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PAEDIATRIC SEIZURES & EPILEPTIC SYNDROMES

DR. MOHAMMAD AL NASSERConsultant Pediatric Neurologist

Department of PediatricsKing Saud University

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OBJECTIVES

Seizures (ZT’s) a symptom NOT a diseaseClinical observation crucial for Dx, classification,

and Rx.R/O other paroxysmal, non-epileptic disorders.Acute management & prevention of recurrence.Thoughtful & rational patient work-upOptimum use of anti-epileptic drugs (AED’s)Comprehensive patient (not SZ’s) management.

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DEFINITIONS & TERMS

A seizure = abnormal electrical cerebral cortical discharge clinical alteration (in function and in behavior).

Epilepsy = two or more unprovoked seizures.Status epilepticus= a seizure lasting more than

30 mins. or repeated seizures with NO regain in consciousness (convulsive or non-convulsive).

Aura, ictus, postical….interictal.

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AETIOLOGY OF SZ’

Primary (idiopathic)

- extensive w/u unyielding

- genetic vulnerability

Secondary (symptomatic-provoked)

- congenital (e.g. anomalies, infections)

- acquired (e.g. P-HIE, metabolic…etc.)

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Normal SZ’s thresholdStrong provoking factor

Low SZ’s thresholdNo provoking factor

Seizure

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International Classification Old Terms

General Seizures Absence Petit mal - Typical - Atypical Myoclonic Minor motor Clonic seizures Grand mal Tonic seizures Grand mal Tonic-clonic seizures Grand mal Atonic seizures Akinetic, drop

attacks, minor motor

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International Classification

Old terms

General Seizures Absence Petit mal Myoclonic Minor motor Clonic seizures Grand mal Tonic seizures Grand mal Tonic-clonic seizures Grand mal Atonic seizures Akinetic, drop attacks,

minor motor

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International Classification

Old terms

Partial seizures

P. simple seizures

(consciousness not impaired)

Focal or local seizures

With motor symptoms Focal motor

Jacksonian seizures With somatosensory

symptoms

Focal sensory

With automatic symptoms

With psychic symptoms

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International Classification Old terms

Partial Seizures (cont.)

P. Complex seizures Psychomotor seizures

(consciousness impaired) Temporal lobe seizures

Simple partial onset With impairment

consciousness at onset Partial seizures that

secondarily generalize10

APPROACH TO DIAGNOSIS

What is the problem? (clinical)

Where is the problem? (anatomy)

Why is the problem? (pathology)

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DIAGNOSTIC PROCESS

Questions to be answered:

Was it a seizure (see DDx of SZ’s)?

Was it provoked (e.g. hunger, T.V., fever…)?

How was the onset (focal generalized)?

Precise description of the event (eye-witness)?

Prior neuro-developmental status?

Findings on neurolofic & G. physical exam…..?

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LABORATORY INVESTIGATIONS

R/O treatable conditions: - CBC, platelets, smear AED’s serum

levels

- Glucose, Ca, PO4

- BUN, electrolytes, Cr and CO2

- Liver function

- (+/- CSF & CT scan head)

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LABORATORY INVESTIGATIONS

Neurophysiology:

- EEG (regular, sleep-deprived, videotape)

Neuro-imaging:

- Ultrasound, SXR, CT scan, MRI

(anatomic) PET & SPECT (functional)

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ACUTE MANAGEMENT OF A SEIZURE ATTACK

ABC’s:

- suction

- O2

- position What if:

- can not get I.V. access?

- SZ is refractory?

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ACUTE MANAGEMENT OF A SEIZURE ATTACK (cont.)

I.V. line:

- Get blood

- Give anticonvulsant

a. glucose, Ca

b. benzodiazepine to abort

c. long acting AED to prevent recurrence

What aetiologic diagnosis & manage accordingly.

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LONG TERM PROPHYLAXISTreat or not to treat?Choose drug of choice for type of SZ’s.Single AED & not polypharmacy. Increase till response or side effects.Wait 5 x t ½ after each increment.Add another AED similarly → +/- withdraw 1st one.Monitor drug levels (& evidence of side effects)

timely & appropriately.Consider withdrawing AED/s carefully and rationally.Patient & parent continued education is crucial.? Epilepsy surgery?

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QUESTIONS & ISSUES TO BE

CLARIFIED Do seizures damage the brain?

Why there is no cure for epilepsy?

Is patient going to outgrow this?

Can epileptics function normally?

Do AES’s have long-term side effects?

For how long Rx will be continued?18

FEBRILE CONVULSION

Seizure with fever:

- Seizure (not shivering [rigors])

- Fever, documented, source

outside CNS

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FEBRILE CONVULSION

Simple (typical) FC:

- GTC’s

- less than 15 mins

- no recurrence within 24 hrs.

- no postical abnormality

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FEBRILE CONVULSION

Complex (atypical) FC:

- Mostly focal

- More than 15 mins.

- Recur within 24 hrs.

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FEBRILE CONVULSION

Investigations:

- Like any seizures disorder

- R/O intracranial infection

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FEBRILE CONVULSION

Treatment:

- Abort the attack

- Prophylaxis

- No treatment

- Daily treatment x 2 yrs. (P.B/VPA)

- PRN treatment (Rectal diazepam)

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FEBRILE CONVULSION

Treatment:

40% recurrence of FC- young age at onset- family predisposition- complex-type SZ’s- day nursery

10% atypical SZ’s → non-febrile SZ’s (epilepsy)

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INFANTILE SPASMS

Myclonic spasms: - mixed → flexor → extensor

Hypoarrhythmias on EEG Mental retardation Typically:

- Onset at 3-7/12 of age. - In cluster on awakening - Missed as infantile “colic”

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INFANTILE SPASMS

Aetiology:

• Idiopathic (10-40%):- normal prior development- no brain pathology

• Symptomatic:- brain malformations;- tuberous sclerosis- others

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INFANTILE SPASMS

Investigations:

As other types of SZ’s

Treatment:

Steroids, benzodiazepines, valproate, pyridoxine.

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INFANTILE SPASMS

Prognosis: - ? Underlying cause - Good in 40% if:

- idiopathic - normal development- early treatment

- Bad in 60% if:- symptomatic- develop other SZ’s e.g. Lennox-Gastaut. S.

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PAROXYSMAL DISORDERS MIMICKING SZ’s

Decrease cerebral blood flow (CBF)

Sleep disorders.

Movement disorders

Psychologic disorders

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SIMPLE FAINTING (SYNCOPE)

Mostly in school age children.

Usually non-convulsive.

R/O cardiac dysrhythmias.

Precipitant → vasovagal response → venous

pooling → decrease CBF.

Rx….. Avoid precipitants

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CYANOTIC BREATH-HOLDING ATTACKS

3% of children Few months – 4 years Fright or pain → cry → hold breath in

expiration May show few jerks Slow EEG intra attack but NOT epileptic Rx……?

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REFLEX ANOXIC SEIZURES

“Pallid breath-holding” attacks. Minor trauma → minimal crying → stiff, pale +/-

jerks. Decrease threshold to vagal cardiac inhibitory

reflex → a systole. In 1% of children, mostly 12-18/12 of age. May co-exist with the cyanotic breath-holding. ECG should be done. Rx…..? (transdermal anticholinergic)

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CARDIAC DYSRHYTHMIAS

Consider if: - Syncope:

→ tonic/clonic movements→ prolonged confusion

- Exercise-induced “seizures” - Relatives (“epileptic” or sudden deaths)

Prolonged Q-T int. & sick sinus syndromes. Extensive cardiac investigation is mandatory.

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SLEEP DISORDERS

Nigthmares & night terrors

Narcolepsy & cataplexy

Somnambulish & somniloquy

Sleep apnea

Bruxism, noct, enuresis, noct, myoclonus

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PSYCHOLOGIC DISORDERS

panic attacks

day dreaming

conversion reactions

fictitious epilepsy

hyperventilation syndrome

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