Non-Epileptic Paroxysmal Disorders in Children

Question 1

t/f a pause in breathing for greater than 20 s is commonly seen in premature children during active sleep

Answer 1

true, due to brainstem immaturity

Question 2

t/f when there is a real seizure the heart rate increases

Answer 2

true

Question 3

apnea vs seizure

Answer 3

apnea has eye opening or closure depending on which part of sleep

Question 4

etiology of paroxysmal extreme pain disorder

Answer 4

scn9a sodium channel gene mutation

life-long

Question 5

autonomic manifestations of pepd

Answer 5

skin flushing in all cases
harlequin color change and tonic attacks
dramatic syncope with bradycardia and sometimes asystole

Question 6

pepd in older children

Answer 6

attacks of excruciating deep burning pain often in rectal, ocular, or jaw areas but also diffusely in some
triggered by defecation, cold, wind, eating, and emotion

Question 7

what is jitteriness

Answer 7

excessive response to stimulation (touch or loud noise)

typically awake

Question 8

associations of jitteriness

Answer 8

drug withdrawal
hypocalcemia
hypoglycemia
hypoxic ischemic encephalopathy

Question 9

jitteriness is lessened by

Answer 9

consoling the child
removing the stimulus
relaxing the affected limb

Question 10

what is paroxysmal tonic upgaze

Answer 10

starts before 3 mos
continuous or episodic upward gaze
preserves horizontal eye movements
normal imaging, labs, and neuro exam

Question 11

what is benign neonatal sleep myoclonus

Answer 11

repetitive myoclonic jerks
non-rapid eye movement sleep
bilateral symmetric movement of arms and legs

Question 12

factors that distinguish myoclonic jerks from epileptic events

Answer 12

absence of autonomic disturbances
myoclonic jerks occurring only while asleep
normal eeg
normal neuro and dev exam

Question 13

what are breath holding spells

Answer 13

occurs 6 mos-6 yo

patho: dysfunction of ans, iron deficiency anemia

Question 14

what are cyanotic breath holding spells

Answer 14

trivial precipitant -> emotional response > crying -> breath holding in forced expiration -> collapse with limpness and loss of consciousness

Question 15

effects of prolonged apnea

Answer 15

decorticate or decerebrate posturing

Question 16

what are pallid breath holding spells

Answer 16

child loses consciousness after a minor fall or blow to head or upper body

loss of consciousness delayed up to 30s after trauma

• -> pale, diaphoretic, limp
• -> generalized increased tone of trunk and extremities + incontinence + low amplitude clonus

Question 17

diagnosis of pallid breath holding spells

Answer 17

reproduced with 10 seconds of ocular pressure during ecg monitoring

Question 18

characteristics of benign myoclonus of infancy

Answer 18

myoclonus, spasms with brief tonic contractions, shuddering, and/or atonia
occurs in clusters, frequently mealtime
occur only when awake

Question 19

shuddering attacks

Answer 19

brief episodes of altered muscle tone
stiffening, flexion, and elevation of the arms with a low amplitude tremor
spells during feeding, excited, or distressed (stimulus overflow)
never during sleep or being held
resolve by 2nd decade

Question 20

sandifer syndrome

Answer 20

intermittent paroxysmal spells of generalized stiffening and opisthotonic posturing that are caused by ger in infants
pain response to acidic reflux into esophagus
associated with feeding (30 mins after)

Question 21

dystonia

Answer 21

abnormal posture due to sustained contraction of both agonist and antagonist muscle groups

Question 22

common etiology of dystonia

Answer 22

acute reaction to drugs

Question 23

basilar type migraines

Answer 23

acute neurologic events without significant headache

loss or alteration of consciousness

Question 24

when to suspect migrain

Answer 24

vomiting with the episode

history of severe headaches without neuro symptoms

Question 25

a rare type of migraine with a motor aura of weakness

Answer 25

familial hemiplegic migraine

Question 26

manifestations of familial hemiplegic migraine

Answer 26

negative phenomena (absence of symptoms that were there before)
positive phenomena
progressive and successive occurrence of visual, sensory, motor, aphasic, and basilar s/sx

Question 27

benign paroxysmal vertigo

Answer 27

recurrent episodes of brief disequilibrium where child is frightened and off balance
reaching out to steady himself
<1 mi, recurring and remitting course
resolves without treatment

Question 28

factors that distinguish nonepileptic staring spells from true absence seizures

Answer 28

when the child is bored or active
never associated with automatisms, myoclonus, or other motor signs
can be interrupted by tactile or vocal stimulation

Question 29

psychogenic nonepileptic seizures

Answer 29

dramatic behavioral events that are misdiagnosed
teenagers with affective and anxiety disorders

generalized atypical appearing motor activity and prompt return of consciousness

Question 30

t/f comorbid epilepsy is common in psychogenic nonepileptic seizures

Answer 30

true

Question 31

syncope

Answer 31

abrupt loss of consciousness due to interruption of energy sources to brain because of sudden reduction of cerebral perfusion
vasovagal in origin
lack of postictal state

Question 32

factors distinguishing syncope and seizures

Answer 32

emotional stress
prolonged upright posture
pain

Question 33

motor disorders

Answer 33

rarely confused with seizures when its sustained rather than episodic
no impairment of consciousness