EPILEPSY in INFANCY,

CHILDHOOD and
ADOLESCENCE
PROF. DR. SANDA MGUREANU
DR. DIANA BRC
Pediatric Neurology Department,
Al Obregia Clinical Hospital

History

Epilepsy was described and recognized as a

disease on its own in antiquity, but
identifying its many forms has been done far
more recently the XIX-th century when it
was divided into the 2 main categories:
petit mal/grand mal

What is epilepsy?

A chronic disorder of the brain, characterized

by:
an enduring predisposition to generate epileptic
seizures

with neurobiological, cognitive, psychological and
social consequences

!! The definition of epilepsy requires the

occurrence of at least one epileptic seizure

What is an epileptic seizure? Definition

A transient occurrence of signs and/or symptoms due to abnormal

excessive or synchronous neuronal activity in the brain;

Expressed in signs and/or symptoms which reflect the cerebral

structures and neuronal circuits involved in that specific seizure (
motor, sensitive, psychic signs)

It can be convulsive or nonconvulsive +/- LOC (loss of conscience);

sudden change in behavior characterized by changes in sensory perception or

motor activity

I can be generated in a normal brain as response to various aggression

(drugs, alcohol, hypoglicemia, infections, trauma, etc) occasional
seizures acute symptomatic/provoked seizures;

EPILEPSY- definition

CHRONIC DISORDER OF THE BRAIN

recurrent seizures;

> 2 spontaneous epileptic seizures;

particular mechanism;
+/- psychiatric signs;
age at onset;
evolution;
response to treatment;

EPILEPSY - features

The tendency to reoccur:



structural anomalies;

genetic/ constitutional predisposition;

both;

Incidence: 1 new case/2000 inhabitants;

Prevalence: 0,5-0,8% of general population (1 epileptic person /200

inhab);

EPILEPTIC SEIZURE = transitory event

EPILEPSY = chronic disorder

Seizures and epilepsies classification why?

Communication

Evolution & outcome

Evaluation: what tests and when?

Treatment: when? what? for how long?

Research identifying the genes

Associated comorbidities

Proposed Diagnostic Scheme for New

Classification of the Epilepsies

Axes 1 Ictal phenomenology

Axes 2 Seizure type
Axes 3 Syndrome
Axes 4 Etiology
Axes 5 Impairment ( optional)

1. Ictal phenomena

Semiological Seizure Classification ( purely on

ictal signs! ):
Auras

Motor

negative seizures ( atonic, astatic, hypomotor,
akinetic)

2. Seizure type

Self-limited/ continuous seizure types:

generalized

partial ( focal)

INTERNATIONAL SEIZURES CLASSIFICATION

SEIZURES CLASSIFICATION

PARTIAL (FOCAL)

Simple

GENERALIZED

Complex
Absence Myoclonic

May generalize secondarily

Atonic

Tonic
Clonic
Tonic-clonic

SEIZURE CLASSIFICATION
Partial sz

ATTENTION SIGNAL
SYMPTOM!

onset of seizure in one area of one

cerebral
hemisphere

Manifestations of
the seizure are
determined by the
cortical site at
which the seizure
arises;

EEG focal
abnormalities +/secondary
generalization;

Generalized sz

uncertain origin;

involvement of both
cerebral hemispheres;

Clinical - generalized
appearance;

EEG
generalized
epileptiform
abn;

PARTIAL ( FOCAL)
SEIZURES

1. Simple partial seizures

onset motor, somatosensory, autonomic, psychic

symptoms, etc
conscience retained
may secondarily generalize

2. Complex partial seizures

impairment of consciousness/awareness, without its complete loss

stereotyped ex: epigastric sensation, dj vu
+ purposeless automatisms
>50% of partial seizures originate from the temporal lobe auras
semiology varies with site of origin

Partial ( focal) onset seizure left T

Generalized sz

EEG FOCAL abnormalities

DIN COLECTIA CLINICII DE

NEUROLOGIE PEDIATRICA A SP
OBREGIA

Particularities of partial seizures in children:

Classification of simple / complex partial seizures

is very difficult at this age;

At pre-scholar age: automatisms - reduced oral

movements (different from the adulthood);

EEG-s doesnt help to diagnose the level of

consciousness;

Memory of recognizing the ictal events difficult

to evaluate (preverbal patients);

GENERALIZED
SEIZURES

Generalized seizures
1.
2.
3.

4.
5.
6.

Absence (sudden unresponsive staring);

Myoclonic (myo muscle, clonus - jump);
Tonic (sudden increase in muscle tone and
posturing );
Clonic ( repetitive, rhytmic muscular jerks);
Tonic-clonic ;
Atonic (without muscle tone abrupt falls ).

Absences
History : described first by Poupart in 1705;
CLINICAL PICTURE:
-

short duration, sudden onset and ending , no postictal phenomena

LOC arrest, staring
+ tonic / atonic/ clonic/ vegetative component;
minor automatisms;

TYPES:
typical
atypical (gradual onset and end, prominent automatisms);

Specific epileptic syndromes : childhood absence epilepsy, juvenile absence

epilepsy;

EEG: characteristic! eg.: bilateral generalized 3 Hz spike-and-wave

discharges, provoked by hyperventilation +/- photic stimulation ( CAE)

Clonic seizures:
CLINICAL PICTURE:
-

Rapid muscular contraction/relaxation alternations movement not

influenced by contention!

+ tonic component frequently

Duration: minutes;

Depending on seizure overall duration postictally: rapis

recovery/comatose/ confusional state

EEG: sharpwaves and spikes with low frequency

Focal clonus localization value to contralateral rolandic region !

Tonic-clonic seizures:

Classical, historical epileptic seizure

LOC, tonic flexion tonic extension + apnea,
cyanosis, autonomic signs;
Clonic phase clonic repetitive movements, ,,
respiratory sounds , hypersalivation
+/- sphincterian emission, postictal somnolence

3. Syndromic classification

Syndrome:
a complex of signs and symptoms which appear
together, repeatedly, at a certain age

anatomical localization;

Trigger factors;

Circadian rhythm;

etiology;

EEG;

Prognostic;

Value of syndrome classification

A boy aged 8, no significant history, had a

generalized tonic-clonic seizure.

too little information:

solitary seizure, OR

Sleep deprivation seizure OR

The first sign of childhood absence epilepsy OR

The first sign of a systemic disorder

EPILEPTIC SYNDROMES


-

-

-

Idiopathic epilepsies (20%):

Normal brain
Familial history + for epilepsy;
Normal children;
Good response to treatment; some spontaneously cure;
Symptomatic epilepsies(40%):
Focal or diffuse cerebral lesions , fixed or evolutive;
Neurological deficits/ abn brain imaging;
Cryptogenic epilepsies (40%):
They are suppose to be symptomatic;
The cerebral lesion can not be proved by available investigations;

EPILEPTIC SYNDROMES

Localization-related (focal):
Idiopathic: BCECTS

Symptomatic: e.g frontal lobe epilepsy

Cryptogenic

Generalized:
Idiopathic: CAE, JME

Cryptogenic/symptomatic: Lennox-Gastaut

Symptomatic: specific or non specific

EPILEPSY - DIAGNOSTIC
1. History
witnesses/patient;
2. Seizure description


sudden, abrupt, unexpected;


short, stereotyped paroxysmal episode;


stertor;


postictal phenomena: fatigability, myalgia, confusion


the occurring circumstances (awake/sleep);
3. EEG:


helps the + dg;


semiological sindromological classification ;


surveillance;
4. Other investigations: cerebral CT/RMN/SPECT/PET;

Diagnostic algorithm

Is it a epileptic seizure or a nonepileptic paroxysmal

event?

Is it an acute symptomatic convulsion?

Which are the triggers?

Semiology of the seizure!

Epileptic syndrome!

GENERALIZED EPILEPSIES
CRYPTOGENIC/SYMPTOMATIC
WEST SYNDROME

Onset: infancy (max 3-12 mo) peak at 5 mo

Clinic: clusters of tonic flexion/extension, at awakening
INFANTILE SPASMS;
Normal or delayed psychomotor development prior to onset of
seizures
regression/ delayed development afterwards
EEG: interictally hypsarrhythmia;
Treatment: corticotherapy - Synachten, valproate (VPA),
vigabatrin (VGB in West sdr in tuberous sclerosis), topiramate
(TPM), benzodiazepine (BZD);
Poor developmental outcome (mental retardation, autism,
hyperkinetic syndrome, Lennox-Gastaut syndrome);

WEST SYNDROME
TRIAD

1. CHARACTERISTIC CLINICAL APPEARANCE

INFANTILE SPASMS
2. HYPSARRHYTHMIA ON EEG
3. USUALLY POOR DEVELOPMENTAL
OUTCOME

1. EPILEPTIC SPASMS


Sudden movements

short lasting 0,2-2sec

clusters - 1-30/day, 1 cluster = 20-150 spasms

Tonic axial + limbs contractions - flexion/extension/flexion-extension.

Symmetric ( 1-30% asymmetric, with head + eyes deviation, cerebral lesions)




occuring at awakening ( mostly) or at falling asleep, rarely in non REM , exceptionally in REM
sleep stage
LOC ( 90 sec)

Breathing problems, cry

Very subtle expression: yawning, facial grimaces, hiccuping

! A history of a couple of wks/mo of subtle movements it is frequently present before hospital

admission


+ other sz

2. Severely abnormal EEG pattern:

pattern disorganized, discontinuous, high
amplitude, multifocal spikes called HYPSARRHYTHMIA( gr Hypsos=
Hypsos= high/tall)
high/tall)

DIN COLECTIA CLINICII DE

NEUROLOGIE PEDIATRICA A SP
AL.OBREGIA