4. Case
• 16 mo boy previously well brought by ED
because of sz at home
• Lasted 1 min
• Eyes rolled back, arms twitching
• Crying ++ after
• Has had URTI over past 2-3 days
08:084
5. Simple Febrile Seizures - Definition
• Normal preceding neuro and dvt status
• Age 6 mo – 5 yrs
• brief (<15 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.
08:085
7. Febrile Seizures - Risk Factors
• Height of temperatture
• Male
• Family Hx of febrile seizures
08:087
8. Febrile Seizures - Epidemiology
• 2-5% of children
• Peak onset is 1 yo
• 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%)
08:088
9. Febrile Seizures and Immunizations
• Increased in
– 1 Day of DPTP-Hib
– 8-14 days post MMR
• NO long term consequences
08:089
12. 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 (<24
hrs)
• Up to ½ of recurrences occur in 1st
2 hrs
08:0812
13. Febrile Seizures – Recurrence Risk
• Meta-analysis of recurrence risk
– Onset younger than 12 mo
– Febrile or afebrile seizure in 1st
degree relative
– Rectal temp < 40 C during 1st
sz
– Offringa et al
08:0813
14. Consequences of Febrile Seizures?
• Epilepsy
– General population – 1%
– Simple febrile szs – risk of afebrile szs by age 25
increases to 2.4%
– >1 focal complex febrile sz – may by up to
30%
• Cognitive Outcome
– No changes in several american and british studies
08:0814
15. 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
08:0815
16. Febrile Seizures – Prevention?
• Antiepileptics
Phenobarb – definite reduction in recurrences if taken
continuously, no difference if intermittent
• Hyperactivity, irritability, blood levels, sleep d/o
VPA – definite reduction in recurrences also if continuous
• Hepatotoxicity, thrombocytopenia, pancreatitis, wt change
Intermittent diazepam – reduction by 44% per person per year
• Ataxia, lethargy, irritability, sleep
Phenytoin, carbamazepine – no difference
08:0816
17. Febrile Seizure - Prevention
Committee on Quality Improvement Subcommittee
on Febrile Seizure of the AAP 1999
• “Based on the risk and benefits of effective
therapies, neither continuous nor intermittent
anticonvulsant therapy is recommended for children
with one or more simple febrile seizures. AAP
recognises that recurrent episodes of febrile seizures
can create anxiety in some parents and their children
and as such appropriate educational and emotional
support should be provided”
08:0817
18. Neonatal seizure
• brief and subtle
– eye blinking
– mouth/tongue movements
– “bicycling” motion to limbs
• typically sz’s can’t be provoked
• autonomic changes
• EEG less predictable
08:0821
19. neonatal seizure
• etiology
– hypoxic-ischemic encephalopathy
• Presents within first day
– congenital CNS anomalies
– intracranial hemorrhage
– electrolyte abnormalities – hypoglycemia and
hypocalcemia
– infections
– drug withdrawal
– pyrodoxine deficiency
08:0822
20. Case #2
• 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 ED, 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
08:0823
21. Status Epilepticus
• Continuous or repetitive seizure activity of at
least 30 minutes with failure to regain
consciousness between convulsions.
08:0824
23. 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 <1 yo, 6% in those > 3yo
• Mortality
– 3%
08:0826
24. S. E. - Therapies
• Benzodiazepine
• Phenytoin/Fosphenytoin
• Phenobarbitone
• Refractory S.E. Tx
08:0827
25. S.E. - Benzodiazepine
• Lorazepam vs Diazepam vs Midazolam
• Lorazepam 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
08:0828
26. 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
08:0829
27. S.E. - Barbituates
• Phenobarbital
– Drug of choice in neonates
– High sedative and CR depression which may be
enhance with prior benzo Tx
08:0830
28. Refractory S.E.
• Failure to respond to sequential treatment
with benzo, phenytoin, phenobarb
• Midazolam infusion
• Propofol infusion
• VPA infusion
08:0831
29. 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
08:0832
31. • Meningitis: inflammation of the meninges
• History:
– Acute Bacterial Meningitis:
• Rapid onset of symptoms <24 hours
– Fever, Headache, Photophobia
– Stiff neck, Confusion
• Etiology By Age:
– 0-4 weeks: E. Coli, Group B Strep, Listeria
– 4-12 weeks: neotatal pathogens, S. pneumo, N. meningitides, H. flu
– 3mos – 18 years: S.pneumo, N. menin.,H. flu
08:0834
32. Meningitis
• Lymphocytic Meningitis (Aseptic/Viral)
– Gradual onset of symptoms as previously listed over 1-7
days.
– Etiology:
• Viral
• Atypical Meningitis
– History (medical/social/environmental) crucial
– Insidious onset of symptoms over 1-2 weeks
– Etiology:
• TB
• Coccidiomycosis, crytococcus
08:0835
33. Meningitis
• Physical Exam Pearls
– Infants lack the usual signs and symptoms.
– Look for papilledema, focal neurologic signs, Ophthalmoplegia
and rashes
– As always full exam
• Checking for above
• Brudzinski’s sign
• Kernigs sign
– KEY POINT: If you suspect meningococcemia do NOT delay
antibiotic therapy, MUST start within 20 minutes of arrival!!!!!
08:0836
34. Meningitis
• Emergent CT Prior to LP
– Those with profoundly depressed Mental Status
– Seizure
– Head Injury
– Focal Neurologic signs
– Immunocompromised
– DO NOT DELAY ANTIBIOTIC THERAPY!!
08:0837
35. Meningitis Management
• Antibiotics By Age Group
– Neonates(<1month) = Ampicillin + Gent. Or Cefotaxime + Gent
- Infants (1-3mos) = Cefotaxime or Ceftriaxone + Ampicillin
- Children (3mos-18yrs) = Ceftriaxone + Vancomycin
- Adults (18yr-up) = Ceftriaxone + Vancomycin
08:0838
36. Meningitis Management
• Steroids
– In children, dexamethasone has been shown to be of
benefit in reducing sensiorneural hearing loss, when given
before the first dose of antibiotic.
– Indications:
• Children> 6 weeks with meningitis due to H. flu or S. pneumo.
• Adults with positive CSF gram stain
– Dose: 0.15mg/kg IV
08:0839
37. Case #3
• 12 yo old female presents with fever, headache,
neck stiffness, swallowing difficulties and
altered mental status
• Symptoms have worsened over past 2 days.
• noted a change in behaviour for the past week
• 2 weeks ago had a bad URTI- missed 2 days of
school
08:0840
38. Encephalitis
• Defined as acute CNS dysfunction with radiographic
or laboratory evidence of brain inflammation
1. Primary Encephalitis
– cause bloodstream infection, then enter the CNS
2. Post- or Parainfectious
– not caused by direct CNS infection
– consequence of the host’s immune response
08:0841
39. Encephalitis
• HSV - typically infects neurons in the temporal
lobe
• Rabies - predominantly affects the pons, medulla,
cerebellum, and hippocampus
• Japanese encephalitis virus affects the brainstem
and basal ganglia.
08:0842
40. Post- Para infectious Encephalitis
• Occurs days to weeks after the onset of an infection
• Hypothesized to be caused by an aberrant immune
response against brain antigens such as myelin
basic protein
• Subsequent demyelination causes focal or global
CNS dysfunction
08:0843
41. Encephalitis - Epidemiology
• overall incidence of hospitalization was
7.3 cases/100,000 annually
• Children < 1 yo
13.7 cases/100,000 per yr
08:0844
45. Encephalitis
• CT scan – normal at the onset of encephalitis.
• MRI – much more sensitive for acute changes
• EEG - helpful adjunct +/- localize the region of
encephalitic involvement
– considerably more sensitive
08:0848
46. Management
• Appropriate antiviral, antimicrobial or
antifungal therapies
• Intravenous immune globulin, corticosteroids,
or other immune system modulators.
08:0849
47. Herpes Simplex Virus Encephalitis
• Most common encephalitis diagnosed
• Fever
• Personality change
• Autonomic dysfunction
• Dysphagia
• Seizures
• Headache
• Altered level of consciousness
08:0850
48. HSV
• Mildly elevated CSF WBC counts (lymphocyte predominant)
and CSF protein
• CT and MRI studies - normal if obtained early in the course of
illness
– Unilateral or bilateral temporal lobe involvement (most common
finding)
• Diagnostic test - HSV DNA detection by PCR on the CSF
– both highly sensitive and specific
– If initial result negative, test should be repeated on a second CSF
specimen.
08:0851
49. Treatment
• IV acyclovir 10 mg/kg per dose every 8 hours
for 2 to 3 weeks.
• Better outcomes if:
– age < 30 yo
– shorter duration of symptoms before initiation of
treatment
– GCS >10 at the time of presentation.
08:0852
51. 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
08:0854
52. 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
08:0855
54. 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
• Decreased tearing
• hyperacusis
08:0857
55. Bell’s Palsy - Treatment
• Eye protection
• 85-90% in children spontaneously resolve with
most occurring within 2 months of onset
08:0858
56. 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
08:0859
57. 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
08:0860
58. Closed Head Injury
• Definitions :
– Concussion: refers to a transient LOC following head
injury. Often associated with retrograde amnesia that also
improves.
– “Coup” = injury beneath the site of trauma
– “Countre coup” = injury to the side polar opposite to the
traumatized area.
– Diffuse Axonal Injury : tearing and shearing of nerve
fibers at the time of impact secondary to rapid
acceleration/deceleration forces. Causes prolonged coma,
injury, with normal initial head CT and poor outcome.
08:0861
59. Closed head Injury Facts
• The single most important factor in the neurologic
assessment of the head injured patient is level of
consciousness. (LOC)
• Always assume multiple injuries with serious
mechanism.
– Especially Cervical - SPINE!!!!
– Unless hypotensive WITH bradycardia and WARM
extremities; hypotension is ALWAYS secondary to
hypovolemia from blood loss in the trauma patient!
• The most common intracranial bleed in CHI is
subarachnoid hemorrhage.
08:0862
60. Closed Head Injuries with Hemorrhage
• Cerebral Contusion
– Focal hemorrhage and edema under the site of impact.
– Susceptible areas are those in which the gyri are in close
contact with the skull
• Frontal lobe
• Temporal lobes
– Diagnostic Test of Choice: Head CT
– Treatment: Supportive with measures to keep ICP normal.
Repeat Neuro checks. Repeat Head Ct in 24 hours. Good
prognosis.
08:0863
62. Subdural Hematoma
• Occurs secondary to acceleration/deceleration injury with
resultant tearing of the bridging veins that extend from the
subarachnoid space to the dural sinuses.
• Blood dissects over the cerebral cortex and collects under the
dura overlying the brain.
• Patients at risk:
– Alcoholics
– Elderly
– Anticoagulant users
• Appears as “sickle shape” and does not extend
across the midline
08:0865
64. Epidural hematoma
• Occurs from blunt trauma to head especially over the
parietal/temporal area.
• Presents as LOC which then patient has lucid interval then
progressive deterioration, coma , death. ( Patient talks to you
& dies!)
• Commonly associated with linear skull fracture
• Mechanism of bleed is due to tear of artery, usually middle
meningeal.
• PE reveals ipsilateral pupillary dilitation with contralateral
hemiparesis.
• CT Scan : a BICONVEX (lens) density which can extend across
the midline
08:0867
66. Management of Closed Head Injuries
• As always ABC’s with C-Spine precautions
• IV, O2, Monitor.
• Stabilize and resuscitate
– Sao2>95%
– Treat Fever
• Head of Bed 30% (once C-Spine cleared)
• Head CT with Stat Neurosurgical evaluation for
surgical lesions.
• Repeat Exams, looking for signs of herniation.
08:0869
67. Signs of Herniation / Increased ICP
• Headache, nausea, vomiting
• Decreasing LOC
• Sixth nerve paresis (one or both eyes adducted)
• Decreased respiratory rate
• Cushing reflex (hypertension/bradycardia/bradynpea)
• Papilledema
• Development of signs of herniation
– Fixed and dilated pupil
– Contralateral hemiparesis
– Posturing
08:0870
68. Herniation Syndromes
• Uncal Herniation:
– Occurs when unilateral mass pushes the uncus (temporal
lobe) through the tentorial incisa, presenting as:
• Ipsilateral pupil dilatation
• Contralateral hemiparesis
• Deepening coma
• Decorticate posturing
• Apnea and death
08:0871
69. Herniation Syndromes
• Cerebellar Herniation
– Downward displacement of cerebellar tonsils
through the foramen magnum.
– Presents as :
• Medullary compression
• Pinpoint pupils
• Flaccid quadriplegia
• Apnea and circulatory collapse
08:0872
Editor's Notes
Very wordy – overall no AED recommended for febrile seizure
CNS dysfunction includes seizures, focal neurologic findings, and alteration in mental status.
Encephalitis tends to be a rare complication after viremia because entry to the brain is carefully regulated at the blood-brain barrier. An alternative mechanism used by herpes simplex virus (HSV), rabies, and possibly poliovirus is retrograde transport in neurons.
An alternative mechanism used by the amoeba Naegleria fowleri is entry through the olfactory mucosa.
Primary Encephalitis
cause bloodstream infection, then enter the CNS via endothelial cell transport, or carriage in cells entering the CNS.
gray matter often is targeted
2. Post- or Parainfectious
illness is not caused by direct CNS infection
neurologic effects are the consequence of the host’s immune response, which often affects the white matter
Once an organism has entered the brain, a variety of anatomic sites can become infected.
Histologically, the host response can include perivascular inflammation, gliosis, and brain edema.
Postinfectious encephalitis often is called acute disseminated encephalomyelitis (ADEM
Children &lt;1yo and adult&gt; 65yo had the highest incidence.
Herpesviruses, varicella, and arboviruses were the viral causes reported most frequently.
The most common confirmed or probable viral pathogens were HSV, enterovirus, and Epstein-Barr virus (EBV).
most arboviral encephalitis has diffuse brain involvement, and early fever, vomiting, obtundation, and coma are typical.
In contrast, HSV encephalitis can begin focally with hemiparesis, seizures, or cranial nerve defects. (temporal lobe)
Cat-scratch disease and EBV,may have a brief course with full recovery
HSV has a guarded long-term prognosis despite the availability of specific antiviral treatment.
Mild-to-moderate CSF abnormalities are typical in infectious encephalitis.
Elevated WBC counts often with a predominance of lymphocytes.
Protein concentrations can be elevated, but usually are less than 200 mg/dL
Glucose values usually are in the normal range.
HSV and other causes of necrotizing encephalitis may be associated with an elevated red blood cell count and more dramatic changes in WBC and protein levels.
Results of routine blood chemistry and hematology tests usually are normal.
CT scan without contrast often is performed swiftly for a patient who has an acute neurologic syndrome, but this study rarely
shows an abnormality at the onset of encephalitis.
Magnetic resonance imaging (MRI) is much more sensitive than CT scan for acute changes associated with encephalitis.
EEG can be a helpful adjunct in the early assessment of encephalitis.
EEG may be needed to assess seizure activity and may help localize the region of encephalitic involvement.
Compared with CT scan, EEG is considerably more sensitive in detecting focal encephalitis at the time of presentation
only HSV and varicella have well-established
therapy. In contrast, most bacterial, fungal, and parasitic
causes have accepted treatments for systemic infection.
Prior to the availability of antiviral agents, approximately 70% of HSV encephalitis patients died; acyclovir decreased the overall
mortality rate to 19%.
In long-term follow-up studies of surviving acyclovir recipients, 38% were normal, 9% were moderately impaired, and 53% were severely impaired.