Seizures and Epilepsy

 A seizure is a transient neurological dysfunction caused by excessive activity of cortical neurons resulting in
paroxysmal alteration of behaviour and/or EEG changes.

Can be a symptom of acute insult to the brain such as:

 Alcohol and illicit drug use/withdrawal
 Brain injury/abnormality (tumor, trauma, vascular)
 CNS infection
 Fever (children)
 Metabolic (hypoglycemia, electrolytes abnormalities, liver or renal failure)
 Medications
 Manifestation of epilepsy
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 Epilepsy is a chronic condition characterized by two or more unprovoked seizures or underlying

predisposition to seizures in a patient who has already had at least one seizure. Epileptic seizures are
generally classified as focal (or partial), arising from one region of the cortex, or generalized seizures, which
arise from both hemispheres, simultaneously. Approximately 4% to 10% of children experience at least one
seizure. The incidence of childhood epilepsy is 1% to 2%.
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Etiology
 Genetic
  Structural (prior stroke, tumour, meningo/encephalitis, perinatal insult, vascular malformation,
malformation of cortical development, neurodegenerative
 Unknown

Perinatal Infections Metabolic Poisoning Neurocutaneous Systemic Other Causative

Conditions Conditions Syndromes Disorders Disorders/Conditions
Malformation Encephalitis Hypoglycemia Lead Tuberous sclerosis Vasculitis Accidental trauma
of cortical
development
Intrauterine Meningitis Hypocalcemia Drug Neurofibromatosis Systemic lupus Child abuse
infection toxicity erythematosus
Hypoxic- Brain Hypomagnesemia Drug Sturge-Weber Hypertensive Increased
ischemic abscess withdrawal syndrome encephalopathy intracranial pressure
encephalopathy
Trauma Hyponatremia Incontinentia Hepatic Tumor
pigmenti encephalopathy
Hemorrhage Storage diseases Remote brain injury
Reye syndrome Febrile illnesses
Degenerative Cryptogenic (no
underlying disorder
found)
Porphyria Familial
Pyridoxine
dependency and
deficiency
N.B: Highlighted are the most common ones.

Classification
 Classification of Seizures and Epilepsy Syndromes
Focal/Partial Seizures Generalized Seizures Epilepsy syndromes
Simple partial (consciousness not
impaired)
 Motor signs
 Special sensory (visual,
auditory, olfactory,
gustatory, vertiginous,
somatosensory)
 Autonomic
Complex partial/focal dyscognitive Myoclonic (rapid, shock-like
(consciousness impaired)
 Psychic (déjà vu, fear)
Absence (staring,
unresponsiveness)
Tonic (sustained contraction)
Clonic (rhythmic contractions)
Tonic-clonic (tonic phase
followed by clonic phase)
contraction)
Symptomatic
 Underlying cause is
identified
Example: Focal epilepsy
associated with a remote
stroke
Idiopathic
 Presumed to have a
genetic etiology
Example: Benign childhood
epilepsy with centrotemporal
spikes, childhood absence
epilepsy, juvenile myoclonic
epilepsy
Cryptogenic
 Epilepsy of uncertain
etiology
Example: Infantile spasms
with no identifiable cause
  Impaired consciousness at
onset
 Development of impaired
consciousness)
Focal seizures with secondarily Spasms (flexion or extension of
generalized convulsions trunk and extremities for <2 secs)
 Jacksonian seizures

Focal seizures
Simple focal (or partial seizures)
 Arise from a specific anatomic focus and may or may not spread to the surrounding brain regions (focal
without loss of awareness).
 Clinical symptoms include:
 Motor (tonic, clonic, myoclonic) – postural, phonatory, forceful turning of
eyes and/or head, focal muscle rigidity, jerking with or without Jacksonian
march (spreading to adjacent muscle groups)
 Sensory: unusual sensations affecting vision, hearing, smell, taste, or touch
 Autonomic: epigastric discomfort, pallor, sweating, flushing, piloerection,
pupillary dilatation
 Psychiatric: symptoms rarely occur without impairment of consciousness and
are more commonly complex partial
Complex partial seizures
 Associated with alteration of consciousness (focal with loss of awareness)
 Dyscognitive features (showing of altered mental status) may occur
 Classic complex seizure is characterized by automatisms such as chewing, swallowing, lip-smacking,
scratching, fumbling, running, disrobing, and other stereotypic movements
 Focal seizures manifesting only with psychic or autonomic symptoms, such as déjà vu during a temporal
lobe seizure, can be difficult to recognize
 It is said to be secondarily generalized when the focal seizures involve the whole brain which produces a
generalized seizure. Such spread is described as Jacksonian march (progression from face to arm to leg)
N.B It is difficult to distinguish primary generalized tonic and clonic seizures from secondarily generalized tonic
and clonic seizures from secondarily generalized focal seizures clinically.

Generalized Seizures
 Tonic, clonic and biphasic tonic clonic may occur alone or in association with other seizure types.
 Seizure begins abruptly but occasionally is preceded by a series of myoclonic jerks.
 During a tonic-clonic seizure, consciousness and control of posture are lost, followed by tonic stiffening
and upward deviation of the eyes.
 Pooling of secretions, pupillary dilatation, diaphoresis, and hypertension are common.
 Clonic jerks follow the tonic phase
 In the post-ictal phase, the child may be hypotonic. Irritability and headache are common as the child
awakens.

Absence Seizure (petit mal)

  Usually seen in children, unresponsive for 5-10s with arrest of activity, staring, blinking or eye-
rolling, no post-ictal confusion; 3 Hz spike and slow wave activity on EEG
 Clinical hallmark is a brief (less than 15 secs) loss of environmental awareness accompanied by
eye fluttering or simple automatisms, such as fumbling with fingers and lip smacking.
 Begins between 4 and 6 years of age.
 Neurologic examination and brain imaging are normal.
 A clinical seizure can be provoked by hyperventilation or strobe light stimulation.

Differentiation Absence Seizure and Complex Partial Seizure

Feature Absence Seizure Complex Partial Seizure
Duration Seconds (<10 seconds; rarely >30 Minutes (>1 minute; rarely <10
seconds) seconds)
Provoking maneuver Hyperventilation (very frequently) Variable, but often none
Photic stimulation (frequently) Hyperventilation (Occasionally)
Photic stimulation (rarely)
Aura Never Frequently
Onset and termination Abrupt Gradual
Eye blinking Common Occasionally
Automatisms Common Frequently
Postictal phase None (return immediately to Confusion, sleepiness
baseline)
Number of seizures Many per day Infrequent (rarely more than one
per day)
EEG features Interictal: normal Interictal: focal slowing or sharp
Ictal: 3-Hz generalized spike-wave waves
 Ictal: Focal discharges (with or
without secondary generalization)
Neurological examination Normal Normal, or focal deficits
Neuroimaging Normal* Normal, or focal abnormalities
(mesial temporal sclerosis, focal
cortical dysplasia, neoplasm,
encephalomalacia)
First-line treatment Ethosuximide or valproic acid** Oxcarbazepine

* A diagnosis of absence epilepsy should be made without neuroimaging

** Oxcarbazepine and carbamazepine are relatively contraindicated for children with typical absence epilepsy, as
their seizures can be exacerbated by these medications

Tonic
 muscleMuscle rigidity in flexion or extension

Tonic – clonic (grand mal)

 mayMay have prodrome of unease or irritability hours to days before the episode
 tonicTonic-ictal phase: muscle rigidity
 clonicClonic-ictal phase: repetitive violent jerking of face and limbs, tongue biting, cyanosis, frothing,
incontinence.
 Post-ictal phase: flaccid limbs, extensor plantar reflexes, headache, confusion, aching muscles, sore
tongue, amnesia, elevated serum CK lasting hours, may have focal paralysis (Todd’s paralysis)
Atypical Absence, Myoclonic and Atonic Seizures
 Atypical absence seizures manifest as episodes of impaired consciousness with automatisms, autonomic
phenomena and motor manifestations, such as eye opening, eye deviation, and body stiffening.
 Associated with slower EEG discharges (2 Hz) and other seizure types.
 Myoclonus is a sudden jerk of all or part of the body (not all are myoclonus); or sporadic contractions
localized to muscle groups of one or more extremities
 Non-epileptic myoclonus may be benign, as in sleep myoclonus, or indicate serious disease.
 Myoclonic epilepsy usually is associated with multiple seizure types.
 The underlying disorder producing myoclonic may be static (juvenile myoclonic epilepsy) or
progressive and associated with neurologic deterioration (neuronal ceroid lipofuscinosis).
 Myoclonic absence refers to the body jerks that commonly accompany absence seizures and
atypical absence seizures.
 Atonic seizures are typically brief (lasting 1 – 2 seconds), they are quite disabling because of a sudden
loss of postural tone, resulting in falls and injuries.

Classic Factors Differentiating Seizure, Syncope and Pseudoseizure

Characteristic Seizure Syncope Pseudoseizure*(Psychogenic
non-epileptic seizure)
Timing Day or night Day Day; other people present
Onset Sudden, in any position Gradual; Upright position Provoked by emotional
(not recumbent) disturbance or suggestion
Prodrome Possible specific aura Lightheadedness, pallor, Variable
diaphoresis
Duration Brief or prolonged Brief Often prolonged
Incontinence Occurs in tonic-clonic or Possible but rare Rare
complex partial
Post-Ictal Occurs in tonic-clonic or No Variable, often none
complex partial
Motor Activity Synchronous, Occasional brief jerks Opisthotonos, rigidity, eye
stereotypic, automatisms closure, irregular extremity
(common in absence and movements, shaking head,
complex partial), lateral pelvic thrust, crying,
tongue biting, eyes rolled geotropic eye movements,
back tongue biting at the tip
Injury Common Rare unless from fall Rare
EEG Usually abnormal; with Normal Normal
or without interictal
discharges
*Pseudoseizures do not rule out seizures (not uncommon to have both)

Investigations
  CBC, electrolytes, fasting blood glucose, Ca2+, Mg2+, ESR, Cr, liver enzymes, CK, prolactin • also
consider toxicology screen, ETOH level, AED level (if applicable)
 CT/MRI (if new seizure without identified cause or known seizure history with new neurologic
signs/ symptoms)
 LP (if fever or meningismus)
 EEG

Treatment
 Avoid precipitating factors
 Indications for antiepileptic drugs (AED): 2 or more unprovoked seizures, known organic brain
disease, EEG with epileptiform activity, first episode of status epilepticus, abnormal neurologic
examination or findings on neuroimaging
 Psychosocial issues: stigma of seizures, education of patient and family, status of driver’s license,
pregnancy issues
 Safety issues: driving, operating heavy machinery, bathing, swimming alone
 Refer for evaluation for possible surgical treatment if focal and refractory
 Febrile Seizures
 A febrile seizure represents the most common cause of seizures occurring in the presence of a
fever among children between 6 months and 6 years of age in a neurologically and
developmentally normal child without evidence of intracranial infection of defined cause.
 It is not considered a form of epilepsy, which is characterized by recurrent non-febrile seizures.
Criteria for diagnosing febrile seizures include:
 A convulsion associated with an elevated temperature > 38°C
 A child > 6 months and < six year of age
 Absence of CNS infection or inflammation
 Absence of acute systemic metabolic abnormality that may
produce convulsions
 No hxn of previous afebrile seizures

Types of Febrile Seizures

 Simple/Typical – are generalized at onset, last < 15 minutes, and occur only once in a 24-hour
period in a neurologically and developmentally normal child. Has an incidence of 70-80%.
  Complex/Atypical Febrile Seizures – are focal at onset, last > 15 minutes, or recurs within 24
hours, or if the child has pre-existing neurologic challenges. Has an incidence of 20-30%
 Febrile status epilepticus – Genetic epilepsies with febrile seizures (GEFS+): Most common
phenotype of GEFS+ consists of seizures with fever in early childhood that, unlike typical febrile
seizures, continue beyond six years of age or are associated with afebrile tonic-clonic seizures.
The epilepsy typically remits by mid-adolescence but can persist into adulthood.

Psychogenic Nonepileptic Seizures

 Previously termed pseudoseizures, may be the manifestation of conversion disorders or malingering.

 Children with genuine epilepsy may, consciously or subconsciously, exhibit concurrent nonepileptic spells.

Differentiation between PNES and Epileptic Seizures

PNES Epileptic Seizures

Patient’s eyes are often closed during seizure Patient’s eyes are open during seizure

Movement is tremulousness or trashing True tonic-clonic activity

 Verbalization and pelvic thrusting are more commonly Less commonly seen
seen

Urinary and Fetal continence is usually preserved Not preserved

Injury does not occur Injury does occur

Tongue is bitten at the tip of the tongue Tongue is bitten to the sides of the tongue

Can be initiated or terminated by suggestion Cannot be initiated or terminated by suggestion

EEG during PNES does not show epileptiform patterns EEG during epileptic seizure does show epileptiform
patterns

Epilepsy Syndrome

 Refers to a group of clinical characteristics that consistently occur together with similar seizure type, age of
onset, electroencephalographic (EEG findings, precipitating factors, inheritance pattern, natural history,
prognosis, and response to antiepileptic drugs (AED’s).

 Syndromes by age at onset

 Neonatal period:
 Benign familial neonatal epilepsy (BFNE)
 Early myoclonic encephalopathy (EME)
 Ohtahara syndrome (aka early infantile epileptic encephalopathy or early infantile
epileptic encephalopathy with burst suppression pattern)
 Infancy:
 West syndrome
 Dravet Syndrome
 Benign familial infantile epilepsy
 Epilepsy of infancy with migrating focal seizures

Syndrome by age

 Children:
 Benign epilepsy with centrotemporal spikes (BECTS) / Rolandic epilepsy
 Lennox – Gastant syndrome
 Childhood absence epilepsy (CAE)
  Adult:
 Juvenile absence epilepsy
 Juvenile myoclonic epilepsy

Benign familial neonatal epilepsy (BFNE)

 The diagnosis of this syndrome in a child with seizures is based on five criteria:
 Normal neurologic examination
 Negative evaluation for another etiology of the seizures
 Normal developmental and intellectual outcome
 Positive family history of newborn or infantile seizures with benign outcome
 Onset of seizures during the neonatal or early infantile period

Clinical features:
 Seizures begin during the first week of life, usually on the third day and goes away within 1 to 4 months.
 The seizures can involve only one side of the brain (focal seizures) or both sides (generalized seizures).
 The seizures usually occur frequently for a few days and then stop. The infant is usually alert and vigorous
during the interictal period.
 Clonic seizures may appear focal or multifocal (most frequent type), although generalized seizures have
also been reported.
 The seizures are generally brief, lasting for approximately 1 to 2 minutes, but may occur as many as 20 to
30 times a day.

Early myoclonic encephalopathy (EME)

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