Pediatric Seizure and Status Epilepticus in the ED - University of

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					        Pediatric Seizure and
Status Epilepticus Management in the
       Emergency Setting

     Edward P. Sloan, MD, MPH
         Associate Professor
  Department of Emergency Medicine
         University of Illinois
             Chicago, IL
                        Case
A 7-year old boy presents to the ED with a history of
staring spells, some shaking movements, and
headache over the past day. He has no history of
seizures or epilepsy. In the ED, he has three episodes
of tachycardia, staring and confusion that last several
minutes and resolve without therapy. He then has a
similar episode associated with diaphoresis and
urinary incontinence. His most likely diagnosis is:


                                Edward Sloan, MD, MPH
         Most likely diagnosis:
A.   Absence status epilepticus
B.   Complex partial status epilepticus
C.   Benign childhood epilepsy
D.   Lennox-Gastaut syndrome
E.   Generalized convulsive status
     epilepticus

                         Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
Assuming that the above episodes in the 7-year
old represent repeated seizures, all of the
following are acceptable initial therapies except:
A.   Rectal diazepam
B.   Rectal diazepam gel
C.   IM midazolam
D.   IV lorazepam
E.   IV phenobarbital

                            Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
                      Case
A 13-year old female presents at mid-morning to the ED
with a one-day history of a frontal headache, consistent
with prior migraines, that was relieved with ibuprofen.
She also was noted by family members to be restless in
bed, and was noted to “thrash about for a brief period
of time.” The family denied that this was a generalized
seizure, and denied any history of epilepsy, trauma,
drug ingestion, or similar episodes. The patient has a
similar episode in the ED, and then has a generalized
seizure.
                                Edward Sloan, MD, MPH
    Most likely diagnosis:

A. Primary generalized seizure
B. Absence seizure
C. Complex partial seizure with
   secondary generalization
D. Juvenile myclonic epilepsy
E. Non-convulsive status epilepticus
                    Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
                Case
A 21-year old male college student presents
in the early morning to the ED with a one-
day history of having a generalized seizure
upon awakening. The patient had been
partying after final exams, and had not been
getting much sleep for several days. Over
the phone, his mom noted that he had a
history of “staring spells” as a child.
                         Edward Sloan, MD, MPH
    Most likely diagnosis:
A. Primary generalized seizure
B. Absence seizure
C. Complex partial seizure with
   secondary generalization
D. Juvenile myoclonic epilepsy
E. Non-convulsive status epilepticus


                      Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
The above 21-year old patient had
 two seizures in the ED which were
 controlled with lorazepam. If a
 load of a longer acting AED was to
 be given in the ED in order to
 prevent status epilepticus, what
 would be the optimal drug to
 administer?
                    Edward Sloan, MD, MPH
Most optimal drug:

A.   IV midazolam
B.   IV phenytoin
C.   IV fosphenytoin
D.   IV valproate
E.   IV phenobarbital


                Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
What is the optimal loading dose of IV
 valproate in patients at risk for SE?

  A.   1-5 mg/kg
  B.   10-15 mg/kg
  C.   20-30 mg/kg
  D.   90-100 mg/kg



                      Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
If an IV valproate load of 25 mg/kg were
given to this patient, what would be the
expected valproate level once the
infusion had ended?




                        Edward Sloan, MD, MPH
Expected IV Valproate level?
       A.   25 mg/L
       B.   50 mg/L
       C.   75 mg/L
       D.   100 mg/L
       E.   125 mg/L

                   Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
If this patient were to develop
status epilepticus, what is the
fastest time of infusion possible
for a loading valproate infusion
of 2500 mg (25 mg/kg x 100 kg)?


                    Edward Sloan, MD, MPH
Fastest Infusion Time Possible?
  A. 4 minutes (6 mg/kg/minute)
  B. 8 minutes (3 mg/kg/minute)
  C. 24 minutes (1 mg/kg/minute)
  D. 2 minutes (0.3 mg/kg/minute)
  E. 216 minutes (0.1 mg/kg/minute)

                    Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
             Overview
         Global Objectives
•   Learn more about pediatric seizures
•   Focus on peds sz etiologies
•   Increase awareness of Rx options
•   Enhance our ED management
•   Improve patient care & outcomes
•   Maximize MD & patient satisfaction

                        Edward Sloan, MD, MPH
            Overview
   Pediatric Sz Epidemiology
• Common EMS & ED problem
• Szs are up to 6% of EMS encounters
• Up to 1% of all ED visits are peds sz
  – Peds febrile: 1 in 125 visits (0.8%)
  – Peds afebrile: 1 in 500 visits (0.2%)


                           Edward Sloan, MD, MPH
                Overview
       Pediatric Sz Epidemiology
•   2-5% have a febrile seizure
•   1% have an afebrile sz by age 14
•   Highest afebrile sz rate before age 3
•   0.4-0.8% of children develop epilepsy
•   SE most common before age 1


                         Edward Sloan, MD, MPH
             Overview
    Pediatric Sz Epidemiology
• Mean age 3.2 yrs, median age 1 year
• 61% by age 3
• Etiology age dependent
  – 25% is febrile SE
  – Before age 1, 75% due to acute insult
  – Epilepsy, fever, CNS infection common

                         Edward Sloan, MD, MPH
        Pediatric Sz Etiologies
              Meningitis
•   Altered mental status universal
•   Seizures in 23% of meningitis cases
•   Complex & GTC seizures common
•   Simple seizures rarely seen
•   HIB vaccine makes this etiology rare


                         Edward Sloan, MD, MPH
             Hyponatremia
•   Causes long duration szs and SE
•   Infants < 6 months old, no clear etiol
•   Too much water in formula
•   Hypothermia (Temp < 36.5 degrees)




                          Edward Sloan, MD, MPH
          Cocaine Toxicity
•   Consider in new onset seizures
•   Crack cocaine rocks ingested
•   Especially when no other etiology
•   Common in urban EDs




                         Edward Sloan, MD, MPH
         Pediatric Seizures
         Seizure Outcome
• Immature CNS, myelinization
  – More prone to seizures
  – More resistant to consequences
• Continuous seizures less toxic
• SE carries a low mortality (3-6%)


                        Edward Sloan, MD, MPH
          Pediatric Seizures
            SE Outcome
• Based on CNS status prior to SE
• Normal CNS, 64% remain intact
• Mortality related to two factors:
  – Acute neurologic insult
  – Chronic CNS condition



                          Edward Sloan, MD, MPH
        Pediatric Seizures
    Seizure Type Classification
• Generalized
  – Involves both cerebral hemispheres
  – Convulsive: tonic-clonic seizures
  – Non-convulsive: absence seizures
• Partial
  – Involves one cerebral hemisphere
  – Simple: no impaired consciousness
  – Complex: impaired consciousness

                            Edward Sloan, MD, MPH
       Seizure Classification
       Generalized Seizures
• Convulsive seizures
  – Tonic sz: sustained contractions
  – Clonic sz: rhythmic flexor spasms
  – Tonic-clonic sz: combined movements
• Non-convulsive
  – Simple absence: impaired consciousness
  – Complex absence: brief motor mvmts
                        Edward Sloan, MD, MPH
        Seizure Classification
           Partial Seizures
• Simple seizures (no LOC)
  – Focal motor (Jacksonian)
  – Sensory or somatosensory
  – Autonomic
  – Psychic
• Complex (impaired consciousness)
  – Involves some cognitive, affective sx
  – Temporal lobe, psychomotor seizures
                             Edward Sloan, MD, MPH
         Pediatric Seizures
     Other Generalized Sz Types
•   Neonatal seizures
•   Benign childhood epilepsy (Rolandic)
•   Infantile spasms (West syndrome)
•   Lennox-Gastaut syndrome
•   Atonic seizures
•   Febrile seizures

                        Edward Sloan, MD, MPH
        Pediatric Seizures
     Status Epilepticus Types
• Convulsive SE: tonic-clonic sz
• Non-convulsive SE: no tonic-clonic sz
  – Absence SE
  – Complex partial SE
• Subtle SE: prolonged convulsive SE
  – Worst prognosis, mortality > 30%
  – Persistent coma, focal motor mvmts only
                         Edward Sloan, MD, MPH
         Specific Seizure Types
            Febrile Seizures
•   Age: 6 months to 5 years
•   Related to rapid rise in temperature
•   Brief, self-limited generalized sz
•   Complex: Focal, > 10-15 min, flurry
•   25% recurrence, esp if in child < 1 yr old
•   Risk of epilepsy not significantly greater


                             Edward Sloan, MD, MPH
       Specific Seizure Types
     Juvenile Myoclonic Epilepsy
•   Common in teens, young adults
•   Etiology of generalized TC seizures
•   History of staring spells
•   History of AM clumsiness, myoclonus
•   Sleep deprivation, EtOH precipitants
•   Phenytoin: worse myoclonus, absence sz


                          Edward Sloan, MD, MPH
       Specific Seizure Types
      Generalized Convulsive SE
•   Seizure lasting greater than 5-10 min
•   Refractory to initial benzo therapy
•   Flurry of seizures and coma
•   CNS injury likely after 30-40 minutes
•   Glutamate, cell death, tissue necrosis
•   Injury even if systemic sx controlled

                          Edward Sloan, MD, MPH
        Specific Seizure Types
         Non-Convulsive SE
• No generalized tonic-clonic sz
    – Absence SE
    – Complex partial SE
•   No frank coma
•   More common in children
•   Not always due to co-morbidity
•   Mortality ?? Not as high as in GCSE
                           Edward Sloan, MD, MPH
       Seizure Therapy
  Generalized Seizure Protocol
• Benzodiazepines
  – PR diazepam, IM midazolam, IV lorazepam
• Phenytoins
  – Fosphenytoin can be given IV or IM
• Phenobarbital or valproate
  – Less sedation with valproate
• Propofol or midazolam infusions
  – EEG monitoring, BP support key
                         Edward Sloan, MD, MPH
               Seizure Therapy
              Ongoing Therapies
• Absence:         ethosuximide, valproate
• Atonic:          valproate, clonazepam,
                   ethosuximide
• Myclonic:        valproate, clonazepam
• Partial:         carbamazepine,
                   phenytoin, valproate
• Generalized:     carbamazepine,
                   phenytoin, phenobarb,
                   primidone, valproate

                            Edward Sloan, MD, MPH
         Case Presentation
     ED Pediatric Seizure Cases

• Pediatric complex partial SE
• New onset SE in an adolescent
• New onset sz in a college student




                       Edward Sloan, MD, MPH
           Pediatric SE
                Hx
• 7 year old male
• Seizure-like activity?
• Patient with staring spells
• Some headache and shaking movement,
  esp of hands
• Frontal headache, vomiting

                    Edward Sloan, MD, MPH
             Pediatric SE
              Hx (cont.)
• Seen at 2130, 2230 sign-out
• AMS, r/o seizure disorder
• “Once all of the labs are back, he should
  be OK to go home…”




                       Edward Sloan, MD, MPH
              Pediatric SE
                   Px
•   98.7, 98/60 72 20
•   Well-hydrated
•   CV, lung exams normal
•   Neuro exam intact



                       Edward Sloan, MD, MPH
              Pediatric SE
             Clinical Course
•   0220 “episode”
•   Tachycardia, BP OK, airway OK
•   Confused, staring off into space
•   Episode lasted < 5 minutes
•   Resolved without any Rx


                         Edward Sloan, MD, MPH
             Pediatric SE
        Clinical Course (cont.)
•   Three more episodes over 40’
•   Similar autonomic symptoms
•   Some non-purposeful ext mvmts
•   Diaphoresis, urinary incontinence
•   Remained somnolent between episodes


                       Edward Sloan, MD, MPH
               Pediatric SE
                    Dx
•   Repetitive episodes with AMS
•   Autonomic symptoms noted
•   Non-purposeful mvmts noted
•   Rule out complex partial status
    epilepticus (CPSE)


                         Edward Sloan, MD, MPH
           Pediatric SE
                Rx
•   IV lorazepam
•   IV valproate
•   Transfer to Children’s
•   ICU observation
•   Uncomplicated course


                      Edward Sloan, MD, MPH
             Adolescent SE
                  Hx
•   13 year old female
•   Frontal HA and prior migraines
•   HA relieved with ibuprofen
•   AMS in AM, with ?? motor activity
•   Restless at home, thrashing on bed
•   No other systemic sx or recent illness
                         Edward Sloan, MD, MPH
             Adolescent SE
                  Px
•   Vitals OK, afebrile
•   Alert, O x 3, NAD
•   Head/Neck OK
•   Chest/cor/abd OK
•   Neuro: No focal deficit. MS OK


                         Edward Sloan, MD, MPH
             Adolescent SE
             Clinical Course
•   Labs, tox screen neg
•   CT negative
•   Neuro consult: EEG and then D/C
•   Dx: AMS, r/o Seizure; migraine HA
•   While EEG applied, pt with AMS
•   Agitation, thrashing on cart
                         Edward Sloan, MD, MPH
             Adolescent SE
         Clinical Course (cont.)
•   During EEG, pt with R face focal sz
•   Leftward gaze noted
•   Seizure then generalized
•   Meds were given
•   Seizure terminated


                         Edward Sloan, MD, MPH
             Adolescent SE
         Clinical Course (cont.)
•   Seizure terminated with Rx
•   Pt stabilized, still somnulent
•   ALS transfer team to Children’s
•   Pt with resolving AMS at time of D.C



                         Edward Sloan, MD, MPH
           Adolescent SE
                Dx
• New onset SE
• Complex partial seizures with
  generalized seizure / SE
• Hx migraine headaches



                       Edward Sloan, MD, MPH
            Adolescent SE
                 Rx
• Lorazepam to Rx the acute sz
  – 2 mg IVP x 2
• Valproate for ongoing protection
  – 25 mg/kg load administered
  – Infused over 20 minutes
• PRN meds during transfer

                         Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
Edward Sloan, MD, MPH
         Juvenile Myoclonic Sz
                   Hx
•   21 year old college student
•   No prior neuro history
•   Final exams, sleepless
•   Great party after the last exam
•   Pt with single generalized sz
•   Seizure upon awakening
                          Edward Sloan, MD, MPH
         Juvenile Myoclonic Sz
                   Px
•   Vitals OK
•   Neuro: slightly post-ictal
•   Exam otherwise normal
•   Patient has a 2nd seizure in the ED



                          Edward Sloan, MD, MPH
      Juvenile Myoclonic Sz
                Dx
• Juvenile myoclonic epilepsy
• Related to sleep deprivation, alcohol
  consumption
• Occurs upon awakening
• Responds best to valproate
• Phenytoin may exacerbate sx

                        Edward Sloan, MD, MPH
         Juvenile Myoclonic Sz
                   Rx
•   Benzodiazepines to Rx the acute sz
•   Ongoing protection an issue
•   Valproate is likely the drug of choice
•   Phenytoin may not be optimal
•   Avoid status epilepticus


                          Edward Sloan, MD, MPH
           Conclusions
           Clinical Pearls
• Acute, repetitive spells = sz
• Ongoing altered mental status =
  complex partial SE
• Treat acute szs with lorazepam
• Valproate is the etiology-specific
  ongoing Rx in many young people
• Know the specific JME clinical setting
                       Edward Sloan, MD, MPH
             Conclusions
            Learning Points
•   Acute, repetitive spells = sz
•   Multiple meds and routes possible
•   Opportunity to optimize Rx
•   Acute seizure control: IV benzos
•   2nd line Rx may differ based on Dx
•   Ongoing needs may influence 2nd Rx
•   EEG may be of use in ED SE
                        Edward Sloan, MD, MPH
         Recommendations
       Management Implications
•   Educate about sz etiologies
•   Make multiple drugs available
•   Alternate routes should be used
•   A protocol should exist
•   Utilize EEG when necessary
•   Be aware of optimal Rx at disposition
                         Edward Sloan, MD, MPH

				
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