Pediatric Seizures Muhammad Waseem MD Emergency Medicine Lincoln

Pediatric Seizures Muhammad Waseem, MD Emergency Medicine Lincoln Hospital

Few things are more frightening to parents than to witness their child having a seizure

Objectives n n n Wide spectrum of Pediatric seizure Etiologies specific to children Treatment modalities in children Quality of life issues Legal implications

Seizure n n Common neurologic disorder 3 - 5% of children 1/2 classified as febrile seizures Epilepsy (0. 5 - 1%)

Seizure n n n 10% ambulance calls for children 1. 5% of total ED visit Most resolve in the pre-hospital setting

Seizure - ED visits n n Febrile seizure Established epilepsy New-onset seizure Status epilepticus 53% 31% 10% 5%

Causes n n n Idiopathic Developmental Infection Head trauma Other 76% 13% 5% 3% 2%

Seizure n n Fit Spell Attack Convulsion

What is Seizure?

Seizure n n Paroxysmal, time-limited event that results from abnormal neuronal activity in the brain Paroxysmal alteration in neurologic function (i. e, behavioral, motor, or autonomic function, or all three - volpe 1989.

Convulsion n A seizure with prominent motor manifestation

Epilepsy n n n Disorder of CNS whose symptoms are seizures Recurrent seizures Unprovoked

Seizure n n Most seizures are not epileptic Non-epileptic seizures are physiologic n n n Hypoxia Fever Toxins

Seizure n n n Seizure is a symptom of a disorder that need further investigations Does not constitute a diagnosis May occur in both normal & abnormal tissue

Non-epileptic Events

Mimic Seizures n n n Breath-holding spells Syncope Migraine Tics Night terror Pseudo-seizures

Non-epileptic Events n n Inaccurate diagnoses Inappropriate use of AED

Non-epileptic Events Careful history

Breath-holding spells n n Frightening 6 months - 4 years Inciting event-Shrill cry-Breath holding. Cyanosis Disappear spontaneously before school age

Night Terrors n n n n 5 - 7 years Between midnight and 2 AM Slow wave sleep stage 3 or 4 Frightened and screaming Increased autonomic activity Sleep follows in few minutes No recall

Pseudo-seizure n n n Diagnosis of exclusion 10 - 18 years Bizarre, unusual postures Verbalization Uncharacteristic movements Can be persuaded to have an attack on request

Pseudo-seizure n n n n Lack of cyanosis Talking during seizure Normal reaction to pupil No loss of sphincter control Normal plantar responses Lack of post-ictal drowsiness Poor response to AED

Seizure n First step in identifying the epileptic syndrome is correctly identifying the type of seizure

Why Should I know type of Seizure?

Seizure n n n Clue to cause Appropriate treatment Prognosis

Epileptic Seizures n n n Partial (40%) Generalized Unclassified

Partial Seizure n n n Simple Partial Complex Partial with secondary generalization

Generalized n n Convulsive Non convulsive n Absence Seizure

Generalized- Convulsive n n n Myoclonic Clonic Tonic-clonic Atonic

Simple Partial Seizures (SPS) n n Consciousness not altered Aura Motor activity (face, neck or extremity) “Feeling funny” or “something crawling inside me” n No post-ictal phenomenon

Complex Partial Seizures (CPS) n n Impairment of consciousness Aura Brief blank stare or sudden cessation or pause in activity Automatism (lip smacking, chewing, swallowing and excessive salivation)

Complex Partial Seizures (CPS) n n Dystonic posturing, tonic or clonic movement Postictal phase Duration 1 - 2 minutes Usually during waking hours

Absence Seizure n n n Sudden cessation of motor activity or speech Blank facial expression Flickering of eye lids

Absence Seizure n n n Uncommon before age 5 year Girls No Aura No postictal state Rarely persist longer than 30 sec

Absence Seizure n n Hyperventilation induces an absence seizure 3/sec spike on EEG

Myoclonic n n Quick muscle jerks Loss of body tone Consciousness usually unimpaired Specific epilepsy syndromes

Tonic n n n Tonic spasms of truncal & facial muscles Flexion of upper extremities Extension of lower extremities

Clonic n n n Resembles myoclonus Loss of consciousness Slower

Tonic-clonic n n n Extremely common Begins suddenly without warning Tonic contraction of the trunk Rhythmic clonic contraction alternating with relaxation of all muscle groups Marked increase in HR and BP incontinence

Tonic-clonic n n Seizure last 1 to 2 minutes Post-ictal 30 minutes to 2 hours

Atonic Seizures n n n Suddenly dropping to the floor Lanox-Gastaut syndrome Can occur without LOC

Case 1

Case 1 n n 9 -year-old boy Parents were aroused one night by noise from his bed room Noted bed sheets awry & breathing deeply bitten his tongue

Case 1 n n Confused Afebrile

First Non-Febrile Seizure

History n Was this a true seizure or a nonepileptic event?

History n Circumstances n n n Normal activity vs. provoked Upon awakening Duration Aura Abnormal motor movements Abnormal eye movements/automatism

History n n n Post-ictal period Urinary or fecal incontinence Fever, trauma or drug Birth history Delayed milestones Family history of seizures

Physical Examination n Vital signs Level of consciousness Head circumference (percentile)

Always undress and examine the child

Café-au-lait spot n n n Uniformly hyper-pigmented sharply demarcated macules Normal children (1 -3 spots) 10% of normal children May be present at birth or develop later

Neurofibromatosis (NF-1) n n n Six or more, >5 mm in prepubertal Six or more, >15 mm in postpubertal Crowe sign n freckled appearnace in axilla

Neurofibromatosis (NF-1) n n n Present in 100% of patients present at birth Increase in size, number & pigmentation Predilection for trunk & extremities Spare face

Lisch nodules n n n Pigmented hamartomas of the iris NF-1 Prevalence increases with age n n n 5% 42% 100% (<3 years) (3 -4 years) (21 years)

Lisch nodules n n Asymptomatic Do not correlate with the extent & severity Do not occur in normal individuals Best identified with slit lamp

Adenoma Sebaceum n n Erythematous papules over nose & malar areas Develop between 4 and 6 years of age coalesce & assume fleshy appearance Tuberous sclerosis

Ash-leaf spots n n n Hypo-pigmented Irregular borders May be present at birth Detectable by 2 years in 50% Wood’s ultraviolet lamp

Shagreen patch n n n Roughened raised lesion Orange-peel consistency Primarily lumbo-sacral area

Tuberous Sclerosis n n Infantile spasm Hypsarrhythmic EEG pattern

CT Scan n Periventricular calcifications

MRI n Multiple cortical tubers

Port-wine stain n n Macular cutaneous nevus Present at birth Always involves upper face & eye lids unilateral Sturge-Weber Disease

Port-wine stain n Tonic clonic seizure contralateral to the side of facial nevus Refractory to anticonvulsant hemiparesis

CT Scan n n Normal at birth Gyriform contrast enhancement Hemispheric atrophy Parenchymal calcification n Railroad track

Physical Examination n n Café-au-lait spots (NF) Adenoma sebaceum (TS) Facial hemangioma (Sturge-Weber) Petechiae (meningitis)

Physical Examination n n Hematoma or skull fractures Signs of raised ICP Retinal hemorrhages (Child abuse) Signs of meningeal irritation

Diagnostic Evaluation n n Bedside glucose Serum Ca & Mg (< 3 months old) Urine drug screen CT head Outpatient EEG

Rolandic Epilepsy Benign Partial Epilepsy with Centrotemporal Spikes (BPEC)

Rolandic Epilepsy n n n Common in childhood 2 - 14 years Peak age 9 -10 years Normal children Unremarkable past history Normal neurologic examination

Rolandic Epilepsy n n n n Simple partial seizure 3 -13 years (peak 9 -10 years) Almost always at night (75% sleep) EEG (centrotemporal spike) Carbamazepine Excellent prognosis Spontaneous remission by age 15 year

Infantile Spasm (West’s synd) n n n Sudden jerks of group of muscles 4 -12 months Characteristic EEG (hypsarrhythmia) Poor prognosis ACTH/Steroid

Case 2

Case 2 n 7 -month-old boy with runny nose and fever. His pediatrician saw him & diagnosed URI. He received tylenol. On the same afternoon while sitting on his mother’s lap he began to stare and had a generalized tonic-clonic seizure. The entire episode lasted approx 5 minutes

Case 2 n n n n He fell asleep after the seizure. Normal development T 102 F, HR 124, R 30 BP 90/50 Wt 7. 9 Kg (50%) Ht 66. 5 cm (50%) HC 44 cm (50%) No NC lesions

Febrile Seizures

Febrile seizures n Most common type of seizures in the pediatric age n usually benign n Can cause considerable parental anxiety

Febrile seizures n Seizures that occur in infancy or childhood usually occurring between 3 months and five years, associated with fever, but without evidence of intracranial infection or defined cause

Febrile Seizures n n n Age dependent Rare before 9 months & after 5 years Peak age 9 -20 months Incidence 3 - 4% Family history Diagnosis of exclusion

Febrile Seizures n Risk factors n n n Height of temperature Male sex Family history of febrile seizure

Febrile Seizures n A family history of epilepsy has not been shown to be a risk factor first febrile seizures

Febrile Seizures n Risk factors for recurrence n n n Young age at onset Febrile seizures in first degree relative Lower degree of fever

Febrile Seizures n n Generalized tonic-clonic Duration few seconds to 10 minutes Excellent prognosis 20% are complex

Febrile Seizures n Complex febrile seizure n n n > 15 minutes More than once in 24 hours Focal neurologic features

Febrile Seizures n n Risk of recurrence 34% Most recurrences within 6 -12 months

Lumbar Puncture n The decision to perform LP should be based on the age of the child at presentation (AAP)

Lumbar Puncture n < 12 months n n 12 - 18 months n n Strongly recommend Should consider > 18 months n If history & physical examination suggest intracranial infection

Febrile Seizures n Signs of meningeal irritation n Unreliable under 18 months

Red flags n n n Focal seizure Suspicious physical examination findings (eg, rash, petechiae) cyanosis, hypotension, or grunting Abnormal neurologic examination

Febrile Seizures n Meningitis must be ruled out n Difficult if the patient is on antibiotics

Febrile Seizures n n Determine and treat the cause of fever IV benzodiazepine Rectal diazepam No routine AED prophylaxis

Febrile Seizures n Incidence of epilepsy n n 1% (No other risk factor) 9% (Other risk factors)

Epilepsy n n n Family history of later epilepsy Preexisting neurologic abnormality Complex febrile seizure n n n > 15 minutes duration > 1 febrile seizure per 24 hour Focal febrile seizure

Neonatal Seizures

Neonatal Seizures n n n Seizures during first 28 days 0. 5% of all live births Do not indicate epilepsy

Jitteriness Vs Seizure n n n Movements are stimulus sensitive Appear during active state (crying) Disappear on passive flexion Not jerky No abnormal eye movements

Neonatal Seizures n Neonates are at particular risk n n Metabolic Toxic Structural Infectious

Neonatal Seizures n Not generalized tonic-clonic n n n incomplete myelination Can be very subtle Minimal physical findings

Neonatal Seizures n n Subtle Tonic Clonic Myoclonic

Subtle Seizure n n n More common in premature infants Eye deviation + jerking eyelid blinking fluttering smacking or drooling Apneic spells

Causes n n n Perinatal asphyxia Intracranial hemorrhage Metabolic - hypoglycemia, hypocalcemia Infections Drug withdrawl

History n Family history n n n metabolic Maternal drug history Delivery n n n Mode & nature of delivery Fetal intrapartum status Resuscitative measures

Physical Examination n n Gestational age Blood pressure Presence of skin lesions Presence of hepatosplenomegaly Neurologic evaluation

Lab n n n Serum chemistry Spinal fluid Metabolic work-up n n serum ammonia amino-acids

Lab n Head sonogram n n CT head n n IVH/periventricular Hemorrhage Calcifications Malformations EEG

Management n n The method of treatment depends on the cause Anticonvulsant n Phenobarbital

Status Epilepticus

Status Epilepticus n n Seizure >30 minutes Intermittent seizures longer than 30 minutes from which the patient does not regain consciousness

Status Epilepticus (SE) n n Highest incidence in very young children 5% of ED visit of seizing children 70% of children with epilepsy experience at least one episode of SE Mortality rate 8 to 32%

Status Epilepticus (SE) n n Any type of seizure Generalized (most common) Absence or partial (10%) Febrile SE (25%)

Life-threatening causes n n n Bacterial meningitis Hypoglycemia Increased intra-cranial pressure Hypoxemia Toxins n TCA, Cocaine, Theophylline, insulin

Management n n n Rapid stabilization of cardio-respiratory functions Termination of both clinical & electrical seizures Diagnosis & treatment of life threatening precipitant

Status Epilepticus n “The child is often given too much IV benzodiazepine…. Blood gases are measured and perhaps the values are found to be slightly decreased. The child is then paralyzed, intubated, and sent to the intensive care unit to recover from the iatrogenic morbidity. ”

Status Epilepticus n Freeman JM: Status epilepticus: It’s not what we’ve thought or taught. Pediatrics 1989; 83: 444 -445

Status Epilepticus n n n Primary goal is to stop the seizure First line (benzodiazepine) Second line (phenytoin or fosphenytoin)

Diazepam n n n Rapid onset (3 - 5 minutes) Orally, IV, IM, IO or Rectal Duration of action 20 - 30 minutes Respiratory depression, sedation, hypotension Diastat (rectal gel)

Diazepam n n IV 0. 1 - 0. 5 mg/kg Rectal 0. 2 - 2 mg/kg (maximum 10 mg)

Lorazepam n n n n Slower onset Longer duration (12 - 24 hours) Orally & IV Inappropriate for rectal administration 0. 05 - 0. 2 mg/kg “Must be refrigerated” Tachyphylaxis

Phenobarbital n n Long duration (24 hours) IV 10 -20 mg/kg bolus rate 1 -2 mg/kg/min Intubation (>30 -40 mg/kg) Respiratory depression, hypotension & bradycardia

Phenytoin n n 1950 - Massachusetts General Hospital p. H 12, limited solubility in water Propylene glycol & ethanol 1956 - Parenteral formulation approved 1962 - pediatric dose recommendation 1986 - Revised Pediatric dose (15 -20 mg/kg, 1 -3 mg/kg/min)

Phenytoin n High p. H n n Burning & cutaneous reactions Purple glove syndrome

Phenytoin n Propylene glycol n n n n Seizures Arrhythmia Asystole Hepatic & renal damage Hemolysis Hyperosmolality Lactic acidosis

Phenytoin n The amount of propylene glycol in a typical loading dose of phenytoin administered to a 1 kg premature neonate is about seven times greater than WHO standard

Fosphenytoin 1996 n n n Pro-drug of phenytoin p. H 8 Far more soluble in water No organic solvent Both IV & IM Rapid & complete conversion to phenytoin

Sports Participation

Sports Participation n n Unnecessary restrictions Successful athelete with epilepsy n Gary Howatt (hockey player)

Sports Participation n Which sport “Common sense” Significant metabolic imbalance n n Scuba diving Potential for serious injury

AMA Committee for Sports n “Patients with epilepsy will not be affected by indulging in any sport, including football, provided the normal safegaurds for sports participation are followed, including adequate head protection”

Permitted Sports n n n n Baseball basketball broad jumping hockey gymnastic Soccer wrestling

Reasonable precautions n n n Bicycling Diving Football Skating Swimming

Prohibited Sports n n n Boxing Bungee jumping Polo Scuba diving Skydiving Waterskiing

Driving & Regulatory Issues

Driver Licensing n n Each state has its own regulations “Seizure free period” n 1 Year (NY)

Reporting responsibility n n Patient responsibility Physician responsibility n CA, DE, NE NJ, OR, PA (most states) (Six states)

Employment

Employment n n n Average intelligence Good health Unpredictable loss of consciousness

Employment n n No hard-and-fast rules Should avoid workplaces in which a sudden loss of consciousness may expose them or their coworkers to risk or injury

Employment n n n Interstate truck Forklift Working in heights

Pregnancy & Epilepsy

Pregnancy & Epilepsy n n 20, 000 births women with epilepsy Lower seizure threshold

Offspring & AED

Offspring & AED n Pheytoin n n Valproate n n fetal hydantoin syndrome neural tube defect Carbamazepine n spina bifida

Labor & Delivery

Labor & Delivery n Bleeding tendency in neonate n n induction of hepatic enzymes overcome by Vitamin K

Breast feeding & AED

Breast feeding & AED n n n Nearly all epileptic drugs are transferred in breast milk Phenytoin 18% Phenobarbital 36% Carbamazepine 41% Valproate 5% Breast feeding is not contraindicated

Oral contraceptives & AED

Oral contraceptives & AED n Increase the dose of Oral contraceptives (AED induces hepatic metabolism of hormones)

Don’t forget child abuse Discrepancy between history & injury

“You are mandated by law to protect these children”

It’s not optional n New York State Law (Social Services Law Section 413) requires that any health professional who suspects that a child is being endangered or maltreated must report his/her suspicion to NY City, to the local child protection services

New AED’s

New AED’s n n Gabapentin (Neurontin) Lamotrigine (Lamictal) Vigabatrin (Sabril) Felbamate (Felbatol)

Take home message n n n Wide range of presentation Efficiently obtain information Always undress & examine Establish underlying etiology Suspect abuse with inconsistent history