2.seizures in childhood
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SEIZETRANSCRIPT
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SEIZURES IN CHILDHOOD
Kindu Woldemichael, MD
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Seizure (Sz)• Defn: paroxysmal, time-limited change in motor
activity and/or behavior that results from abnormal electrical activity in the brain.
• Common, occur in ~10% of children, - most are provoked by somatic disorder
originating outside the brain: high fever, infection, hypoxia, toxins, arrhythmias, or electrolyte abnormalities (Na+, Ca++);
- may also signal serious systemic/ CNS problem.
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Epilepsy • A condition in which Sz is triggered recurrently from
within the brain without obvious precipitants. • for epidemiologic purposes- it is defined as two or
more unprovoked Sz occurring at an interval of >24 hr apart.
• not a specific disease, but a condition arising from variety of pathological insults involving the cerebral cortex.
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Evaluation of the first Sz• In the Hx-• look for features suggesting
– meningitis,– sepsis, – head trauma, – ingestion of drugs, toxins;
• details of pregnancy & delivery; [perinatal asphyxia]
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• detailed description of the Sz type:– focal/generalized,– duration, – consciousness,– sensory/motor (if motor: tonic, tonic-clonic,
clonic, myoclonic, spasm, atonic, etc), – posture of the patient, loss of sphincter control
• preceding aura (epigastric discomfort ,pain)
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Cont’d…• On examination- - ABC, - Temperature, BP - HC, length/ ht, Wt - papiledema, retinal hemorrhage, - focal signs- subtle hemiparesis/ hyperreflexia,
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Cont’d
- cutaneous signs of neurocutaneous syndromes vitiligo of Tuberous Sclerosis(TS), shagreen patch of TS),café-au-lait spots of NF-1,nevus flammeus of Sturge -Weber syndrome).
• Investigations- depending on suspected cause.
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port-wine stains
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Hypomelanotic macules (ash leaf spots) on the thorax of a child with tuberous sclerosis
Skin-colored and pink-tan, soft papules & nodules on the back- neurofibromata
Shagreen patch on back of patrient with tuberous sclerosis
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Unprovoked Sz• First Sz - potential family hx of epilepsy, - prior neurologic disorder, - hx of Sz with fever, - LP- has limited value, - EEG- recommended
- for Dx of event, - prediction of recurrence risk, - for Dx of focal abnormality.
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Cont’d- Neuroimaging- generally not recommended unless
neurological examinations indicate a possible abnormality.
- anticonvulsants: not recommended for only one Sz.
• Recurrent Sz - epileptic with risk of recurrence, - type of Sz, frequency, precipitating factors (1st
convulsion may be with a viral illness), - behavioral abnormalities before several days,
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Cont’d- prolonged personality change/ intellectual
deterioration (degenerative CNS disease), - vomiting, failure to thrive (structural lesion),- hx of previous Rx, drugs that potentiate Sz.
On examination- - neurologic, ophthalmologic & general exam, - ↑ ICP, neuro-cutaneous synd., structural brain
abnormalities ( injuries, malformations, infections, tumors).
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Cont’d- EEG, . EEG can be abnormal in 3-5% of normal children.- serum electrolyte( Mg, Ca, Na),- LP (occasionally- chronic infection, metabolic abn.)- Neuroimaging- - esp. for those with neurological deficits or partial Sz,
or focal neurological abn.)- MRI- more sensitive than CT for detecting brain
malformations & dysplastic lesions.
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Etiology - Any insult to the cerebral cortex can cause Sz,- Typically arise from the neocortical gray matter & the
limbic system, some may arise from subcortical regions.
- Idiopathic/cryptogenic, - vascular, - traumatic, - developmental, - infectious, - neoplastic, & - degenerative causes respectively are among the common
causes.
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International Classification of Epileptic Sz (Based on Hx & EEG)
• Partial Sz: - Simple partial (consciousness retained) . motor, sensory, autonomic, psychic
- Complex partial (consciousness impaired) . Simple partial, followed by impaired consciousness . Consciousness impaired at onset
- Partial Sz with secondary generalization
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Classification cont’d
• Generalized Sz - Absences- typical, atypical - Generalized tonic-clonic, tonic, clonic - Myoclonic - Atonic - Infantile spasms
• Unclassified Sz
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[myoclonus]
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[infantile spasm]
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[frontal lobe epilepsy]
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[geliastic]
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[automatism]
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[atonic seizure]
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[atonic]
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Epilepsy syndromes• Using age of onset, cognitive development,
neurological exam, Sz type & EEG findings;• Syndromic classification enables appropriate Mx
(including surgery) & Px;• Examples : - Infantile spasms, - Benign myoclonic epilepsy of infancy, - Lennox- Gastaut syndrome, - Rolandic epilepsy of childhood,
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Partial Sz -Most common Sz type in all age groups, >50% of all Sz in children;
• Simple partial Sz (SPS)– motor activity most common, clonic/tonic, involve face,
neck, & extremities,– no automatism, – some may have aura,– average duration- 10-20 sec,– conscious,– no post-ictal event.
DDx- tics (but these can be suppressed) - EEG- spikes/sharp waves uni-/bi/ multifocal.
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• Complex partial Sz (CPS)
• may begin with SPS. • aura is present in 1/3• Aura always indicates a focal source of seizure• Loss of consciousness may coincide with onset or
proceed after seizure.• brief blank stare, sudden pause• period of impaired consciousness brief & few,• automatism common (50-75% of cases), develop
AFTER loss of consciousness & may persist into the post-ictal state: include lip smacking, chewing, & swallowing.
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Cont’dIn older children- picking & pulling at clothes,
walking/running- nondirective, repetitive; - CPS can result in 2º generalization, - duration average 1-2min, - EEG- anterior temporal lobe sharp waves/ focal
spikes, & multifocal spikes; - CT & MRI- abnormality in the temporal lobe
sclerosis, hamartoma, gliosis, glioma, AVMs.
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Generalized Sz Absence Sz: Simple (typical) absence (Petit mal)- Sudden cessation of motor activity or speech with a
blank facial expression & flickering of the eyelids, no loss of body tone,
- uncommon before 5 yr of age; girls>boys;- usually <30sec, no aura / post-ictal state,- countless Sz daily, automatism frequent,- Hyperventilation for 3-4 min produces it.- EEG- Typical 3/sec spike & generalized wave discharge
DDx- CPS (few in #, aura, postictal state, motor signs)
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• Generalized Tonic-Clonic Sz (Grand mal)
- most common generalized Sz, may follow a partial Sz or occur de novo;
- may be ass’ed with aura (focal origin);
- duration- few minutes;
- loss of consciousness, eyes roll back, tonic contraction, clonic movements, & loss of bladder control, ± cyanosis, apnea.
- Post ictally semi comatose & then deep sleep from 30min to 2 hr.
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• Myoclonic Epilepsies of Childhood - repetitive Sz consisting of brief, often symmetric
muscular contractions, loss of body tone & falling/slumping forward
- 5 distinct sub groupings:Benign Myoclonus of InfancyTypical Myoclonic Epilepsy of early childhoodComplex Myoclonic Epilepsies (Lennox-Gastaut)Juvenile Myoclonic EpilepsyProgressive Myoclonic Epilepsies
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• Infantile Spasms - begin between 4-8 mo of age; - brief, symmetric, contractions of the neck, trunk, and
extremities; - 3 types: flexor, extensor, & mixed[most common]; Flexor- sudden flexion of neck, arms, & legs,Extensor- extension of the trunk & extremities,Mixed- most common.
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Cont’d
- a cry may precede or follow - occur during sleep/arousal, usu. awake - EEG- hypsarrhythmia – bilateral - classified as cryptogenic & symptomatic. * In the symptomatic pts. many pre-, peri-, & post natal
factors: Hypoxic-Ischemic Encephalopathy (HIE), IntraVentricular Hemorrhage (IVH), PeriVentricular Leukomalacia (PVL), Cong. infections, neurocutaneous synd., CNS infections precede the spasms;
80-90% risk of MR.
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Pathogenesis of infantile spasms- several theories
- dysf’n of the monoaminergic neurotransmitter system in
the brainstem; - derangement of neuronal structure in the brainstem; - abnormal immune system; - CRH- [corticotropin releasing] stress/injury to infant during a critical period of
neurodevelopment, CRH overproduction neuronal hyper excitability & Sz. (exogenous ACTH & steroids used in Rx)
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Febrile Seizures• Most common Sz disorder in childhood;• Age dependent- common b/n 9mo-5y, peak age~14-
18mo;• Incidence- 3-4% of young children;• Possible genetic predisposition;• Generally-excellent prognosis, but may also signify
serious underlying infection, e.g. meningitis;• Usu. generalized tonic clonic, rarely focal;
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Clinical manifestations• Core temp. ≥ 39 ºC;• Generalized tonic-clonic Sz, few sec. to 10min;
• Atypical – duration >15 min, repeated Sz within the same day, focal Sz/ findings;
• ~30-50% have recurrence with later fever;
• Generally no greater risk of later epilepsy, but some factors are associated with ↑ risk:
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Cont’d - atypical, family history, 1st episode at < 9mo, - delayed developmental milestones, - preexisting neurological abnormality,These factors ↑ the risk to ~9% (Vs 1%).• Determine the cause of fever & R/o meningitis, - Viral infections of the URT, AOM, tonsillitis.
Lab. - RBS, LP esp. if 1st episode and in infants, - Other inv. depending on the presentation.
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Rx of febrile convulsion• ABCs,• Arrest convulsion using diazepam,• Careful search for the cause of the fever,• Control the fever- paracetamol & sponging;• Antiepileptics - have no effect,• Reassurance of parents- excellent Px, - fever control at home, - possible recurrence with febrile episodes, - goes away by 5-6 yr of age.
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Cont’d…
Mechanisms of Sz- - precise mechanism unknown.• Needs neurons generating significant burst discharge
& GABAnergic inhibitory system, • Excitatory amino acid neurotransmitters produce
neuronal excitation on specific receptors.• Underdeveloped brain (young age) more susceptible.
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Cont’d
• Sz may arise from areas of neuronal death, abnormal tissue (tumors, AVMs, gliosis).
• Genetic factors- ~20% of all epilepsies.
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Use of the EEG to Dx epilepsy
• paroxysmal discharges during Sz- diagnostic,• the interictal EEG normal in ~40% of cases.
• hyperventilation, eye closure, phototic stimulation, sleep deprivation- ↑ positivity
Rx of Epilepsy• 1st step- ensure that the patient has a Sz
disorder and not pseudoseizures,
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NON-EPILEPTIC EVENTS Vs EPILEPTIC
• Generalized motor seizures cannot be interrupted by vocal or tactile/painful stimulation, with pseudoseizures sudden return to normal after a painful stimulus,
• An abrupt return of consciousness and subsequent loss of consciousness is more typical of a pseudoseizure.
• Waxing & waning of clonic/ tonic activity or stimulus-provoked clonic/tonic activity during a generalized motor seizure a pseudoseizure.
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• A sudden loss of tone in a neurologically normal individual with protective behavior (i.e., outstretched arms) is suggestive of a non epileptic seizure;
• If the patient keeps the eyes tightly closed during the seizure and if passive opening is actively resisted pseudoseizure;
• An abrupt return of normal alertness (i.e., no postictal state) after a generalized motor seizure or prolonged loss of consciousness is unusual in a true seizure.
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• A patient with pseudoseizures will often avoid painful stimuli or, if "unconscious," manifest a positive "arm on face" maneuver, where an apparently flaccid arm avoids hitting the face when dropped from above the face.
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Rx of Epilepsy….- 1st afebrile convulsion, healthy child, no family hx, normal
examination & EEG- Do not treat!!
• 2nd step- choosing an anticonvulsant when indicated. Depends on type of the Sz.
- Goal: use only one drug with few side effects, : dose ↑ slowly until Sz control.• Benzodiazepines – e.g. diazepam, lorazepam. - initial Rx of status epilepticus, - may cause CNS depression esp. with other CNS depressant drugs.
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Mechanisms of Action of AED• AED appear to act primarily by blocking the
initiation or spread of seizures. The mechanisms include:
- inhibition of Na-dependent AP in frequency-dependent manner (e.g., phenytoin, carbamazepine, lamotrigine, topiramate, zonisamide,)
- inhibition of voltage-gated Ca2+ channels (phenytoin),
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- decrease of glutamate release (lamotrigine), - potentiation of GABA receptor function
(benzodiazepines and barbiturates), - ↑ in the availability of GABA (valproic acid,
gabapentin, tiagabine), - ethosuximide and valproic acid, probably act
by inhibiting T-type Ca2+ channels in thalamic neurons.
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• Carbamazepine: - Rx of grand mal & partial Sz.
- plasma conc. decreased by phenytoin, phenobarbital, & valproic acid,
- leukopenia & hepatotoxicity can occur in the 1st 3-4 mo. Do CBC, SGOT & SGPT.
• Ethosuximide - Rx of typical absence Sz.
• Phenobarbital - Rx of grand mal Sz.
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Cont’d
~25% of patients can have behavioral abnormality, may affect cognitive performance.
• Phenytoin - generalized tonic-clonic (primary & secondary),
partial, status epilepticus.
• Valproic acid- - broad spectrum & used in the Rx of grand mal Sz,
absence Sz, atypical absence, & myoclonus.
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Cont’d
• If complete Sz control, a minimum of 2-sz- free years is a safe period of Rx in those with no risk factors.
• risk factors: – age >12yr at onset,– neurologic abnormality,– prior neonatal Sz,– many Sz before control.