Download Common Indications for CT (That I want to remember

Seth Woolf, MD
Fellow, Pediatric Emergency Medicine
July 20, 2016
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It’s 0230 on a Saturday morning and the rush
has finally slowed down and you want to
catch up on some charts
A patch comes through to Yale Pediatrics
from EMS
What’s your patient age/diagnosis?
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16yo female with alcohol intoxication,
actively vomiting
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16yo female with alcohol intoxication,
actively vomiting
2yo with croupy cough
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16yo female with alcohol intoxication,
actively vomiting
2yo with croupy cough
11 month old with “fever” to 1000 F
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16yo female with alcohol intoxication,
actively vomiting
2yo with croupy cough
11 month old with “fever” to 1000 F
20 month old status-post seizure, now with
temperature of 39.20 C
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…are common!
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Occur in approximately 2-5% of children
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Have familial predisposition
◦ 10-20% of patients have a parent or sibling who has
had or will have a febrile seizure
◦ Monozygotic twins with much higher concordance
rate that dizygotic twins or siblings
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Large prospective study (Klein et al. Pediatrics.
2010 Jul;126(1):e1-8) and large cohort
(MacDonald et al. CMAJ 2014)
◦ Higher risk (twice as high) of febrile seizures for
MMRV vs MMR + V
 7-10 days post-vaccine
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Large cohort study (Barlow et al. N Engl J Med.
2001;345(9):656.) showed increased risk of
febrile seizures after vaccines
◦ DPT – increased on the day of vaccine (RR = 5.7)
 DTaP (acellular vs. whole cell) has replaced in US
◦ MMR – increased 8-14 days later (RR = 2.83)
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“The Rule of 6’s”
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“The Rule of 6’s”
◦ 6 months – 6 years old
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Fever
Generalized seizure with NO focality
◦ Usually clonic, can be atonic or tonic as well
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Less than 15 minutes (median duration is 3-4
min.)
Only 1 episode per 24 hours
Return to normal mental status and neuro
exam after postictal period
Often the initial sign of infection
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Those which do not meet simple criteria!
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Those which do not meet simple criteria!
◦ Focal seizure or focal origin (<5%)
◦ Duration >15 minutes, or multiple seizures in <24
hours (<10%)
◦ Abnormal neuro exam after recovery
 Todd’s paresis is rare (0.4-2% of cases)
◦ Abnormal mental status after recovery
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Study by Hesdorffer et al. (Ann Neuro.
2011;70(1):93) – 158 children with 1st FS
◦ Developmental delay and younger age are
associated with complex febrile seizures
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History of afebrile seizures or epilepsy
Metabolic abnormalities
CNS infection
Age <6 months or >6 years (diagnosis of
exclusion)
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CBC?
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Electolytes with divalents and glucose?
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CBC?
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Electrolytes with divalents and glucose?
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Generally very low yield unless patient has
history of vomiting, diarrhea, or abnormal
fluid intake
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AAP recommends to perform LP if:
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AAP recommends to consider LP if:
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AAP recommends to perform LP if:
◦ Meningeal signs or other symptoms suggestive of
CNS infection
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AAP recommends to consider LP if:
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AAP recommends to perform LP if:
◦ Meningeal signs or other symptoms suggestive of
CNS infection
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AAP recommends to consider LP if:
◦ Infants 6-12 months old without immunizations
against Hib and strep pneumo
◦ If the patient is on antibiotics
 Because abx can mask sx of CNS infection
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…also consider with febrile status epilepticus
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Continuous seizures or intermittent seizures
without neurologic recovery
◦ >30 minutes
◦ Clues: persistently open and deviated eyes if motor
activity has stopped
◦ FEBSTAT Prospective multicenter study (Shinnar et al.
Neurology. 2008;71(3):170)
 Clinical setting no different than shorter febrile seizures
 Higher-than-expected family history of epilepsy
 In another study by Shinnar, patients found to have a
higher prevalence of baseline neuro disease and a
personal history of epilepsy when examined
prospectively after febrile status epilepticus
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Not recommended for simple febrile seizure
◦ Not even outpatient MRI
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Definite indications:
◦ Children with abnormally large heads
◦ A persistently abnormal neurologic examination,
particularly with focal features
◦ Signs and symptoms of increased intracranial
pressure
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Teng et al., Pediatrics, February 2006:
◦ Retrospective chart review, 71 patients
 51 had a single complex feature (20 focal, 22 multiple, 9
prolonged), 20 had multiple complex features
◦ None had intracranial pathology that required emergent
neurosurgical or medical intervention
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Kimia et al., PEC, April 2012:
◦ Retrospective cohort review, 526 patients with complex
febrile seizures, 50.4% had emergent head imaging
 4 pts had significant findings (2 ICH, 1 ADEM, 1 focal
cerebral edema)
 3 out of the 4 had other obvious symptoms (nystagmus,
emesis, AMS; hemiparesis; NAT bruises)
 No patients with multiple sz had intracranial pathology
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No need for simple febrile seizure
Generally not recommended urgently in most
circumstances
◦ Some neurologists recommend outpatient if
complex
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Patient with febrile status epilepticus
requiring significant medication to stop
seizures may need urgent EEG
◦ Usually in ICU, not ED
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Attending-dependent…
Simple febrile seizures almost always do
nothing but observe the patient and provide
good anticipatory guidance and parental
teaching
Main goal is to evaluate for source of fever!
◦ Check ears, throat, skin, etc.
◦ Consider urine in patient’s without source
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Will my child have febrile seizures in the future?
◦ Overall recurrence rate is 30-35%
 50-65% if patient was <12 months old
 20% in older children
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Risk Factors:
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Young age at onset
History of febrile seizures in a first-degree relative
Low degree of fever while in the emergency department
Brief duration between the onset of fever and the initial
seizure
 Children who had all four factors were much more likely to
have a recurrent febrile seizure than were those with none
(≥70 versus ≤20 percent).
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My child had a complex febrile seizure, is he
more likely to have another than if it had
been a simple febrile seizure?
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My child had a complex febrile seizure, is he
more likely to have another than if it had
been a simple febrile seizure?
◦ Nope!
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If my child has another febrile seizure, when
will it happen?
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If my child has another febrile seizure, when
will it happen?
◦ Sooner rather than later…
 50-75% within one year
 Almost all within 2 years
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Will my child have developmental problems?
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Will my child have developmental problems?
◦ No greater risk than the general population for either
simple or complex febrile seizures
 No increase in neuro deficits, intellectual impairment or
behavioral disorders
 Longitudinal case-control study (Leaffer et al. Epilepsy
Behav. 2013 Jul;28(1):83-7)
 Population-based studies – National Collaborative
Perinatal Project (NCPP) in US and one in UK and Denmark
 No differences in outcomes in patients with complex febrile
seizures, unless they developed afebrile seizures
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Is my child more likely to develop epilepsy?
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Is my child more likely to develop epilepsy?
◦ Yes
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Is my child more likely to develop epilepsy?
◦ Yes
 Simple febrile seizure doubles risk from 0.5% to 1%
 Complex febrile seizures can increase risk more
depending on how many and which factors
 As high as 49% if complex with all 3 features
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Should my child take seizure medications?
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Should my child take seizure medications?
◦ No.
◦ AEDs may decrease the risk of recurrent febrile
seizures, but the prevention of these benign events
is generally not considered worth the potential
adverse effects of treatment.
◦ The use of AED prophylaxis in children with
complex febrile seizures is individualized based
upon underlying risk factors – no general
consensus.
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Can giving Tylenol or Motrin to my child
anytime he is sick prevent febrile seizures?
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Can giving Tylenol or Motrin to my child
anytime he is sick prevent febrile seizures?
◦ No.
◦ RCT showed that patients mount fevers despite
antipyretics
 Risk of febrile seizure does not change whether or not
the fever is treated
◦ Treat fevers as with any child.
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Definition
◦ Acute neurologic conditions which may lead to
death or permanent disability if not recognized
and treated
 Quick
neuro exam for signs of herniation
syndromes, localization, and nature of
injury/disease
 Pace of symptom evolution
 “Brain failure” degree: GCS
 Survey history for clues to the category of
disease process
Secure ABC(D)
 Recognize intracranial hypertension, herniation
 Prevent secondary injury
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• C-spine in trauma
• Sedation effects
• Maintain oxygen delivery
• Treat/avoid hyperthermia
• Treat/prevent seizures
“Where is the lesion?”
Psyche
Brain
Spinal Cord
Spinal root
Nerve plexus
Peripheral nerve
N-M Junction
Muscle
What kinds of
diseases cause
lesions in that
location?
Outside the nervous system
“What caused the lesion?”
Trauma
Infection
Inflammation
Intoxication
Neoplasm
Metabolic
(endocrine, nutritional)
Vascular, cardiovasc.
Epilepsy
Congenital malformation
Degenerative
“Where is the lesion?”
Psyche
Brain
Spinal Cord
Spinal root
Nerve plexus
Peripheral nerve
N-M Junction
Muscle
Outside the nervous system
Signs & symptoms
Behavior
Mood
Personality
Thought disorder
Social function
“Where is the lesion?”
Psyche
Brain
Spinal Cord
Spinal root
Nerve plexus
Peripheral nerve
N-M Junction
Muscle
Outside the nervous
system
Signs & symptoms
Supratentorial: seizures, mental status,
special senses, language, motor or
sensory deficit in cortical distribution,
with upper motor neuron features,
involuntary movements
Infratentorial:
Cranial nerve deficits (e.g. diplopia,
pupillary defects, oculomotor
paresis, nystagmus, vertigo)
Cerebellar ataxia
“Where is the lesion?”
Psyche
Brain
Spinal Cord
Spinal root
Nerve plexus
Peripheral nerve
N-M Junction
Muscle
Signs & symptoms
No symptoms “above the neck”
Weakness, bilateral & segmental
Sensory level
Dropped reflexes at the level of lesion
Increased reflexes below lesion
Sphincter dysfunction
Pain is common
Outside the nervous system
“Where is the lesion?”
Psyche
Brain
Spinal Cord
Spinal root
Nerve plexus
Peripheral nerve
N-M Junction
Muscle
Signs & symptoms
No symptoms “above the neck”
Sensory &/or motor deficit is segmental
Dropped reflexes at the level of lesion
No long track signs
No/little sphincter dysfunction
Pain is common
Outside the nervous system
“Where is the lesion?”
Psyche
Brain
Spinal Cord
Spinal root
Nerve plexus
Peripheral nerve
N-M Junction
Muscle
Outside the nervous
system
Signs & symptoms
Sensory &/or motor deficit fits type
& location of nerves involved
e.g. pure motor (flaccid
weakness), mixed
sensorimotor, small fiber
sensory, length-dependent or
stocking glove
Dropped reflexes at the level of lesion
No long track signs
No/little sphincter dysfunction
Pain is common
“Where is the lesion?”
Psyche
Brain
Spinal Cord
Spinal root
Nerve plexus
Peripheral nerve
N-M Junction
Muscle
Outside the nervous
system
Signs & symptoms
Pure motor deficit (flaccid) in
characteristic pattern
Reflexes present but hypoactive
No long track signs
No/little sphincter dysfunction
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14 year old male
Hit in head by swung baseball bat at practice
No helmet
+LOC x ~1 min, back to baseline soon after,
but now more lethargic
Vitals stable
On exam
◦ PERRL, GCS 13 (confused, localizes to pain)
◦ Has large temporal hematoma and palpable
depression in skull
◦ No neuro deficits appreciated
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Primary Goal
◦ Determine severity of the injury and identify ciTBI
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Epidemiology
◦ >600,000 annual visits per year (birth to 19)
◦ ciTBI incidence ranges from 0.02-4.4%
◦ TBI is leading cause of acquired disability in children
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TBI is a spectrum of illness
◦ Minor head injury  concussion  skull fracture 
pneumocephalus  intracranial hematoma  cerebral
edema  DAI  cerebral herniation  death
Identification of children at very low risk of
clinically-important brain injuries after head
trauma: a prospective cohort study
Kuppermann et al (PECARN study)
Lancet 2009; 374: 1160-70
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Obtaining CT exams must balance the
importance of identifying significant injuries
and illnesses with the risks of CT
Cancer risk
◦ 10-fold increase in neoplastic potential in children
compared to adults
◦ Children have longer lifetime during which
radiation-related cancers may evolve
◦ ~1/1000 children who have had a CT scan will
develop a radiation-induced fatal cancer in their
lifetime
 0.35% increase over expected baseline lifetime-risk
Age, Years
<2
Clinical Criteria
Normal mental status
Normal behavior per routine caregiver
No LOC (or <5 sec with low-risk mech.)
No severe mechanism of injury
No nonfrontal scalp hematoma
No evidence of skull fracture
2-18
Normal mental status
No LOC
No severe mechanism of injury
No vomiting
No severe headache
No signs of basilar skull fracture
Children <2 years old
Children 2-18 years old
Altered mental status or abnormal
behavior per caregiver
Altered mental status
Nonfrontal location of scalp
hematoma
Depressed or basilar skull fracture
LOC >5 seconds
Post-traumatic seizure
Depressed or basilar skull fracture
LOC
Bulging anterior fontanelle
Focal neurologic findings
Persistent vomiting
Worsening severe headache
Post-traumatic seizure
Focal neurologic findings
Suspicion of non-accidental trauma
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Diffuse axonal injury (DAI)
◦ Acceleration/deceleration or rotational forces
◦ Shear injuries of axons and blood vessels involving
white matter
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Cerebral edema
◦ Direct insult to neurons
 Local release of inflammatory mediators
 Vascular leakage
 Hypoxemic changes
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Bone involvement:
◦ Parietal > Occipital > Temporal
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Most common causes:
◦ Falls – 35% (most common <2yo)
◦ Recreational Activities – 29%
◦ MVC – 24%
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Unilateral, linear (account for 75% of pediatric
skull fractures)
Incidence of underlying intracranial injury
ranges from 15-30%
◦ More common in complicated fractures
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Predictive of skull fractures in infants <1yo
Clinical significance of scalp abnormalities in
asymptomatic head-injured infants.
Greenes & Schutzman, PEC 2001; 17:88
Prospective study, 422 asymptomatic infants
Hematomas in parietal (OR 38) or temporal (OR
16) regions increased likelihood of underlying
skull fracture
Hematomas in frontal region did not (OR 0.6)
Risk of skull fracture increases with size of
hematoma
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No underlying intracranial injury
No distracting traumatic injury
Normal neurologic exam
No concern for NAT
Reliable caretakers
Neurologically intact children with an isolated skull
fracture may be safely discharged after brief
observation
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Rollins et al., J Pediatr Surg 2011; 46:1342.
Retrospective review of 235 previously healthy
children, median age 11mos
0/58 patients d/c’d from ED after obs (avg 3
hours) returned for further care
177 hospitalized – avg LOS 18 hours
◦ 5 returned for persistence of symptoms
◦ 1 had delayed onset of an extraaxial intracranial
hemorrhage
Management of uncomplicated skull fractures in
children: is hospital admission necessary?
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Vogelbaum et al, Pediatr Neurosurg 1998;
29:96
Prospective study of 44 pediatric patients
Average age 1.8yo
No clinical complications in any admitted
patients
Infants with isolated skull fracture: what are their
clinical characteristics, and do they require
hospitalization?
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Greenes & Schutzman, Ann Emerg Med 1997;
30:253
Retrospective study of 101 patients <2yo
None had a decline in clinical status during
hospitalization
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Complicated fractures
Basilar skull fractures
Open skull fractures
Fractures associated with intracranial injury
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80% contain at least one of 6 classic physical
examination findings
◦ Subcutaneous bleeding over mastoid process
 Battle Sign
◦ Subcutaneous bleeding around the orbit
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 Raccoon Eyes
Hemotympanum
CSF rhinorrhea
CSF otorrhea
Cranial nerve deficits
 Facial paralysis, anosmia, nausea, vomiting, vertigo,
nystagmus, tinnitus, hearing loss
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Not recommended initially for basilar skull
fractures with non-penetrating trauma
◦ ASHP guidelines
◦ Einhorn and Mizrahi (Am J Dis Child.
1978;132(11):1121)

Risk of meningitis is significantly increased if
CSF leak >7 days
◦ Observational studies in children and adults

Use is generally recommended in patients
with open skull fractures to prevent
osteomyelitis (anti-Staph)
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8 year old male previously healthy
Found seizing at home in bed
No reports of previous headache, fever, or
focal neuro deficits
Presented to OSH
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Stabilized, intubated
Transport – pupils fixed and dilated
On arrival here, GCS 3, pupils same
Admitted to PICU, OR for repair
Declared brain dead, care withdrawn POD#1
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Subfalcine
◦ Cingulate
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Central
Uncal
◦ Transtenorial

Tonsillar
Localized mass
effect
Compression of:
- other structures
- vessels
- CSF outflow routes
Secondary injury:
- ischemia
- direct compression
- stretch
Central:
Uncal:
generalized hemisphere
edema, acute hydro
subdural, epidural,
contusion
midbrain & bilateral 3rd
n. , aqueduct,
post cerebral art.
unilateral midbrain,
unilateral 3rd n., aqueduct
basilar art. stretch - pons basilar art stretch - pons
& medulla ischemia
& medulla ischemia
Disrupted brainstem = decorticate posturing,
respiratory depression, bradycardia, pupil
dilation, lethargy, death
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Presentations differ depending on age
Infants
◦ Poor feeding, vomiting, irritability, bulging
fontanelle, altered mental status
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Children
◦ Headache, vomiting, visual changes, neck stiffness,
focal neuro findings, seizure, altered mental status,
lethargy, obtundation
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Cushing’s Triad
◦ Bradycardia, hypertension, respiratory depression

Vascular abnormalities
◦ Fistulous connection of arteries and veins without
normal intervening capillary bed
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Nearly all thought to be congenital
Most commonly supratentorial (90%)
Most common presentation is intracerebral
hemorrhage
Other presentations
◦ Headache
◦ Focal neurologic deficits
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Size of AVM not predictive of future
hemorrhage
◦ Series of 168 patients

Diagnostic imaging
◦ CT scan: good initial tool, especially in case of
acute hemorrhage
◦ MRI with or without angiography (MRA)
 Superior to CT in unruptured AVMs
 Delineates details of AVM architecture
 Sensitive in revealing subacute hemorrhage
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Neurosurgical involvement immediately
Surgical resection
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Major hemorrhage
Progressive neurologic deterioration
Inadequately controlled seizures
Intractable headache
Venous restrictive disease
Overall mortality rate of AVM hemorrhage is 10%
◦ Certain locations such as basal ganglia and thalamus
carry mortality rate of 42.9%

ABCs
◦ Consider atropine in children <1 year to blunt vagal
response to intubation
◦ Lidocaine at 1-2 mg/kg to prevent potential
increased ICP by blunting airway reflexes
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Elevate head of bed
Maintain C-spine immobilization
Ventilation to maintain PaCO2 at 35-40 mmHg
Continuous sedation to prevent agitation
Hypertonic saline (6-10 mL/kg boluses)
Mannitol (1 gram/kg)
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4 year old male presents to ED with wobbly
gait
Parents admit persistence of symptoms for one
month, but worsening over past few days
Random episodes of emesis, worse in morning
Physical exam
◦
◦
◦
◦
Vitals normal
EOMI, but boy blinks several times on testing
Normal motor and sensory exam
Wide-based gait with difficulty turning corners
Enlarged
Ventricles
Mass

MRI brain and spine with and without contrast
◦ Once that is done, then…
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Neurosurgical consult
Pre-op labs
ICP management
Discuss with NES, oncology about Decadron
Admission to ICU and preparation for
resection likely with placement of
ventriculostomy

Obstructive
◦ Stenosis of cerebral aqueduct
 Congenital, tumor, following hemorrhage or infection
◦ Posterior fossa tumors, arachnoid cysts, congenital
malformations (e.g. Dandy-Walker)

Non-obstructive (communicating)
◦ As a result of scarring following intraventricular
hemorrhage (IVH) in premature infants
◦ Following meningitis
◦ Congenital myelomeningocele
◦ Post-traumatic

Clinical Recognition
◦ Infants: macrocephaly, sunsetting (downward eye
deviation, lid retraction, convergence-retraction
nystagmus)
◦ Older children: visual field deficits or double vision (from
compression of optic chiasm or abducens nerve palsy)

Diagnosis
◦ CT (initial test) or MRI (provides greater anatomical
detail, also better for tumors)

Treatment
◦ Airway and cardiorespiratory maintenance
◦ Neurosurgical consultation for definitive management
Acute
Subacute or Chronic
Nausea
Change in behavior
Vomiting
Neuropsychological signs
Irritability
Change in feeding patterns
Seizures
Developmental delay
Headache
Change in school performance
Lethargy
Change in attention span
Coma
Daily headaches
Stupor
Increase in head size

Diagnostic imaging
◦ Shunt series (x-rays)
◦ Quick brain MRI (to compare size of ventricles to
previous studies)

Radiographic changes in asymptomatic
patients with changes in physical exam (e.g.
papilledema, increase in head circumference)
◦ Urgent neurosurgery consult

Radiographic changes and symptomatic
◦ Emergent neurosurgery consult
•
•
•
•
Previously healthy nine year old boy is
walking down the hallway at school
He feels like he can’t take the next step and
falls down
Brought by ambulance to hospital
Physical Exam
• Blood Pressure 150/90
• Patient seems unable to follow commands
• Mild right facial droop
• 5/5 strength LUE and LLE, and 3/5 strength RUE and RLE
CT 20 hrs after onset shows L basal ganglia infarct
Confirmed on MRI @ 24 hrs by axial T2 (left) and DWI (right)
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Relatively rare in children
Classified as either ischemic or hemorrhagic
At risk populations
◦ Sickle cell disease (6-9% incidence)
◦ Cardiac disease (valvular disease, endocarditis,
arrhythmias, etc.)
◦ Metabolic conditions (homocystinuria, organic
acidemias)
◦ Coagulopathies (e.g. hemophilia, Factor V Leiden, ATIII,
protein C & S def.)
◦ Neurocutaenous syndromes (neurofibromatosis,
tuberous sclerosis)
◦ Structural arterial disease (e.g. moyamoya)


Unlike adults, there is currently no consensus on
primary treatment for acute stroke in childhood
Stabilization and prevention of secondary neuronal
injury
◦ Normotension, normothermia, euglycemia, avoid hypoxia
◦ Treat hypertension cautiously (maintain cerebral perfusion)

Initiate anti-coagulation therapy
◦ Aspirin, LMWH, or unfractionated heparin
◦ Thrombolytic therapy: safety and dose of alteplase for the
treatment of children with arterial ischemic stroke have not
been established (not FDA-approved for children <18)

Hemorrhagic stroke
◦ Involvement of neurosurgery
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Can present with NO symptoms, mild
symptoms like headache and vomiting, or
severe symptoms like acute mental status
changes, seizures, hemiparesis, CN deficits
Clinical history may reveal dehydration,
mastoiditis/otitis media, hypercoagulable
state, inflammatory conditions (SLE, UC)
Management
◦ ABCs, hydration
◦ Consider anticoagulation: LMWH or heparin drip
◦ IV antibiotics if underlying infection (mastoiditis)
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11 year old male noted his arms were heavy when he boarded
the bus for school
On arrival to school, he collapsed on trying to stand and was
unable to walk
2 episodes of urinary incontinence
No fevers or sick contacts
Physical Exam
Mental status and CN intact
Motor: normal bulk; spasticity legs > arms and left side > right side
Decreased rectal tone
Strength 2/5 in LUE and LLE, 3/5 in RUE and 2-3/5 in RLE
Sensory: decreased in all modalities distal greater than proximal from
C5 down
◦ Reflexes: 4+ at left ankle, 3+ in other lower extremities, 3+ upper
extremities
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Sensory level and/or motor level and
decreased rectal tone indicate spinal pathology
Imaging: STAT MRI spine (want several
segments above and below area to which
lesion localized)
Monitor respiratory and cardiac function very
closely
◦ MD should accompany patient to imaging
T2 Hyperintensity
From C3-5
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Trauma: MVCs, diving, sports, traumatic delivery, be
aware of SCIWORA
Infection/Parainfectious: usually febrile, local bone
pain in diskitis, epidural abscess, TB, vertebral
osteomyelitis, HIV
Tumor: subacute or chronic symptoms/deficits and
back pain, primary intrinsic neuroepithelial tumors,
Schwannomas in NF, metastatic tumors
Vascular/hemorrhagic: AVM, post-LP, intramedullary
tumor, cord infarction
Demyelinating: Transverse myelitis, ADEM, Multiple
sclerosis
Anatomic: Tethered cord, Chiari malformations,
myelomeningoceles, atlanto-axial dislocations
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Rare, acquired autoimmune disorder
Demyelination and inflammation in
spinothalamic and pyramidal tracts, posterior
columns
Involves both halves of spinal cord over
variable length
May be post-infectious/idiopathic (~2/3)
◦ EBV, CMV, measles, mumps, Campylobacter,
Mycoplasma
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May be associated with systemic illness (~1/3)
◦ SLE, scleroderma, multiple sclerosis
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Presentation can be acute or subacute
Most common location is thoracic, but can
occur anywhere
Treatment
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Supportive
80% have some degree of recovery
All children should be hospitalized
Systemic corticosteroids (no RCTs, just consensus)
Insufficient evidence to support IVIG
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Typically back pain precedes other symptoms
◦ Fever, headache, vomiting, spinal compression sxs
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Bony tenderness may indicate vertebral
osteomyelitis or discitis
Treatment
◦ Emergent surgical decompression
◦ Start antibiotics (anti-Staph aureus)
 Vancomycin along with 3rd or 4th generation
cephalosporin
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Etiologies
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Congenital and acquired bleeding disorders
Hemorrhagic tumors
Spinal AVMs
Instances of increased ICP
Trauma
MRI of spine is definitive diagnostic measure
Decompression is key procedure to improve
outcomes
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If no mass lesion: Lumbar puncture performed for
cell count, gram stain/culture, protein, glucose, viral
studies (myelin basic protein, oligoclonal bands,
protein electrophoresis if white matter lesion)
MRI brain with contrast to rule-out ADEM, look for
lesions (suspicious for multiple sclerosis) – nonemergent
Basic rheumatology labs (eg ANA, CRP, ESR,
antiphospholipid antibodies)
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4 year old male with no PMH presents to ED
with inability to walk up stairs
Mild URI symptoms for 2 days
Physical exam
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Moderate respiratory distress, minimal effort
Bilateral ptosis, uncomfortable with EOM
Motor testing: proximal muscle weakness
Strength decreases on repeated attempts at activity
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10 year old male with no PMH presents with
bilateral lower extremity pain and difficulty
walking over past 2 days
No bowel or bladder incontinence, no fevers
Mother reports AGE 3 weeks prior
Physical exam
◦ Motor testing: 2/5 strength in bilateral lower
extremities, 4+/5 in bilateral upper extremities
◦ Reflexes: diminished at ankle and patellar
◦ Sensory: hyperesthesia to feet
◦ CNs intact, rectal tone normal
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4 month old female ex-full term, no PMH,
presenting with 3 days of poor breast
latching and hypotonia
Parents report no bowel movements over past
4 days (usually goes every day)
No fevers
Father is farmer
Physical exam
◦ Mild hypotonicity
◦ Poor suck and gag reflexes
◦ Bilateral ptosis
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Anterior Horn Cell
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Peripheral Nerve
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Neuromuscular Junction
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Muscle
◦ Poliomyelitis, postasthmatic amyotrophy, SMA, amyotrophic
lateral sclerosis
◦ Guillain-Barré syndrome, Erb/Klumpke palsy, heavy metal
poisoning, toxins/ingestions, marine toxins, acute intermittent
porphyria
◦ Botulism, myasthenia gravis, tick paralysis, organophosphates,
neuromuscular blockers, snake envenomations
◦ Muscular and myotonic dystrophies, dermatomyositis,
infectious (e.g. viral), metabolic abnormalities, periodic
paralysis, rhabdomyolysis/myoglobinuria, endocrine disorders,
steroid myopathy
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Acute management
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Cardiopulmonary monitoring
Get vital capacity, negative inspiratory force
CXR
Emergent neurology consult
 EMG and Tensilon testing (have atropine at bedside)
 Decision on treatment (pyridostigmine (Mestinon))
◦ If intubation required, avoid succinylcholine
 Have anesthesia available
◦ If patient rapidly deteriorating, other therapies
include prednisone, IVIG, plasmapheresis
◦ Admission to PICU
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Failure of neuromuscular transmission due to
antibodies against acetylcholine receptor at postsynaptic NM junction
3 forms in children
◦ Transient neonatal
◦ Infantile (congenital)
◦ Juvenile (most common – mimics adult disease)
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Has a fluctuating, non-predictable course
◦ Exacerbated by illnesses and certain drugs (e.g.
aminoglycosides)
◦ Rapid worsening of symptoms and resp compromise
(myasthenic crises) may be difficult to differentiate from
cholinergic crises (overdose of anticholinesterases)
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Acute management
◦ Cardiopulmonary monitoring
 Autonomic dysfunction common
◦ Vital capacity, Negative inspiratory force
◦ Emergent neurology consult
◦ Admission for all patients with suspected GBS
 Chance of progression to respiratory failure (10-20%)
◦ LP
 Elevated protein, <10 WBCs, glucose normal
◦ Consider MRI - enhancement of spinal nerve roots
◦ IVIG or plasma exchange
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Primary demyelinating disorder causing
symmetric ascending paralysis
Autoimmune phenomenon
Antecedent respiratory or GI illness
◦ 75% of childhood cases
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Weakness is commonly insidious
Paresthesias/sensory complaints occur in 50%
DTRs diminished or absent
CN abnormalities occur in 30-40% of cases
Miller Fisher variant
◦ Oculomotor palsies, ataxia, areflexia without motor
extremity weakness
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Acute management
◦ Cardiopulmonary monitoring
◦ Rule-out other causes (consider LP, head/spine
imaging)
◦ Admission for close observation for respiratory
compromise (77% required intubation in a large
series of 57 patients), NG tube feeds
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Think of botulism in an infant with worsening
lethargy, feeding difficulties and constipation
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Infants usually <6 months old
Intestinal colonization by Clostridium botulinum
◦ Honey
◦ Spores found in soil and agricultural products
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Diagnosis: C. botulinum toxin (A or B) in stool
Treatment
◦ Human botulism immunoglobulin (BabyBIG)
 Trials have shown a decrease in duration of illness
◦ No evidence to support use of antibiotics, cathartics or
other laxatives
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Neurological and neurosurgical emergencies
are frequent causes of pediatric ER visits
A good history and the temporal pattern of
the symptoms often aid in diagnosis
A good neurological examination helps to
localize the problem and guide further
testing and management
Recognition and treatment in the ER may
improve outcomes for these children