Pediatric Pediatric Neurology Neurology

EmergenciesEmergenciesDr. Mohamed I. Abunada

Head of pediatric Neurology Department Alrantissi ped Hospital

23:521

Objectives• Febrile Seizures– AAP recommendations– Prophylaxis• Neonatal seizure• Status Epilepticus• Meningitis• Encephalitis

• Idiopathic Facial Nerve Paralysis• Closed Head Injury

23:522

FEBRILE SEIZURE

23:523

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

23:524

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.

23:525

Complex Febrile Seizure

• Focal• > 15 minutes• >1 in 24 hrs• Post-ictal involvement (Todd Paralysis)

23:526

Febrile Seizures - Risk Factors

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

23:527

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%)

23:528

Febrile Seizures and Immunizations

• Increased in– 1 Day of DPTP-Hib– 8-14 days post MMR

• NO long term consequences

23:529

Febrile Seizures – AAP Guidelines

23:5210

AAP Guidelines

23:5211

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

23:5212

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

23:5213

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

23:5214

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

23:5215

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

23:5216

Febrile Seizure - PreventionCommittee on Quality Improvement Subcommitteeon 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”

23:5217

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

23:5221

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

23:5222

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

23:5223

Status Epilepticus

• Continuous or repetitive seizure activity of at least 30 minutes with failure to regain consciousness between convulsions.

23:5224

S.E. Etiology in Pediatrics

• 26% acute CNS insult– Bleed/trauma– Infection

• 21% underlying sz D/O– Sudden discontinuation of Meds– Fever

• 53% unknown!!!

23:5225

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%

23:5226

S. E. - Therapies

• Benzodiazepine• Phenytoin/Fosphenytoin• Phenobarbitone• Refractory S.E. Tx

23:5227

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

23:5228

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

23:5229

S.E. - Barbituates

• Phenobarbital– Drug of choice in neonates– High sedative and CR depression which may be

enhance with prior benzo Tx

23:5230

Refractory S.E.

• Failure to respond to sequential treatment with benzo, phenytoin, phenobarb

• Midazolam infusion• Propofol infusion• VPA infusion

23:5231

AED Doses - PediatricDrugDoseOnset

Lorazepam0.05-0.1 mg/kg IV/PR2-3 min

Diazepam0.1-0.3 mg/kgIV0.5 mg/kg PR

1-3 min

Phenytoin10-20 mg/kg IV10-30 min

Fosphenytoin20 mgPE/kg10-30 min

Phenobarbital20 mg/kg10-20 min

Midazolam0.1-0.2 mg/kgInf. 1-3 ug/kg/m

Propofol1-2 mg/kgInf. 25-100 ug/kg/min

VPA15-20mg/kgInf. 5 mg/kg/hr

23:5232

Infectious Neurologic Emergencies

Meningococcemia

23:52 33

• 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

23:5234

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

23:5235

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!!!!!

23:5236

Meningitis

• Emergent CT Prior to LP– Those with profoundly depressed Mental Status– Seizure– Head Injury– Focal Neurologic signs– Immunocompromised – DO NOT DELAY ANTIBIOTIC THERAPY!!

23:5237

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

23:5238

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

23:5239

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

23:5240

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

23:5241

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.

23:5242

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

23:5243

Encephalitis - Epidemiology

• overall incidence of hospitalization was 7.3 cases/100,000 annually

• Children < 1 yo13.7 cases/100,000 per yr

23:5244

23:5245

Clinical presentation

• Fever • Headache• Altered mental status • Focal neurologic signs • spectrum of clinical evolution during encephalitis varies

widely

23:5246

23:5247

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

23:5248

Management

• Appropriate antiviral, antimicrobial or antifungal therapies

• Intravenous immune globulin, corticosteroids, or other immune system modulators.

23:5249

Herpes Simplex Virus Encephalitis

• Most common encephalitis diagnosed• Fever• Personality change• Autonomic dysfunction• Dysphagia• Seizures• Headache• Altered level of consciousness

23:5250

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.

23:5251

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.

23:5252

Investigations

• CT head• LP• CBC, Blood culture• Lytes• Glucose• ESR, CRP

23:5253

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

23:5254

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

23:5255

Bell’s Palsy• DDx

– Lyme disease (may be up to 50% in endemic areas)– AOM, mastoiditis– Ramsay Hunt (Herpes Zoster Oticus)– Tumor

• Leukemia/lymphoma• Schwannoma• parotid

– Neurofibromatosis– Brainstem infarcts– Stroke– Trauma

23:5256

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

23:5257

Bell’s Palsy - Treatment

• Eye protection• 85-90% in children spontaneously resolve with

most occurring within 2 months of onset

23:5258

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

23:5259

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

23:5260

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.

23:5261

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.

23:5262

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.

23:5263

Cerebral Contusion

23:5264

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

23:5265

Subdural Hematoma

23:5266

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

23:5267

Epidural Hematoma

23:5268

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.

23:5269

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

23:5270

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

23:5271

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

23:5272