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Few things are more frightening to parents than to witness their child having a seizure
Objectives
Wide spectrum of Pediatric seizure Etiologies specific to children Treatment modalities in children Quality of life issues Legal implications
Seizure
Common neurologic disorder 3 - 5% of children 1/2 classified as febrile seizures Epilepsy (0.5 - 1%)
Seizure
10% ambulance calls for children 1.5% of total ED visit Most resolve in the pre-hospital setting
Seizure - ED visits
Causes
76% 13% 5% 3% 2%
Seizure
What is Seizure?
Seizure
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
Epilepsy
Seizure
Seizure
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
Non-epileptic Events
Non-epileptic Events
Careful history
Breath-holding spells
Frightening 6 months - 4 years Inciting event-Shrill cry-Breath holdingCyanosis Disappear spontaneously before school age
Night Terrors
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
Diagnosis of exclusion 10 - 18 years Bizarre, unusual postures Verbalization Uncharacteristic movements Can be persuaded to have an attack on request
Pseudo-seizure
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
First step in identifying the epileptic syndrome is correctly identifying the type of seizure
Seizure
Epileptic Seizures
Partial Seizure
Generalized
Absence Seizure
Generalized- Convulsive
Consciousness not altered Aura Motor activity (face, neck or extremity) Feeling funny or something crawling inside me No post-ictal phenomenon
Impairment of consciousness Aura Brief blank stare or sudden cessation or pause in activity Automatism (lip smacking, chewing, swallowing and excessive salivation)
Dystonic posturing, tonic or clonic movement Postictal phase Duration 1 - 2 minutes Usually during waking hours
Absence Seizure
Sudden cessation of motor activity or speech Blank facial expression Flickering of eye lids
Absence Seizure
Uncommon before age 5 year Girls No Aura No postictal state Rarely persist longer than 30 sec
Absence Seizure
Myoclonic
Quick muscle jerks Loss of body tone Consciousness usually unimpaired Specific epilepsy syndromes
Tonic
Tonic spasms of truncal & facial muscles Flexion of upper extremities Extension of lower extremities
Clonic
Tonic-clonic
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
Atonic Seizures
Suddenly dropping to the floor Lanox-Gastaut syndrome Can occur without LOC
Case 1
Case 1
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
Confused Afebrile
History
History
Circumstances
History
Post-ictal period Urinary or fecal incontinence Fever, trauma or drug Birth history Delayed milestones Family history of seizures
Physical Examination
Caf-au-lait spot
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)
Six or more, >5 mm in prepubertal Six or more, >15 mm in postpubertal Crowe sign
Neurofibromatosis (NF-1)
Present in 100% of patients present at birth Increase in size, number & pigmentation Predilection for trunk & extremities Spare face
Lisch nodules
5% 42% 100%
Lisch nodules
Asymptomatic Do not correlate with the extent & severity Do not occur in normal individuals Best identified with slit lamp
Adenoma Sebaceum
Erythematous papules over nose & malar areas Develop between 4 and 6 years of age coalesce & assume fleshy appearance Tuberous sclerosis
Ash-leaf spots
Hypo-pigmented Irregular borders May be present at birth Detectable by 2 years in 50% Wood s ultraviolet lamp
Shagreen patch
Tuberous Sclerosis
CT Scan
Periventricular calcifications
MRI
Port-wine stain
Macular cutaneous nevus Present at birth Always involves upper face & eye lids unilateral Sturge-Weber Disease
Port-wine stain
Tonic clonic seizure contralateral to the side of facial nevus Refractory to anticonvulsant hemiparesis
CT Scan
Railroad track
Physical Examination
Caf-au-lait spots (NF) Adenoma sebaceum (TS) Facial hemangioma (Sturge-Weber) Petechiae (meningitis)
Physical Examination
Hematoma or skull fractures Signs of raised ICP Retinal hemorrhages (Child abuse) Signs of meningeal irritation
Diagnostic Evaluation
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
Common in childhood 2 - 14 years Peak age 9 -10 years Normal children Unremarkable past history Normal neurologic examination
Rolandic Epilepsy
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
Sudden jerks of group of muscles 4-12 months Characteristic EEG (hypsarrhythmia) Poor prognosis ACTH/Steroid
Case 2
Case 2
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
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
Most common type of seizures in the pediatric age usually benign Can cause considerable parental anxiety
Febrile seizures
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
Age dependent Rare before 9 months & after 5 years Peak age 9-20 months Incidence 3 - 4% Family history Diagnosis of exclusion
Febrile Seizures
Risk factors
Febrile Seizures
A family history of epilepsy has not been shown to be a risk factor for first febrile seizures
Febrile Seizures
Young age at onset Febrile seizures in first degree relative Lower degree of fever
Febrile Seizures
Generalized tonic-clonic Duration few seconds to 10 minutes Excellent prognosis 20% are complex
Febrile Seizures
Febrile Seizures
Lumbar Puncture
The decision to perform LP should be based on the age of the child at presentation (AAP)
Lumbar Puncture
< 12 months
Strongly recommend Should consider If history & physical examination suggest intracranial infection
12 - 18 months
> 18 months
Febrile Seizures
Red flags
Focal seizure Suspicious physical examination findings (eg, rash, petechiae) cyanosis, hypotension, or grunting Abnormal neurologic examination
Febrile Seizures
Febrile Seizures
Determine and treat the cause of fever IV benzodiazepine Rectal diazepam No routine AED prophylaxis
Febrile Seizures
Incidence of epilepsy
Epilepsy
Family history of later epilepsy Preexisting neurologic abnormality Complex febrile seizure
Neonatal Seizures
Neonatal Seizures
Seizures during first 28 days 0.5% of all live births Do not indicate epilepsy
Jitteriness Vs Seizure
Movements are stimulus sensitive Appear during active state (crying) Disappear on passive flexion Not jerky No abnormal eye movements
Neonatal Seizures
Neonatal Seizures
incomplete myelination
Neonatal Seizures
Subtle Seizure
More common in premature infants Eye deviation + jerking eyelid blinking fluttering smacking or drooling Apneic spells
Causes
Perinatal asphyxia Intracranial hemorrhage Metabolic - hypoglycemia, hypocalcemia Infections Drug withdrawl
History
Family history
metabolic
Physical Examination
Gestational age Blood pressure Presence of skin lesions Presence of hepatosplenomegaly Neurologic evaluation
Lab
Lab
Head sonogram
CT head
EEG
Management
Phenobarbital
Status Epilepticus
Status Epilepticus
Seizure >30 minutes Intermittent seizures longer than 30 minutes from which the patient does not regain consciousness
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%
Any type of seizure Generalized (most common) Absence or partial (10%) Febrile SE (25%)
Life-threatening causes
Management
Rapid stabilization of cardio-respiratory functions Termination of both clinical & electrical seizures Diagnosis & treatment of life threatening precipitant
Status Epilepticus
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
Freeman JM: Status epilepticus: It s not what we ve thought or taught. Pediatrics 1989;83:444-445
Status Epilepticus
Primary goal is to stop the seizure First line (benzodiazepine) Second line (phenytoin or fosphenytoin)
Diazepam
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
Lorazepam
Slower onset Longer duration (12 - 24 hours) Orally & IV Inappropriate for rectal administration 0.05 - 0.2 mg/kg Must be refrigerated Tachyphylaxis
Phenobarbital
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
1950 - Massachusetts General Hospital pH 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
High pH
Phenytoin
Propylene glycol
Seizures Arrhythmia Asystole Hepatic & renal damage Hemolysis Hyperosmolality Lactic acidosis
Phenytoin
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
Pro-drug of phenytoin pH 8 Far more soluble in water No organic solvent Both IV & IM Rapid & complete conversion to phenytoin
Sports Participation
Sports Participation
Sports Participation
Scuba diving
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
Reasonable precautions
Prohibited Sports
Driver Licensing
1 Year (NY)
Reporting responsibility
Employment
Employment
Employment
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
Pheytoin
Valproate
Carbamazepine
Nearly all epileptic drugs are transferred in breast milk Phenytoin 18% Phenobarbital 36% Carbamazepine 41% Valproate 5% Breast feeding is not contraindicated
Increase the dose of Oral contraceptives (AED induces hepatic metabolism of hormones)
It s not optional
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
Wide range of presentation Efficiently obtain information Always undress & examine Establish underlying etiology Suspect abuse with inconsistent history