Pediatric Seizures And infant * Benign familial neonatal

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Pediatric Seizures

• And infant

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Objectives

• How to investigate possible seizures
• History of greatest importance
• How to approach epilepsy syndromes
• How to predict seizure recurrence
• How to choose an anti-epileptic drug

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An approach to Seizures
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The Approach of the Pediatric Neurologist

• Confirm the presence of seizure
• Determine the seizure type
• Consider an epilepsy syndrome
• Select the AED based on seizure type and syndrome
• Outpatient management/continuity of care

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Approach if the child presents after the event?

• Determine if truly seizure
• Determine seizure type
• Identify precipitant or cause and treat
• Determine if further work up needed
• Determine if anticonvulsant therapy is
  appropriate

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Definition

• SEIZURE
• Abnormal, excessive, synchronous electrical
  discharge from a group of cortical neurons.
• Result paroxysmal change in sensory, motor,
  cognitive and/or autonomic function.
• The clinical appearance depends upon the location
  and extent of cortex involved.
• EPILEPSY
• two or more unprovoked seizure

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What is the difference between seizures and
epilepsy ?

• A seizure is a paroxysmal event characterized by
  a change in behavior of the patient
• results when a large population of neurons in the
  brain discharges synchronously
• Epilepsy is the occurrence of two or more
  unprovoked seizures

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Pathophysiology

• Imbalance between excitatory and inhibitory
  neurons
• Immature CNS (cortex) is more prone to seize ...
  period of vulnerability
• excitatory neurons predominate
• inhibitory neurons are under developed
• NT synthesis is altered
• reduced number of synaptic connections

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Pathophysiology

• The spread of a sz is limited by
• active synapse inhibition
• hyperpolarization of normal neurons
  circumferential to the focus

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Seizure mimics in Kids

• Breath holding spells
• Migraine
• Syncope
• dystonia

• Benign nocturnal myoclonus
• Cyclic vomiting
• Acute dystonia
• Sandifer Syndrome
• Choreoathetosis
• Proxysmal torticollis

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Differential Diagnosis - SeizuresNonepileptic
Neurologic Paroxysms

• Anoxic-Ischemic Events
• cyanotic breath holding
• pallid breath holding
• syncope
• toxins and drugs
• Migraine
• Sleep disorders
• night terrors - sleep myoclonus
• sleepwalking

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Differential Diagnosis - SeizuresNonepileptic
Neurologic Paroxysms

• movement disorders
• tics - choreoathetosis
• spasmus nutans - dystonias
• others
• Sandifers syndrome
• benign paroxysmal vertigo
• shuddering attacks
• hyperventilation syndrome
• psychological problems
• pseudo sz - panic attacks

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Seizures and brain damage

• Children with seizures at a significant risk for
  cognitive impairment and behavioral abnormality
• It is difficult to distinguish the effect of
  seizures from the underlying pathology and the
  effect of anticonvulsants
• There is a growing evidence pointing to the
  lasting effect of repetitive , brief seizures in
  early childhood

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First-Time, Non-Febrile Seizures

• 120 in 100000 in US children annually
• 30 go on to develop epilepsy
• Etiology of seizures different based on age

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Afebrile seizures - Classification

• Simple Partial Sz
• alert and interactive
• motor, somatosensory, autonomic,or psychic sx
• no postictal depression
• Complex Partial Sz
• impaired consciousness
• an aura often present
• staring spells and automatisms common
• postictal depression /- amnesia of the ictal
  event

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Classifying Seizures

• Partial seizures
• Simple versus Complex
• Focal motor
• Focal sensory
• Secondary Generalization

• Generalized Seizures
• Convulsive or non-convulsive
• Tonic-Clonic (Grand mal)
• Myoclonic
• Absence (petit mal)
• Atonic
• Tonic
• Clonic

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Afebrile seizures - Classification

• GENERALIZED SZ
• abrupt LOC due to near simultaneous activation of
  the entire cortex
• premonitory sx may exist
• Convulsive
• significant post ictal phase
• Tonic-clonic
• Tonic
• Clonic

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Afebrile seizures - Classification

• Nonconvulsive
• Atonic
• Myoclonic
• Absence

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What the difference between partial and
generalized seizures?

• Partial seizures involve only part of the brain
  at onset , clinically distinguished from GS by a
  lack of complete loss of conscious

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Partial seizures are further subdivided into
simple and complex partial seizures, What the
difference between them ?

• Simple partial seizures do not impaired
  consciousness, complex partial seizures do and
  the patient usually amnestic for the ictal event
• Either may spread and become secondary
  generalized
• An aura may occur at the beginning of either type
  ( noxious smell or taste )

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Generalized seizures are further subdivided into
convulsive and nonconvulsive seizures, What the
difference between them ?

• Convulsive seizures include tonic-clonic, tonic
  and clonic seizures , with post ictal confusion
• Nonconvulsive include absence , myoclonic and
  atonic seizuers
• No post ictal drowsiness in absence seizures

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The Goals of the Emergency/Pediatric Physician

• Correctly identify a seizure
• Acutely manage the seizing patient
• Identify precipitating causes and treat
• Rule out badness
• Pediatric Neurologist Consultation

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What are the etiology of seizures?

• Febrile seizures
• CNS infection
• Trauma ( contusion, hematoma and impact )
• Toxins ( intoxication or withdrawal)
• CNS tumor ( hypothalamic hamartoma)
• Metabolic ( hypoglycemia, electrolyte, inborn
  errors, renal and liver disorders)
• Vascular ( hemorrhage, A-V malformation, cerebral
  vein thrombosis
• Other ( hypoxia, post immunization, V-P shunt
  malf.)

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Afebrile seizures - etiology

• Age-specific approach to diagnosis
• lt 1 MONTH AGE
• HIE, ICH, INFECTION,CNS MALFORMATION,
  CEREBROVASCULAR EVENT, DRUG EXPOSURE, METABOLIC
  DERRANGEMENT (low gluc., Ca ), INBORN ERROR OF
  METABOLISM, PYRIDOXIME DEFICIENCY
• lt 6 MONTHS
• INFECTION, DRUG WITHDRAWL, ELECTROLYTE
  ABNORMALITIES, CNS MALFORMATION, INBORN ERRORS OF
  METABOLISM

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Afebrile seizures - etiology

• 6 MONTHS - 3 YEARS
• BIRTH INJURY, INFECTION, TOXINS, TRAUMA,
  METABOLIC ABNORMALITY, CEREBRAL DEGENERATIVE
  DISEASE
• gt 3 YEARS
• IDIOPATHIC, INFECTION, TRAUMA, CEREBRAL
  DEGENERATIVE DISEASE

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Etiology Sz

• idiopathic
• febrile illness
• acute symptomatic causes
• remote symptomatic causes

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Etiology Acute symptomatic sz

• ROSENS 5TH EDITION TABLE 168-6
• Traumatic
• cerebral contusion , SD, ED, ICH, SAH, impact sz
• Infectious
• meningitis, encephalitis, brain abscess, shigella
• Neoplastic
• primary vs.. metastatic CNS tumour
• Vascular
• AVM, SAH, ICH, cerebral venous

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Acute symptomatic sz - continued

• Toxic
• withdrawal, intoxication
• Metabolic
• hypo- glycemia, natremia, calcemia, magnesemia
• hepatic or renal disorder
• inborn errors of metabolism
• Miscellaneous
• ecclampsia - dialysis
• postimmunization - VP shunt malfunction
• hypoxia - HIE
• neurodegenerative - neurocutaneous

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Acute Symptomatic Seizures

• Hypoglycemia
• Hyponatremia
• Hypocalcemia
• Trauma
• Toxic process

• Meningitis
• Encephalitis
• Hypoxia-ischemia
• Tumor
• Vascular lesion

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Etiology Remote symptomatic sz

• Past Hx of brain insult
• post traumatic
• post infectious
• perinatal asphyxia
• prior vascular insult
• Neurocutaneous disorders
• neurofibromatosis
• tuberous sclerosis
• Sturge-Weber

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First-Time, Non-Febrile Seizures

• Infants lt 6 months
• Idiopathic (32)
• Congenital anomalies (26)
• Inborn Errors (16)
• Electrolyte abnormalities (16)
• Infection (7)
• Trauma (3)

Bui, Delgado, Simon, Am J. of EM, 2002
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Causes of Epileptic Seizures in Children

• Genetic
• Congenital Brain Abnormality
• Brain Insult e.g. trauma, meningitis,
• Progressive lesion e.g. tumor, AVM
• Metabolic

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Genetic Causes of Epilepsy

• Familial neonatal convulsions
• Benign familial infantile epilepsy
• Benign myoclonic epilepsy infancy
• Febrile seizures
• Benign Rolandic seizures
• Absence epilepsies
• Juvenile myoclonic epilepsy

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Epileptic Syndromes

• An epileptic disorder characterized by a cluster
  of signs and symptoms which occur together
• Factors taken into consideration include seizure
  type, etiology, genetics, anatomy, precipitating
  factors and the interictal EEG
• Proposed by ILAE in 1985, revised in 1989
  currently under revision

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Concept of Epileptic Syndromes

• Syndromes are not disease entities
• Etiology and outcome may be diverse
• Lack of concensus about some syndromes
• Not all patients with seizures can be
  pigeon-holed into a specific epileptic syndrome

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Epilepsy Syndromes Infantile

• Benign familial infantileepilepsy
• Febrile seizure

• Infantile Spasms (Wests Syndrome)
• Severe myoclonic epi
• Benign myoclonus epi
• Lennox-gastaut syn

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Epilepsy Syndromes Children

• Benign focal epilepsy of childhood (Rolandic
  epilepsy)
• Childhood Absence epilepsy
• Lennox-Gastaut

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Epilepsy Syndromes Juvenile

• Juvenile absence epilepsy
• Juvenile myoclonic epilepsy

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Diagnostic strategies

• History is the cornerstone
• To differentiate actual and pseudo seizures
• Type of seizure
• The cause or precipitant
• Exam
• Mainly looking for the cause
• No abnormality referred to the seizures

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First-Time, Non-Febrile SeizuresWork Up

• Laboratory studies
• Glucose is the only routine study
• Consider Lytes, Ca, Mg to rule out provoked
  seizure
• Tox screen if possible ingestion
• Metabolic work up if clinically suspicious
• Consider full electrolytes in infants lt 6 months

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Labs

• Not recommended in children who are
  neurologically normal after a sz
• investigations may include
• Electrolytes - Glucose
• Hematology - LFTs
• Toxicology - Prl
• urine and serum organic acids
• CSF

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First-Time, Non-Febrile SeizuresWork Up

• Lumbar Puncture
• Infants lt 18 months
• H/P suggestive of meningitis/encephalitis
• EEG
• All patients
• Post-ictal slowing if done too soon (48 hrs)
• May predict prognosis for recurrences
• May help diagnose an epilepsy syndrome

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How about imaging and EEG after a first seizure?

• Imaging indicated in
• Partial seizures
• Abnormal neurological exam
• EEG
• Rarely needed in the acute setting
• 10-40 dont show epileptiform abnormalities in
  EEG

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EEG

• Reflects the activity of cortical pyramidal
  neurons
• 1/52 post sz , postictal change unlikely to
  interfere
• maximal utility of the EEG is at the start of a
  sz
• ideally EEG obtained during awake to sleep
  transition

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EEG

• prolonged video-EEG monitoring may be required
• sleep deprivation, hyperventilation, and photic
  stimulation may enhance its sensitivity
• Normal EEG does not r/o a sz disorder
• Semiurgent/Emergent indication
• nonconvulsive status

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Imaging

• CT SCAN
• brief exam time
• highly sensitive for blood and bony defects
• will be abnormal in 25 epileptic patients
• MRI
• role in focal sz
• superior anatomical detail (defines temporal lobe
  pathology well)
• limits early bleeding, calcified structures,
  prolonged exam time

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Imaging

• Warranted with
• a prolonged postictal state
• age lt 6 months
• new onset focal deficits
• focal sz or secondarily generalized sz
• sz duration gt 15minutes
• high risk
• neurocutaneous disorder - malignancy
• recent head trauma - shunt revision
• not warranted in generalized unprovoked sz with a
  normal exam

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First-Time, Non-Febrile SeizuresWork Up

• When to get a CT Scan
• Trauma (accidental or non-accidental)
• Post-ictal focal deficits (Todds paresis)
• Persistent ALOC
• Signs of increased ICP
• When to get an (non urgent) MRI
• Abnormal CT scan
• When Peds Neuro requests scan

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Hypsarrhythmia
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Childhood Absence Epilepsy
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Approach in actively convulsing child?

• ABC
• Stop seizure
• Benzo, phenytoin , Phenobarb then
• IV drip ( midazolam, propofol or pentobarbital )
• Prevent seizure recurrent
• Identify precipitant or cause and treat

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Risk factor of recurrence of a seizure ?

• Todds paralysis
• Abnormal EEG
• Family history of epilepsy
• Remote symptomatic seizure
• Seizure while asleep

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Factors influencing recurrence

• Partial seizure type
• First seizure during sleep
• Family history
• H/O a remote neurologic insult
• Epileptic pattern on the EEG

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Sz Prognosis - Recurrence

• Most recur within 1 year (usually lt/ 3 months)
• 90 by two years
• 1/3 children with an unprovoked sz will have
  recurrence
• if a second sz occurs the risk of recurrence
  increases to 75

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Prognosis - Recurrence

• patient risk associated with recurrence
• self limiting sz unlikely to lead to brain damage
  but risk of potential injury must be assessed
• poorly controlled epilepsy may result in
  cognitive decline
• status (associated morbidity and mortality)