CNS Seizures in Childhood
CNS Seizures in Childhood
CNS Seizures in Childhood
Dr.p.natarajan
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Definition
• Seizure:
Paroxysmal, time limited change in brain function
manifested by motor, sensory, autonomic or
psychological phenomena resulting from
abnormal electrical activity in brain
• Seizures occur in about 10% of children. 1-2% in
general population
• Seizures may also signal a potentially serious
underlying systemic or CNS disorder
• 10–20% have persistent seizures refractory to drugs
withy a diagnostic and management challenge
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Epilepsy
• Definition:
Two or more unprovoked seizures >24 hr apart
suggest the presence of an epileptic disorder
• < one third of seizures in children are caused by
epilepsy
• Genetic factors account for 20% of all cases of
epilepsy
• The prognosis is generally good
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Stages of a typical Seizure
preictus-ictus-postictus
1. The prodrome:
change in mood or behaviour or insomnia or feelings
about the impending seizure
2. The aura:
feeling a sense of unexplained fear, a strange smell or a
feeling of numbness; déjà vu phenomenon
3. Epileptic cry:
Some people may cry out at the beginning of a seizure as
the muscles around the vocal cords contract
4. Loss of consciousness
5. Tonic phase:
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6. Clonic phase:
7. Loss of bowel and bladder control.
8. postictal confusion .
9. postictal automatism
10. Post ictal sleep
11. Somnambulism
12. Severe headache.
14. Todd’s palsy
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Common Epilepsy Triggers
1. Flickering or Flashing Light
2. Hyperventilation
3. Lack of Sleep
4. during ovulation, menstruation, pregnancy or
menopause. This is known as “catamenial epilepsy.”
5. Especially foods that are rich in glutamate and aspartame
6. High fever
7. Drugs: inducing hepatic enzymes and rapid metabolism of
antiepileptic drugs
8. Large amounts of alcohol
9. cigarettes acts on receptors for the excitatory
neurotransmitter acetylcholine in the brain, which increases
neuronal firing.
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10. Caffeine
11. Musicogenic Epilepsy
12. Playing chess
13. Hot water- bath epilepsy
14. TV epilepsy
15. Lunar nfluence
16. Stress – white coat
17. Pain and touch
18. Primary reading epilepsy
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• Focal seizures:
Turning of the head and eyes to one side,
Unilateral clonic movements
Sensory disturbance localized to a specific area.
• Tonic seizures ;
Are characterized by increased tone or rigidity,
• Atonic seizures:
Are characterized by flaccidity or lack of movement
during a convulsion.
• Clonic seizures:
Consist of rhythmic muscle contraction and relaxation;
• Myoclonus :
Shocklike contraction of a muscle.
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International classification of seizure types (1981)
by the International League Against Epilepsy (ILAE)
classification Type subtypes
1. Motor
a. Simple partial 2. Sensory
Consciousness present 3. Autonomic
1.Partial 4. psychic
(focal seizures) 1. Simple partial onset, followed by
B. Complex partial impairment of consciousness
Consciousness impaired 2. With impairment of consciousness at
onset
a. Absence (Petit mal) 1. Absence seizures (petit mal)
1. Myoclonic seizures
2. Clonic seizures
2. Generalized 3. Tonic seizures
b. Generalized
4. Tonic–clonic seizures (grand mal)
5. Atonic seizures
6. Infantile spasm
3. Unclassified Febrile seizures
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Pathophysiology
1. Seizures can be viewed as resulting from an imbalance
between excitatory and inhibitory processes in the brain
2. Abnormalities in the membrane properties of neurons,
3. Decreased inhibitory neurotransmission which is primarily by
gamma-amino butyric acid (GABA), or
4. Enhanced excitatory neurotransmission which is primarily
mediated by glutamate.
5. Small group of abnormal neurons undergo prolonged
depolarizations associated with the rapid firing of repeated
action potentials.
6. Epileptic neurons recruit adjacent neurons creating a storm
of electrical activity in the brain leading to clinical seizure
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Simple Partial Seizures
• 40% of childhood seizures; consciousness is maintained
• sub types:
1. With motor signs
2. With sensory symptoms
3. With autonomic symptoms or signs
4. With psychic symptoms
• Characterized by:
1. Asynchronous clonic or tonic movements,
2. Aura (chest discomfort, headache or fear)
3. Involve the face, neck, and extremities
4. Head turning and conjugate eye movements are common
5. Persists for 10–20 sec
6. EEG: spikes or sharp waves unilateral or bilateral 12
COMPLEX PARTIAL SEIZURES (CPS).
(Temporal lobe or Psychomotor seizures)
Subtypes:
1. Simple partial onset, followed by impairment of
consciousness
2. With impairment of consciousness at onset
Characterized by:
1. Begins with or without an aura, followed by
impaired consciousness;
2. Brief blank stare
3. Sudden cessation in activity
4. Automatisms - lip smacking, chewing, swallowing,
walking or running etc
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Partial seizures evolving to generalized seizures
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Epilepsia partialis continua
1. Recurrent motor epileptic seizures that are
focal (hands and face), and
2. Recur every few seconds or minutes for
extended periods (days or years).
3. Cause: focal cortical inflammatory processes
in children (rasmussen's encephalitis),
possibly caused by chronic viral infections, or
autoimmune processes.
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BENIGN PARTIAL EPILEPSY WITH CENTROTEMPORAL SPIKES (BPEC).
Benign Rolandic Epilepsy
• BPEC is a common type of partial epilepsy in childhood
and has an excellent prognosis.
• BPEC occurs between the ages of 2 and 14 Yr and has a
peak age of onset of 9–10 yr.
• The disorder occurs in normal children with a normal
neurologic examination.
• There is often a positive family history of epilepsy.
• Unilateral tonic-clonic contractures of the lower face
frequently accompany the oropharyngeal symptoms
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Generalized Seizures
ABSENCE SEIZURES – Petit mal
1. Characterized by a sudden cessation of motor
activity or speech with a blank facial expression and
flickering of the eyelids
2. Uncommon before age 5 yr,
3. More prevalent in girls,
4. Never associated with an aura,
5. Rarely persist longer than 30 sec,
6. Not associated with a postictal state
7. May experience countless seizures daily
8. After the seizure, patients resume preseizure
activity with no indication of postictal impairment. 17
GENERALIZED TONIC-CLONIC SEIZURES
Grand mal
1. Secondary generalization or occur de novo.
2. Aura suggests a focal origin
3. Patients suddenly lose consciousness
4. Emit a shrill cry;
5. Eyes roll back,
6. Body undergoes tonic contractions,
7. Rapidly become cyanotic with apnea.
8. Rhythmic clonic contractions alternating with
relaxation of all muscle groups.
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9. Children may bite their tongue
10. loss of sphincter control- urination and defecation
11. Postictally, children are initially semicomatose and
typically remain in a deep sleep
12. Truncal ataxia, hyperactive deep tendon
reflexes, clonus, and a babinski reflex coud be
noted
13. Vomiting and an intense bifrontal headache.
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MYOCLONIC EPILEPSIES OF CHILDHOOD.
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Type Age Symptoms EEG Prognosis
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Type Age Symptoms EEG Prognosis
Juvenile 12 and Frequent 4–6/sec Responds
Myoclonic 16 yr myoclonic jerks irregular dramatically
Epilepsy Later generalized spike to valproate
(Janz tonic-clonic and wave
Syndrome). seizures pattern,
enhanced by
photic
stimulation
Progressive 10 and Generalized Polyspike- Genetic
Myoclonic 18 yr tonic-clonic wave disorders
Epilepsies: seizures. discharges, uniformly
Lafora Myoclonic jerks particularly has a grave
disease appear later in the prognosis
Mental occipital
deterioration region
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INFANTILE SPASMS
• Age: 4 and 8 mo and are characterized by brief
symmetric contractions of the neck, trunk,
and extremities.
• Three types:
1. Flexor: consist of sudden flexion of the neck,
arms, and legs onto the trunk,
2. Extensor: extension of the trunk and extremities
3. Mixed: flexion in some volleys and extension in
others,
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Infantile spasm
Two groups:
1. Cryptogenic: uneventful pregnancy and birth
history; good prognosis
2. Symptomatic: following hypoxic-ischemic
encephalopathy with periventricular leukomalacia,
congenital infections, inborn errors of metabolism,
neurocutaneous syndromes such as tuberous
sclerosis, cytoarchitectural abnormalities including
lissencephaly and schizencephaly, and prematurity;
mental retardation is common; bad prognosis
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Mechanism
• Stresses or injury to an infant during a critical
period of neurodevelopment causes CRH-
corticotropin-releasing hormone
overproduction, resulting in neuronal
hyperexcitability and seizures
• Exogenous ACTH and glucocorticoids suppress
CRH synthesis, which may account for their
effectiveness in treating infantile spasms.
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EEG
• The EEG is referred to as hypsarrhythmia,
• Consists a chaotic pattern of high-voltage,
bilaterally asynchronous, slow-wave activity
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EEG
• The EEG is abnormal in about 15% of healthy
subjects and normal in about 20% of patients with
epilepsy
• Epilepsy is therefore a clinical diagnosis which may
be supported by the EEG.
• Spikes and waves occurring in the α, θ and δ ranges
in the interictal EEG record are the hallmark of
epilepsy
• Spikes and waves may combine to produce spike and
wave complexes
• Activation procedures: including hyperventilation,
eye closure, photic stimulation, sleep deprivation
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Normal rhythm spike wave complex
in epilepsy
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Normal EEG
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Focal Generalized
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Petit mal:
3/sec spike and generalized wave discharge
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Myoclonus
• generalized burst of 4
Hz spike-wave
discharges associated
with myoclonic seizures
of the right hand
(closed triangles).
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Hypsarrhythmia in an 8-Month-Old
with Infantile Spasms
EEG shows a chaotic, high amplitude pattern with
multifocal spikes and sharp waves
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Evaluation
1. Assessment of cardiorespiratory and metabolic status
2. Head circumference, length, and weight
3. Papilledema, retinal hemorrhages, chorioretinitis,
coloboma, and macular changes,
4. Unusual facial features
5. Hepatosplenomegaly
6. Adenoma sebaceum, shagreen patch, multiple
cafeau-lait spots, a nevus flammeus, and the
presence of retinal phakoma indicate a
neurocutaneous disorder
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• Electrolytes, toxicology screening, urine and
serum metabolic testing
• Serum glucose
• ECG: heart blocks; prolonged QT syndrome
• LP: meningitis, encephalitis, sepsis,
subarachnoid hemorrhage, or a demyelinating
disorder
• Neuroimaging: for structural brain
abnormalities including malformations,
injuries, infections, or tumors.
• Metabolic testing: including administration of
pyridoxine
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Treatment of Epilepsy
1. Ensure that the patient has a seizure disorder and
not a condition that mimics epilepsy
2. Choose an anticonvulsant.
3. The drug of choice depends on the classification of
the seizure, determined by the history and eeg
findings
4. Use of only one drug with the fewest possible side
effects for the control of seizures.
5. The drug is increased slowly until seizure control is
accomplished
6. Serum anticonvulsant level should be monitored
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7. Stop medication after a complete seizure
control for a minimum of 2 yr without
other risk factors
8. The weaning process should occur over 3
to 6 mo
9. Associated problems:
1. Hyperactivity
2. Aggressiveness
3. Learning disability
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Antiepileptic drugs
Drug Dose Side effects Rx: epilespy
Phenobar 3-8 Hyperactivity, academic Generalized
bitone mg/kg deterioration, reversal of tonic-clonic
sleep cycles Partial
Status
epilepticus
Phenytoin 5-15 Poor seizure control due to Generalized
mg/kg fluctuating drug levels, tonic-clonic
cosmetic side effects, Partial
hirsutism, ataxia Status
epilepticus
Valparin 10-60 Nausea, vomiting, loss of Generalized
mg/kg appetite, weight gain, tonic-clonic
irregular menstruation, Absence
alopecia, Somnolence Myoclonic 39
Drug Dose Side effects Rx: epilespy
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• Surgery for epilepsy: for focal seizures
• Left vagal nerve stimulation produced by an
implanted device reduced seizures by 31–42%
over 18 mo,
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