Pediatric Neurology Emergencies

1
Pediatric Neurology Emergencies

• Graham Thompson
• Cheri Nijssen-Jordan
• 2003/11/06

2
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

3
Case1

• 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
• Dosent wake from sleep, doesnt wake with H/A

4
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

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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

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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
• gt 15 H/A per month, gt 4 months, gt 4hrs
• Mixed
• Migraine or analgesic abuse

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Headache - Epidemiology

• Prevalence
• 7-9 yo - 60-69
• 15 yo 75
• Chief complaint in 0.7-1.3 of PED visits

8

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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

10
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

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Headache in the PED

• When to worry.
• Chronic progressive pattern
• H/A or vomiting on awakening
• Age lt 3yo
• thunderclap headache
• Focal neuro signs
• VP shunt
• Neurocutaneous syndrome (NF, TS)

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Headache and Tumors

• Childhood Brain Tumor Consortium (JNeurooncol
  1991 1031)
• 3291 tumors
• 62 had H/A
• 98 of these had at least 1 problem on Hx/Px (50
  had gt3)
• 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

13
Pediatric Migraine IHS Definition
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Pediatric Migraine - Classification

• Migraine without aura
• Migraine with aura (classic)
• Migraine equivalents

15
Pediatric Migraine - Epidemiology

• 3-5 of school-aged children - MgtF
• Up to 20 of adolescents - MltF
• Highest frequence Mon,Tues,Wed, daytime

16
Pediatric Migraine Tx Options

• Non-medicinal
• NSAIDs
• Ibuprofen
• Ketorolac
• Dopamine Antagonists
• phenothiazines
• 5-HT agonists
• Ergots
• Triptans
• Novel Therapies
• Valproate

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Migraine - NSAIDs

• RDBPC study of 6-12 yo using 7.5 mg Ibuprofen
• Significant reduction in pain, nausea, need for
  rescue med, significant increase in pain free
  status _at_ 2 hrs
• Lewis et al Headache 2002 42780
• RDBCO study of Ibuprofen 10mg/kg, acetaminophen
  15 mg/kg and placebo
• Significant reduction in pain _at_ 2 hrs.
• Ibuprofen 2X as likely as acet (OR 2.2 CI 1.1-40)
• Hamlainen et al Neuro 1997 48103
• Ketorolac
• No good peds studies
• Advantage - not PO intake

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Migraine Dopamine Antagonists

• Metoclopramide, prochlorperazine, promethazine
• Antiemetic effect helpful in gastric stasis
  delayed absorptionseen in migraines
• Drowsiness, hypotension, extrapyramidal effects

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Migraine 5-HT Agonists

• Triptans
• 50 6-8 yo 0.06 mg/kg SC sumatriptan
• 78 red. in pain (mod-sev to none-mild) in 60 min
• Recurrence 6
• Flushing, dizziness, tingling, chest pain, neck
  stiffness

20
Triptans in Teens

• 1 yr study of 437 adolescents treated 3273
  migraines (10 mg nasal sumatriptan)
• 76 pain relief in 2 hrs, 43 pain free by 2 hrs
• IN dosing ranging from 5-20 mg
• Improvement in VAS _at_ 2 hours, and has low rates
  of recurrence (6)
• Note adolescent had huge placebo effect (up to
  57)
• Winner et al Headache 2002 42675
• Winner et al Headache 2003 43451

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Ergots

• DHE less side effects that Ergotamine (N/V,
  hypertension, flushing, angina-like episodes)
• 0.1-0.5 mg IV
• Rarely used in ED setting in children

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Valproate

• No pediatric studies
• Annals EM June 2003
• 40 pts received 500 cc NS then randomized to 500
  mg VPA vs 10 mg prochlorperazine
• VAS score q 15 min x 1 hr
• VPA had poor response in terms of pain and nausea

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Drugs in Pediatric Migraine
Drug Dose Route
Ibuprofen 10 mg/kg PO
Naproxen 5 mg/kg PO
Ketorolac 0.5 mg/kg (max 30mg/dose) IV/IM
Metoclopramide 1-2 mg/kg (max 10mg/dose) PO/IV/IM
Prochlorperazine 0.1 mg/kg PO/IV/IM
Sumatriptan Child -0.06 mg/kg Adol 6mg Adol 5-20mg SC SC IN
DHE 0.1 0.5 mg IV/IM
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Migraine Equivalents

• Benign paroxysmal torticollis
• Infancy, torticollis, pallor, vomiting,
  behaviour, bilat, 4hrs 4 days, resolve by mid
  childhood
• Benign paroxysmal vertigo (38)
• Unsteadiness, pallor, fear, spinning, 1-5 min.
  1-3 yo, usually leaves within 2-3 yrs
• Abdominal migraine vs cyclical vomiting (18.5)
• Crampy, periumbilical, nausea, vomiting, pallor,
  4 - 10 yo, may develop classic migraines

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Migraine Equivalents

• Acephalgic migraine (28.7)
• Visual auras without H/A, lt 10 min, 5-12 yo
• alice in wonderland
• Acute confusional migraine
• Confusion, agitiation, memory loss, adolescence
• Retrospective!!!!!!
• Made up 9.9 of migraine visits to neurologist

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Pediatric H/A Reviews

• Qureshi F Lewis D Managing headache in the
  Pediatric Emergency Department Clinical Pediatric
  Emergency Medicine 2003 4(3)
• Lewis D Headache in the Pediatric Emergency
  Department Seminars in Pediatric Neurology 2001
  8(1)46

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Case 2

• 16 mo boy previously well brought by EMS because
  of sz at home
• Lasted 1 min
• Eyes rolled back, arms twitching
• Crying after
• Has had URTI over past 2-3 days

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Simple Febrile Seizures - Definition

• Normal preceding neuro and dvt status
• Age 6 mo 5 yrs
• brief (lt15 minutes)
• generalized seizure
• only once in a 24 hour period
• febrile child who did not have either central
  nervous system infection or a severe metabolic
  disturbance.

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Complex Febrile Seizure

• Focal
• gt 15 minutes
• gt1 in 24 hrs
• Post-ictal involvement

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Febrile Seizures - Risk Factors

• Height of temperatture
• Male
• Family Hx of febrile seizures

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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)

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Febrile Seizures and Immunizations

• Increased in
• Day of DPTP-Hib
• 8-14 days post MMR
• NO long term consequences

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Febrile Seizures AAP Guidelines
34
AAP Guidelines
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To LP or not?

• All studies are retrospective
• 503 pts with meningitis (VB) 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

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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 (lt24
  hrs)
• Up to ½ of recurrences occur in 1st 2 hrs

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Febrile Seizures Recurrence Risk

• Meta-analysis of recurrence risk
• Onset younger than 12 mo
• Febrile or afebrile seizure in 1st degree
  relative
• Rectal temp lt 40 C during 1st sz
• Offringa et al

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Consequences of Febrile Seizures?

• Epilepsy
• General population 1
• Simple febrile szs risk of afebrile szs by age
  25 increases to 2.4
• gt1 focal complex febrile sz may by up to
  30
• Cognitive Outcome
• No changes in several american and british
  studies

39
Febrile Seizures Prevention?

• Antipyretics
• No evidence to support use
• Uhari et al J peds 1995 126991 180 kids RDBPC
  (plac plac, plac acet, diaz acet, diaz
  plac) no difference in recurrence x2yrs
• Schnaiderman et al Eur J Peds 1993 152747 104
  kids RCT acet q4h or prn, no difference
• Van Stuijvenberg et al Peds 1998 1021 230 kids
  RDBPC ibuprofen to plac no diff X1yr
• Meremikwa et al Cochrane Database 20024 no
  evidence supporting use of acet to prevent
  Febrile Sz

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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

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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 2311

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Case 3

• 18 mo girl started seizing _at_ 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 didnt
  work
• No fevers, no intercurrent illness
• Last sz 3 months ago

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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.

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S.E. Etiology in Pediatrics

• 26 acute CNS insult
• Bleed/trauma
• Infection
• 21 underlying sz D/O
• Sudden discontinuation of Meds
• Fever
• 53 unknown!!!

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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 lt1 yo, 6 in those gt
  3yo
• Mortality
• 3

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S. E. - Therapies

• Benzos
• Phenytoin/Fosphenytoin
• Phenobarb
• Refractory S.E. Tx

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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 (n86) 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

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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

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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

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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)

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Refractory S.E.

• Failure to respond to sequential treatment with
  benzo, phenytoin, phenobarb
• Midazolam infusion
• Propofol infusion
• VPA infusion

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AED Doses - Pediatric
Drug Dose Onset
Lorazepam 0.05-0.1 mg/kg IV/PR 2-3 min
Diazepam 0.1-0.3 mg/kgIV 0.5 mg/kg PR 1-3 min
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
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Case 4

• 6 yo girl brought in by mother because teacher
  said her face wasnt working properly
• Cant smile properly, L side doesnt move
• Had pain beside L ear yesterday
• Cough and runny nose 2 wks ago
• No fever, no rash

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Bells 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

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Bells 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

56
Bells 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

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Bells Palsy - Treatment

• Eye protection
• 85-90 in children spontaneously resolve with
  most occurring within 2 months of onset

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Bells 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
• Couldnt recommend
• Salman et al J child Neuro 2001 16565
• Systematic review of Bells Palsy in children lt18
• 8 trials, 1 exclusively children, 5 randomized,
  5 blinded
• No evidence for benefit

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Bells Palsy - Treatment

• Acyclovir?
• Cochrane review
• 2 studies Acyc steroid vs Acyc, acyc vs steroid
• Couldnt comment on primary outcomes (reduction
  of incomplete recovery _at_ 1 yr, adverse events,
  paralysis _at_ 6mo) as not enough data
• Couldnt 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 _at_ 3 months using nv function tests
  higher in steroid vs acyclovir
• Adour et al Ann Otol Rhinol Laryngol 1996
  105371
• Quicker return to functional muscle control with
  combined acyclovir and prednisone

60
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 _at_ 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 _at_ day home with other kids, all well

61
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

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Non-Accidental Trauma

• DONT FORGET IT IN YOUR
  DIFFERENTIAL