Pediatric Neurology
Emergencies
Graham Thompson
Cheri Nijssen-Jordan
2003/11/06
Objectives
Approach to H/A in Pediatrics
DDx of Pediatric H/A
Migraines in Pediatrics
Febrile Seizures
AAP recommendations
prophylaxis
Status Epilepticus
Idiopathic Facial Nerve Paralysis
treatment
Case#1
14 yo girl with headache for past 2 wks
Started intermitent but now always there (2-
3/10), sometimes throbs more (8/10) across
front of head
Started to feel nauseated, no emesis
Has been taking tylenol with minimal help
Has had previous H/A but only last few hrs
Dosen’t wake from sleep, doesn’t wake with
H/A
Case #1 cont.
No fevers, but feels stuffy/congested at times
No FHx migraine
No motion sickness
Had concussion playing hockey (AAA boys
league) 8 months ago, no change to current play
No behaviour/school changes
Took minocycline x 3-4 days then stopped
Headache
Pain sensitive structures around the head
Vessels, dura, meninges
Sinuses, teeth
Musclulature
In general (but not a rule!!!)
frontal pain- supratentorial structures and vessels via
trigeminal nv
Occipital pain – posterior fossa via cranial nvs
H/A patterns
Acute
Single episode
URTI, dental pain, bleed, initial migraine, cocaine/amphetamines
Acute-recurrent
Episodic pain with symptom-free intervals
Migraine, tension, cluster, substance abuse, HTN
Chronic progressive
Gradual increase in frequency and severity with worrisome features
Space occupying lesion, chronic meningitis, IIH, hydro, HTN
Chronic nonprogressive
> 15 H/A per month, > 4 months, > 4hrs
Mixed
Migraine or analgesic abuse
Headache - Epidemiology
Prevalence
7-9 yo - 60-69%
15 yo – 75%
Chief complaint in 0.7-1.3% of PED visits
Etiology of Heache in the PED
50
Viral Illness
40 Sinusitis
Percentage
Migraine
30
Post-traumatic
20 Viral Meningitis
Strep Pharyngitis
10 Tension
Other
0
Type of Headache
Headache in the PED
Serious etiology
19/288 (6.6%)
15 – viral meningitis
1 - Shunt malfunction
1 – oncology
1 – hydrocephalus
1 – punctate hemorrhage
All had abnormal hx or px
H/A in PED
Viral URTI 28.5 – 39%
Migraine 8.5 – 21%
Tension 1.5 - 29%
Trauma 1.3 - 20%
Tumor 1.5 - 4%
Shunt 2 – 11.5%
Other
Serious etiology – 6.6 – 15%
All but 1 child in all 4 studies - abN Hx or Px
Lewis DW Sem in Ped Neuro 2001 8(1):46
Headache in the PED
When to worry….
Chronic progressive pattern
H/A or vomiting on awakening
Age 3)
Hx
N/V, changes in gait, strength, personality, speech, school
Px
Papilledema, ataxia, abN EOM, DTR, Visual acuity
“THERE IS NO CLASSIC BRAIN TUMOR HEADACHE”
Pediatric Migraine – IHS Definition
Pediatric Migraine - Classification
Migraine without aura
Migraine with aura (classic)
Migraine equivalents
Pediatric Migraine - Epidemiology
3-5% of school-aged children - M>F
Up to 20% of adolescents - M 15 minutes
>1 in 24 hrs
Post-ictal involvement
Febrile Seizures - Risk Factors
Height of temperatture
Male
Family Hx of febrile seizures
Febrile Seizures - Epidemiology
2-5% of children
Peak onset is 1 yo, mean = 19 – 23 mo
Sz is 1st sign of illness in 25-50% of cases
FHx in 25-40%
1 parent – 4.4X risk
2 parents – 20X risk
Sibling – 3.6X risk
2nd degree relatives 2.7X risk
Infections?
Of 445 cases 34% AOM, 12% URTI, 6% viral exanthem 6% pneumonia
HHV 6/7 – may not be as common as previously thought
HSV & influenza A
Same baseline risk for invasive bacteremia (1%)
Febrile Seizures and Immunizations
Increased in
Day of DPTP-Hib
8-14 days post MMR
NO long term consequences
Febrile Seizures – AAP Guidelines
AAP Guidelines
To LP or not?
All studies are retrospective
503 pts with meningitis (V&B) age 2mo – 15 y
115 had sz but none were isolated sz
452 6mo-5yo with fever and sz
15 meningitis, all had septic appearance, photophobia, stiff
neck or Kernig (+)
241 6 mo-5yo with fever and sz
All had LP, 11 bacteria menngitis, all had one of following
1)seen MD in past 48hrs 2)sz recurring in ED 3)focal sz 4)
abN neuro exam
Febrile Seizures – Recurrence Risk
1/3 will have recurrence, ½ of these will have
mutiple episodes
Highest in
Young at 1st presentation
FHx
Low fever
Short duration between start of fever and sz (1 focal complex febrile sz – may by up to
30%
Cognitive Outcome
No changes in several american and british studies
Febrile Seizures – Prevention?
Antipyretics
No evidence to support use
Uhari et al J peds 1995 126:991 180 kids RDBPC (plac + plac,
plac + acet, diaz + acet, diaz + plac) no difference in
recurrence x2yrs
Schnaiderman et al Eur J Peds 1993 152:747 104 kids RCT
acet q4h or prn, no difference
Van Stuijvenberg et al Peds 1998 102:1 230 kids RDBPC
ibuprofen to plac no diff X1yr
Meremikwa et al Cochrane Database 2002:4 no evidence
supporting use of acet to prevent Febrile Sz
Febrile Seizures – Prevention?
Antiepileptics
Phenobarb – definite reduction in recurrences (OR 0.54
NNT = 8) if taken continuously, no difference if intermitent
Hyperactivity, irritability, bld levels, sleep d/o, SJS
VPA – definite reduction in recurrences (OR = 0.09 NNT =
4) also if continous
Hepatotoxicity, thrombocytopenia, pancreatitis, wt change
Intermitent diazepam – reduction by 44% per person per year
Ataxia, lethary, irritibility, sleep d/o
Pheytoin, carbamazepine – no difference
Febrile Sz papers
Warden CR et al Evaluation and Management of
febrile seizures in the Out-of-hospital and
emergency department setting Annals of
Emergency Medicine 2003 41(2)
Baumann RJ et al Treatment of Children with
Simple Febrile Seizures: the AAP Practice
Parameter Pediatric Neurology 2000 23:11
Case #3
18 mo girl started seizing @ home 2 hours ago
Stiffened, unresponsive, R arm twitching the L starting
Lasted about 4 minutes
Very sleepy post ictal, not responding to parents
Had 2 more similar szs in past 1hr so brought in by EMS, still
not responding to voice (but maintaining airway!!)
Szs again just as you walk in the room
Currently on tegretol because other meds didn’t work
No fevers, no intercurrent illness
Last sz 3 months ago
Status Epilepticus
WHO Definition
“a condition characterized by and epileptic sz that is
sufficitnely prolonged or repeated at sufficiently
brief intervals so as to produce an unvaring or
enduring epileptic condition”
Reality
Continuous or repetitive seizure activity of at least
30 minutes with failure to regain consciousness
between convulsions.
S.E. Etiology in Pediatrics
26% acute CNS insult
Bleed/trauma
Infection
21% underlying sz D/O
Sudden discontinuation of Meds
Fever
53% unknown!!!
SE Problems
Hypoxia
Impaired ventilation, increased secretions, increased O2 consumption,
impaired O2 delivery, metabolic and respiratory acidosis
Brain injury
Hypoxia and perfusion related (CBF unable to keep up with demands
may occur more frequently with younger age leading to MR, behaviour
changes, motor deficits
Morbidity
Age dependent, up to 30% in 3yo
Mortality
3%
S. E. - Therapies
Benzos
Phenytoin/Fosphenytoin
Phenobarb
Refractory S.E. Tx
S.E. - Benzos
Lorazepam vs Diazepam vs Midazolam
Loraz has smaller volume of distribution, longer
acting (12-24 hrs vs 5-30 min), less respiratory
depression
Small study (n=86) loraz 3% vs diaz 31%
Rectal diaz has less resp depression than IV
Midazolam not used in newborns as may lower
sz threshold
Midaz may be used PO, IV, IM, IN
S.E. – Benzos
Cochrane Review August 2003
Lorazepam vs other AED in the PED
Only one study
No difference in stopping szs, recurrence rate,
respiratory depression rate or need for rescue AED
in IV lorazepam vs diazepam
Statistically significant diff in rectal loraz over diaz,
but numbers too small to make recomendations
S.E. – Phenytoin/Fosphenytoin
Fosphenytoin
Phosphate ester pro-drug of phenytoin
Advantages of Fosphenytoin
Not in propylene glycol base so less tissue toxicity and cardiac side effects
(hypotension, bradys, VF)
pH =8 (phenytoin =12)
Compatible with any IV solution including Dextrose
Rapid IV infusion rate (up to 3X faster)
IM route possible,
Disadvantages
Pruritis (usually face and perineum) in up to 50%
paresthesia
High cost – 1G = $90 compared to $6
Recent studies show may have overall institutional cost savings because of
less side effects
S.E. - Barbituates
Phenobarbital
Drug of choice in neonates
High sedative and CR depression which may be
enhance with prior benzo Tx
Prolonged start time (15-20 minutes)
Refractory S.E.
Failure to respond to sequential treatment with
benzo, phenytoin, phenobarb
Midazolam infusion
Propofol infusion
VPA infusion
AED Doses - Pediatric
Drug Dose Onset
Lorazepam 0.05-0.1 mg/kg 2-3 min
IV/PR
Diazepam 0.1-0.3 mg/kgIV 1-3 min
0.5 mg/kg PR
Phenytoin 10-20 mg/kg IV 10-30 min
Fosphenytoin 20 mgPE/kg 10-30 min
Phenobarbital 20 mg/kg 10-20 min
Midazolam 0.1-0.2 mg/kg Inf. 1-3 ug/kg/m
Propofol 1-2 mg/kg Inf. 25-100 ug/kg/min
VPA 15-20mg/kg Inf. 5 mg/kg/hr
Case #4
6 yo girl brought in by mother because teacher
said her face wasn’t working properly
Can’t smile properly, L side doesn’t move
Had pain beside L ear yesterday
Cough and runny nose 2 wks ago
No fever, no rash
Bell’s Palsy
(Idiopathic Facial Nerve Palsy)
Unilateral facial nerve palsy
Sudden onset 1-2 wks post viral infection
Most common infectious involvement
EBV, HSV, mumps, lyme disease, other viral
? Immune demyelination vs edema
Bell’s Palsy
DDx
Lyme disaese (may be up to 50% in endemic areasa)
AOM, mastoiditis
Ramsay Hunt (Herpes Zoster Oticus)
NMD (Myasthenia gravis)
Tumor
Leukemia/lymphoma
Schwannoma
parotid
Neurofibromatosis
Brainstem infarcts
Stroke
Trauma
Bell’s Palsy
Clinical
Unilateral
Pain may precede
Peripheral nerve weakness (lower motor neuron) so involves
upper and lower face
Flat nasolabial fold
Difficulty closing eye – exposure keratitis
Difficulty smiling
½ may loose taste on anterior ipsilat 2/3 of tongue (dysgeusia)
Decreased tearing vs crocodile tears (epiphoria)
hyperacusis
Bell’s Palsy - Treatment
Eye protection
85-90% in children spontaneously resolve with
most occurring within 2 months of onset
Bell’s Palsy - Treatment
Steroids?
Cochrane review
3 studies, n = 117, not great randomization, 1 study had no control
group
No reduction in incomplete recovery or cosmetically disabling
sequelae
Couldn’t recommend
Salman et al J child Neuro 2001 16:565
Systematic review of Bell’s Palsy in children <18
8 trials, 1 exclusively children, 5 randomized, 5 blinded
No evidence for benefit
Bell’s Palsy - Treatment
Acyclovir?
Cochrane review
2 studies Acyc + steroid vs Acyc, acyc vs steroid
Couldn’t comment on primary outcomes (reduction of incomplete
recovery @ 1 yr, adverse events, paralysis @ 6mo) as not enough data
Couldn’t recommend
De Diego et al Laryngoscope 1998 108(4):573
101 pts randomized to prednisone 1mg/kg OD x10 vs acyclovir 800
mg TID x10
Recovery @ 3 months using nv function tests higher in steroid vs
acyclovir
Adour et al Ann Otol Rhinol Laryngol 1996 105:371
Quicker return to functional muscle control with combined acyclovir
and prednisone
Case #5
2 mo infant brought in with lethargy and vomiting x 8
in last 4 hours this pm
Normal U/O and BM
Felt “warm” to touch @ home
Had been crying all night for past 2 nights
Previously well, IUTD, had tylenol yest. to try to stop
crying
Term, uncomplicated G1 preg
Child @ day home with other kids, all well
Case #5 cont
37.8 160 22 – poor effort 94% BP – UA
Looks unwell, pale
Poor spont. movement
Font slightly full
Pupils a bit sluggish, 4mm
No source for infection on exam
SWU done
LP grossly bloody, not clearing over 4 tubes
started on A/B
Non-Accidental Trauma
DON’T FORGET IT IN YOUR
DIFFERENTIAL