Pediatric Seizure and Status Epilepticus Management in the Emergency Setting

University of Illinois College of Medicine Chicago, IL Edward P. Sloan, MD, MPH Associate Professor & Research Development Director Dept of Emergency Medicine University of Illinois College of Medicine Chicago, IL

Attending Physician Emergency Medicine University of Illinois Hospital Our Lady of the Resurrection Medical Center

Pediatric Seizures & SE Clinical Case A 13 year old female presents with a frontal HA and prior migraines that are relieved with ibuprofen She had some AMS in the AM, with unusual motor activity (restless, thrashing on bed) She had no other systemic sx, recent illness, or head trauma She presented with normal vital signs and normal neurologic exam What should the emergency physician do? What is the expected outcome of this patient?

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

Overview Session Objectives Review main peds sz types, etiologies Briefly discuss Rx based on sz type Discuss relevant ED peds sz cases Summarize what Rx options exist Discuss rational treatment decisions

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%)

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 dvlp epilepsy SE most common before age 1

Overview Pediatric SE 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

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

Pediatric Sz Etiologies Hyponatremia Causes long duration szs and SE Infants < 6 months old, no clear etiol Too much water in formula Hypothermia (Temp < 36.5 degrees)

Pediatric Sz Etiologies Cocaine Toxicity Consider in new onset seizures Crack cocaine rocks ingested Especially when no other etiology Common in urban EDs

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%)

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

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

Seizure Classification Generalized Seizures Convulsive seizures Tonic sz: sustained contractions Clonic sz: rhythmic flexor spasms Tonic-clonic sz: combined mvmts Non-convulsive Simple absence: impaired consciousness Complex absence: brief motor mvmts

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

Pediatric Seizures Other Generalized Sz Types Neonatal seizures Benign childhood epilepsy (Rolandic) Infantile spasms (West syndrome) Lennox-Gastaut syndrome Atonic seizures Febrile seizures

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

Specific Seizure Types Generalized Tonic-Clonic Sz Seizure described as a convulsion May occur primarily or secondarily May be preceded by prodrome or aura Tonic, then clonic phase Tongue biting, urinary incontinence Last for minutes, then post-ictal

Specific Seizure Types Absence Seizure Petit mal epilepsy Brief, limited motor activity Sudden interruption of consciousness Slight clonic mvmts, myoclonic jerks Automatisms also can be seen Last about 10 sec, not post-ictal

Specific Seizure Types Partial Seizure Focal motor sz (Jacksonian, frontal) Focus and/or lesion in cerebrum Sz clearly related to a lesion Sz type related to site of sz focus CT scan is useful Simple partial sz pts have no AMS

Specific Seizure Types Complex Partial Seizure Psychomotor, temporal lobe epilepsy Often a history of febrile seizures Complex aura, altered behavior Automatisms: lip smacking, chewing Not complete LOC, instead confused May secondarily generalize

Specific Seizure Types Neonatal Seizure Occur in first 28 days of life Most occur shortly after birth Subtle sz: lip smack, eye mvmt, apnea Perinatal asphyxia, metabolic abn Hypoglycemia, hypocalcemia CNS infection, hemorrhage, lesion

Specific Seizure Types Benign Childhood Epilepsy Rolandic epilepsy Onset between 3 and 13 years of age Often occurs upon awakening Facial mvmts, grimacing, vocalizations EEG diagnosis

Specific Seizure Types Infantile Spasms West syndrome Occurs up to one year May be symptomatic or idiopathic Sudden tonic movements of the head, trunk, extremities Must do full work-up, incl metabolic Caution, AED hepatotoxicity a risk

Specific Seizure Types Lennox-Gastaut Syndrome Onset from 1-8 years Peaks at 3-5 years Multiple seizure types GTC, tonic, absence, atonic szs ED Hx: exac of known sz disorder

Specific Seizure Types Atonic Seizures Astatic or akinetic seizures Sudden loss of motor tone Child falls to the floor May have myoclonic jerks No clear generalized seizure No etiology of apparent syncopal episode

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

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

Specific SE 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

Specific SE 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

Specific SE Types Subtle SE Late manifestation of GCSE, frank coma No longer with tonic-clonic mvmts Still actively seizing (electrical SE) Usu in older patients Marked co-morbidity (encephalopathic) Highest SE mortality

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

Seizure Therapy Ongoing Therapies Absence: Ethosuximide, valproate Atonic: Valproate, clonazepam, ethosuximide Myoclonic: Valproate, clonazepam Partial: Carbamazepine, phenytoin, valproate Generalized: Carbamazepine, phenytoin, phenobarb, primidone, valproate

Case Presentations ED Pediatric Seizure Cases Pediatric complex partial SE New onset SE in an adolescent New onset sz in a college student

Pediatric SE: Pediatric Complex Partial SE How do we Dx complex partial SE? What is the optimal Rx protocol? Why?

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

Pediatric SE Hx (con’t) 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…”

Pediatric SE Px 98.7 98/60 72 20 Well hydrated CV, lung exams normal 98.7 98/60 72 20 Well hydrated CV, lung exams normal Neuro exam intact

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

Pediatric SE Clinical Course (con’t) Three more episodes over 40’ Similar autonomic symptoms Some non-purposeful ext mvmts Diaphoresis, urinary incontinence Remained somnolent between episodes

Pediatric SE Dx Repetitive episodes with AMS Autonomic symptoms noted Non-purposeful mvmts noted Rule out complex partial status epilepticus (CPSE)

Pediatric SE Rx IV lorazepam IV valproate Transfer to Children’s ICU observation Uncomplicated course

Adolescent SE: New Onset AMS/Spells What is the AMS? Is it a seizure? How should we Rx new onset szs? What is the role of the ED EEG? When should it be ordered?

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

Adolescent SE Px Vitals OK, afebrile Alert, O x 3, NAD Head/Neck OK Chest/cor/abd OK Neuro: No focal deficit. MS OK

Adolescent SE Question # 1 What diagnostic tests are indicated at this point?

Adolescent SE Question # 2 Did the patient have a seizure? Does it influence Dx, Rx?

Adolescent SE Question # 3 Does the patient require admission for observation for possible new onset seizures?

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

Adolescent SE Question # 4 Is this repeat spell a seizure? What type?

Adolescent SE Question # 5 Does this AMS and motor activity require Rx? What Rx?

Adolescent SE Question # 6 Does the patient now require admission for observation for possible new onset seizures?

Adolescent SE Clinical Course (con’t) During EEG, pt with R face focal sz Leftward gaze noted Seizure then generalized Meds were given Seizure terminated

Adolescent SE Question # 7 What med is to be used for seizure control / SE termination?

Adolescent SE Question # 8 What med is to be used once SE is terminated? Why?

Adolescent SE Question # 9 How should the meds be given? Why?

Adolescent SE Clinical Course (con’t) SE terminated with Rx Pt stabilized, still somnulent ALS transfer team to Children’s Pt with resolving AMS at time of D/C

Adolescent SE Dx New onset SE Complex partial seizures with generalized seizure / SE Hx migraine headaches

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

Juvenile Myoclonic Sz: College Student, New Onset Sz What is the likely etiology? What is JME? What are the long-term implications? How to RX once the sz terminated?

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

Juvenile Myoclonic Sz Px Vitals OK Neuro: slightly post-ictal Exam otherwise normal Patient has a 2nd seizure in the ED

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

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

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

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