrhythmia, and hypoglycemia; mal de debarquement syndrome (feeling of disembarking from boat) more common in adults; psychiatric disorder diagnosis of exclusion; diagnosing vertigo — assess frequency and duration; seizures 90 to 120 sec (often overestimated by factor of 2-3); benign paroxysmal positional vertigo (BPPV) results from calculus in semicircular canal (severe vertigo with nystagmus, nausea, vomiting); basilar migraine causes cranial nerve symptoms; vestibular neuritis (after, eg, herpes zoster) lasts several days; other causes include perimeningeal reaction to otitis media, Ménière disease, toxins, and drugs (eg, aminoglycosides); psychiatric disorders do not cause nystagmus

Assessment: look for spontaneous horizontal nystagmus with vertigo; Romberg sign assesses for vestibular or proprioceptive disorder; test for BPPV by moving head quickly with 45° downward and 45° lateral rotation (produces nystagmus, nausea); caloric and orthostatic testing not commonly used; test hearing, ECG, and EEG

Management: syncope — β blockers; phenylephrine; postural orthostatic tachycardia syndrome — fluid replacement; salty foods; steroid treatment rarely needed; BPPV — Epley positioning exercises; Ménière disease — dietary triggers alleged; mal de debarquement syndrome — benzodiazepines; consult neurologist or otorhinolaryngologist; vertigo — do not use vestibular suppressant (eg, diphenhydramine [eg, Allermax, Benadryl, Diphedryl]) >3 wk as semicircular canals reset

Parasomnias: events that occur during rapid eye movement (REM) sleep remembered (eg, nightmares), unlike events during non-REM sleep (eg, night terrors, sleep walking); hypnogogic myoclonus — normal and common; myoclonic jerking occurs while falling asleep; insomnia — adolescents and children with developmental delay may not function on 24-hr cycle; night terrors — typically occur ≈5 yr of age; children scream and exhibit diaphoresis, tachycardia, and widely dilated pupils; child partially wakens, then falls back asleep; typically lasts ≈5 min; co-exists with sleep walking; obstructive sleep apnea — signs include excessive daytime sleepiness, withdrawn affect, hyperactivity, recurrent infections, and failure to thrive; treated with tonsillectomy (rarely with nasal continuous positive airway pressure); narcolepsy — excessive daytime somnolence, even after adequate sleep; genetic association complex; cataplexy involves sudden loss of tone (much less common); vivid hypnogogic hallucinations; patients may have sleep paralysis; rarely associated with neurodegenerative disease

Management: delayed sleep phase syndrome — chronotherapy; light therapy; melatonin (synthetic only); adverse effects of diazepam and clonazepam (Klonopin) outweigh benefit; excessive daytime somnolence — stimulants (eg, methylphenidate); cataplexy — sodium oxybutate (consult)

Tics: extremely common; typically begin at 6 to 7 yr of age; simple or complex; may manifest as vocalization, blinking, shrugging, or throat clearing; coprolalia (swearing) rare; familial association; briefly suppressible with voluntary effort; more frequent with stress and sleep deprivation; patients try to convert tic into voluntary movement to avoid notice; usually resolves in ≈2 mo (do not rush to treatment); Tourette syndrome — vocal and motor tics present, lasting >1 yr (diagnosis likely after 3-4 mo); often associated with attention-deficit/hyperactivity disorder and obsessive-compulsive disorder (stimulant therapy not contraindicated); treatment — only for tics that stigmatize, interfere with daily function, or cause pain or discomfort; mainstays guanfacine and clonidine; haloperidol out of favor; fluphenazine and pimozide used less; reduction in tics may not occur for several weeks

Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus (PANDAS), pediatric acute-onset neuropsychiatric syndrome (PANS), and childhood acute neuropsychiatric symptoms (CANS): PANDAS — theorized that streptococcal infection and other conditions can cause neuroimmune phenomenon; onset abrupt with temporal relation to β-hemolytic streptococcal infection; patients have hyperactivity, chorea, or tics; however, most tics have abrupt onset and ≥20% of children exposed to Streptococcus; evidence sparse; overwhelming majority of children with tics do not have PANDAS; CANS and PANS — definition broader

Other conditions: hyperekplexia — exaggerated startle response; genetic (sometimes associated with serious disorder); shudder attacks — benign; spasmus nutans — triad of head bobbing, mild torticollis, and nystagmus at 6 mo to 4 yr of age (resolves); infantile spasms — encephalopathy causes crankiness; hyperekplexia — benign condition (resolves); opsoclonus — rapid eye movements in vertical and horizontal plane (saccades); uncommon; presenting feature of neuroblastoma in 3% of children 18 to 24 mo

Comprehensive behavioral therapy (CBIT): formerly called habit-reversal therapy; effective for tics; child taught to use other movements to suppress tics by occupational therapist

Readings

Efron D et al: Tics and Tourette syndrome. J Paediatr Child Health 2018 Oct;54(10):1148-53; Ekambaram V et al: Non-rapid eye movement arousal parasomnias in children. Pediatr Ann 2017 Sep 1;46(9):e327-31. doi: 10.3928/19382359-20170814-01; Ganos C et al: Tics in the pediatric population: pragmatic management. Mov Disord Clin Pract 2017 Mar-Apr;4(2):160-72; Jahn K: Vertigo and dizziness in children. Handb Clin Neurol 2016;137:353-63; Lei LY et al: Evaluating and managing postural tachycardia syndrome. Cleve Clin J Med 2019 May;86(5):333-44; Leung AKC et al: Breath-holding spells in pediatrics: a narrative review of the current evidence. Curr Pediatr Rev 2019;15(1):22-29; Mandalà M et al: Benign positional paroxysmal vertigo treatment: a practical update. Curr Treat Options Neurol 2019 Dec 5;21(12):66; Muncie HL et al: Dizziness: approach to evaluation and management. Am Fam Physician 2017 Feb 1;95(3):154-62; Sukhodolsky DG et al: Moderators and predictors of response to behavior therapy for tics in Tourette syndrome. Eur J Paediatr Neurol 2018 Mar;22(2):316-20; Van Horn NL et al: Night terrors. StatPearls [Internet]; StatPearls Publishing: Treasure Island (FL);2019 Nov 16; Zibordi F et al: CANS: childhood acute neuropsychiatric syndromes. Eur J Paediatr Neurol 2018 Mar;22(2):316-20; Yang M: Newborn neurologic examination. Neurology 2004 Apr 13;62(7):e15-7. doi: 10.1212/wnl.62.7.e15.

Disclosures

For this program, members of the faculty and planning committee reported nothing to disclose.

Acknowledgements

Dr. Fisher was recorded at the Aloha Update: Pediatrics 2019, presented by the American Academy of Pediatrics, District IX, California Chapter 2, in association with Children’s Hospital Los Angeles Medical Group and held October 12-18, 2019, on Kauai, HI. For information about upcoming CME conferences from the Children’s Hospital Los Angeles Medical Group, please visit www.chla.org/cme-conferences. The Audio Digest Foundation thanks Dr. Fisher, the American Academy of Pediatrics, District IX, California Chapter 2, and Children’s Hospital Los Angeles Medical Group for their cooperation in the production of this program.

CME/CE INFO

Accreditation:

The Audio- Digest Foundation is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The Audio- Digest Foundation designates this enduring material for a maximum of 0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Audio Digest Foundation is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's (ANCC's) Commission on Accreditation. Audio Digest Foundation designates this activity for 0 CE contact hours.

Lecture ID:

NE110802

Expiration:

This CME course qualifies for AMA PRA Category 1 Credits™ for 3 years from the date of publication.

Instructions:

To earn CME/CE credit for this course, you must complete all the following components in the order recommended: (1) Review introductory course content, including Educational Objectives and Faculty/Planner Disclosures; (2) Listen to the audio program and review accompanying learning materials; (3) Complete posttest (only after completing Step 2) and earn a passing score of at least 80%. Taking the course Pretest and completing the Evaluation Survey are strongly recommended (but not mandatory) components of completing this CME/CE course.

Estimated time to complete this CME/CE course:

Approximately 2x the length of the recorded lecture to account for time spent studying accompanying learning materials and completing tests.