Epilepsy 1.pdf

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EPILEPSY in INFANCY,EPILEPSY in INFANCY,CHILDHOOD andCHILDHOOD and

ADOLESCENCE ADOLESCENCE

PROF. DR.PROF. DR. SANDA MSANDA M Ă Ă GUREANUGUREANUDR. DIANA BDR. DIANA B ÂR ÂR CC Ă Ă

Pediatric Neurology Department,Pediatric Neurology Department,““ Al Obre ia Al Obregia”” Clinical HospitalClinical Hospital



HistoryHistory

Epilepsy was described and recognized as aEpilepsy was described and recognized as adisease on its own in antiquity, butdisease on its own in antiquity, butidentifying its many forms has been done faridentifying its many forms has been done farmore recentlymore recently –– the XIXthe XIX--thth century when itcentury when itwas divided into the 2 main categories:was divided into the 2 main categories:

“petit mal/grand mal”“petit mal/grand mal”





What is epilepsy?What is epilepsy? A chronic disorder of the brain, characterized A chronic disorder of the brain, characterized

by:by: an enduring predisposition to generate epileptican enduring predisposition to generate epileptic

seizuresseizures

with neurobiological, cognitive, psychological and with neurobiological, cognitive, psychological and

social consequencessocial consequences

!! The definition of epilepsy requires the!! The definition of epilepsy requires theoccurrence of at least one epileptic seizureoccurrence of at least one epileptic seizure



What is an epileptic seizure? DefinitionWhat is an epileptic seizure? Definition A transient occurrence of signs and/or symptoms due to abnormal A transient occurrence of signs and/or symptoms due to abnormal

excessive or synchronous neuronal activity in the brain;excessive or synchronous neuronal activity in the brain;

Expressed in signs and/or symptoms which reflect the cerebralExpressed in signs and/or symptoms which reflect the cerebralstructures and neuronal circuits involved in that specific seizustructures and neuronal circuits involved in that specific seizure (re (motor, sensitive, psychic signs)motor, sensitive, psychic signs)

It can beIt can be convulsive orconvulsive or nonconvulsivenonconvulsive +/+/-- LOCLOC (loss of conscience);(loss of conscience);

sudden change in behavior characterized by changes in sensory pesudden change in behavior characterized by changes in sensory perception orrception ormotor activity motor activity

I can be generated in a normal brain as response to various agg I can be generated in a normal brain as response to various agg ressionression(drugs, alcohol,(drugs, alcohol, hypoglicemiahypoglicemia, infections, trauma, etc), infections, trauma, etc) – – occasionaloccasionalseizuresseizures – – acute symptomatic/provoked seizuresacute symptomatic/provoked seizures;;



EPILEPSY EPILEPSY -- definitiondefinition

CHRONIC DISORDER OF THE BRAINCHRONIC DISORDER OF THE BRAIN

recurrent seizures;recurrent seizures;> > 22 spontaneous epileptic seizures; spontaneous epileptic seizures;

•• particular mechanism;particular mechanism;

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



EPILEPSYEPILEPSY -- featuresfeatures The tendency to reoccur: The tendency to reoccur:

structural anomalies;structural anomalies;

genetic/ constitutional predisposition;genetic/ constitutional predisposition;

both;both;

Incidence: 1 new case/2000 inhabitants;Incidence: 1 new case/2000 inhabitants;

Prevalence: 0,5Prevalence: 0,5--0,8% of general population0,8% of general population (1 epileptic person /200(1 epileptic person /200inhabinhab ); );



EPILEPTIC SEIZURE =EPILEPTIC SEIZURE = transitorytransitory eventevent

EPILEPSY =EPILEPSY = chronicchronic disorderdisorder



Seizures and epilepsies classificationSeizures and epilepsies classification – – why? why? CommunicationCommunication

Evolution & outcomeEvolution & outcome

Evaluation: what tests and when?Evaluation: what tests and when?

Treatment: when? what? for how long? Treatment: when? what? for how long?

ResearchResearch – – identifying the genesidentifying the genes

Associated Associated comorbiditiescomorbidities



Proposed Diagnostic Scheme for NewProposed Diagnostic Scheme for NewClassification of the EpilepsiesClassification of the Epilepsies

Axes 1 Axes 1 – – IctalIctal phenomenology phenomenology

Axes 2 Axes 2 – – Seizure typeSeizure type

Axes 3 Axes 3 – – SyndromeSyndrome Axes 4 Axes 4 – – Etiology Etiology

Axes 5 Axes 5 – – Impairment ( optional)Impairment ( optional)



1.1. IctalIctal phenomenaphenomena

SemiologicalSemiological Seizure Classification ( purely onSeizure Classification ( purely on

ictalictal signs! ):signs! ):

Auras Auras MotorMotor

“negative” seizures (“negative” seizures ( atonicatonic,, astaticastatic,, hypomotorhypomotor,,

akineticakinetic…)…) ……



2. Seizure type2. Seizure type

Self Self --limited/ continuous seizure types:limited/ continuous seizure types:

generalizedgeneralized

partial ( focal)partial ( focal)



INTERNATIONAL SEIZURES CLASSIFICATION INTERNATIONAL SEIZURES CLASSIFICATION



SEIZURES CLASSIFICATION SEIZURES CLASSIFICATION

PARTIAL (FOCAL) GENERALIZED

SimpleSimple ComplexComplex

May generalize secondarily

Absence Myoclonic Atonic Tonic

Clonic

Tonic-clonic



SEIZURE CLASSIFICATION SEIZURE CLASSIFICATION

Partial Partial sz sz GeneralizedGeneralized sz sz

•• onset of seizureonset of seizure --

in one area of onein one area of onecerebralcerebralhemispherehemisphere

•• Manifestations ofManifestations ofthe seizure arethe seizure aredetermined by thedetermined by thecortical site atcortical site at which the seizure which the seizure

arises;arises;

•• EEGEEG – – focalfocalabnormalities +/abnormalities +/--secondarysecondarygeneralization;generalization;

•• uncertain origin;uncertain origin;

•• involvement of bothinvolvement of bothcerebral hemispheres;cerebral hemispheres;

•• ClinicalClinical -- generalizedgeneralizedappearance;appearance;

•• EEGEEG – – generalizedgeneralizedepileptiformepileptiformabnabn;;

ATTENTIONATTENTION --

SIGNALSIGNAL

SYMPTOM!SYMPTOM!



PARTIAL ( FOCAL)PARTIAL ( FOCAL)

SEIZURESSEIZURES



1. Simple partial seizures1. Simple partial seizures

onsetonset – – motor,motor, somatosensory somatosensory , autonomic, psychic, autonomic, psychicsymptoms, etcsymptoms, etc

conscience retainedconscience retained may secondarily generalizemay secondarily generalize





2. Complex partial seizures2. Complex partial seizures impairment of consciousness/awareness, without its complete lossimpairment of consciousness/awareness, without its complete loss

stereotypedstereotyped – – ex:ex: epigastric epigastric sensation, déjà vu sensation, déjà vu

+ purposeless automatisms+ purposeless automatisms >50% of partial seizures originate from the temporal lobe>50% of partial seizures originate from the temporal lobe – – auras auras

semiology semiology varies with site of origin varies with site of origin



Partial ( focal) onset seizurePartial ( focal) onset seizure – – leftleft T T

GeneralizedGeneralized sz sz



EEG FOCAL abnormalitiesEEG FOCAL abnormalities

DIN COLECTIA CLINICII DENEUROLOGIE PEDIATRICA A SP

OBREGIA



Classification of simple / complex partial seizuresClassification of simple / complex partial seizuresis very difficult at this age;is very difficult at this age;

At pre At pre--scholar age: automatismsscholar age: automatisms -- reduced oralreduced oralmovements (different from the adulthood);movements (different from the adulthood);

EEGEEG--ss – – doesn’t help to diagnose the level ofdoesn’t help to diagnose the level ofconsciousness;consciousness;

Memory of recognizing the ictal eventsMemory of recognizing the ictal events – – difficultdifficultto evaluate (preverbal patients);to evaluate (preverbal patients);

Particularities of partial seizures in children:Particularities of partial seizures in children:



GENERALIZEDGENERALIZEDSEIZURESSEIZURES



Generalized seizuresGeneralized seizures

1.1.

Absence (sudden unresponsive staring); Absence (sudden unresponsive staring);2.2. MyoclonicMyoclonic (“(“myomyo’’ – – muscle, “muscle, “clonusclonus”” -- jump);jump);

3.3. Tonic (sudden increase in muscle tone andTonic (sudden increase in muscle tone and

posturing );posturing );4.4. ClonicClonic ( repetitive,( repetitive, rhytmicrhytmic muscular jerks);muscular jerks);

5.5. TonicTonic--clonicclonic ;;

6.6. Atonic Atonic (without muscle tone(without muscle tone – – abrupt falls ).abrupt falls ).



Absences Absences

History :History : described first bydescribed first by PoupartPoupart in 1705;in 1705;

CLINICAL PICTURECLINICAL PICTURE::

-- short duration, sudden onset and ending , noshort duration, sudden onset and ending , no postictalpostictal phenomenaphenomena-- LOCLOC – – arrest, staringarrest, staring-- + tonic /+ tonic / atonicatonic// clonicclonic/ vegetative component;/ vegetative component;--

minor automatisms;minor automatisms;TYPESTYPES::-- typicaltypical-- atypical (gradual onset and end, prominent automatisms);atypical (gradual onset and end, prominent automatisms);

Specific epileptic syndromesSpecific epileptic syndromes : childhood absence epilepsy, juvenile absence: childhood absence epilepsy, juvenile absenceepilepsy;epilepsy;

EEGEEG: characteristic!: characteristic! egeg.:.: bilateral generalized 3 Hz spike-and-wavedischarges, provoked by hyperventilation +/- photic stimulation ( CAE)





ClonicClonic

seizures:seizures:

CLINICAL PICTURE:CLINICAL PICTURE:-- Rapid muscular contraction/relaxation alternationsRapid muscular contraction/relaxation alternations – – movement notmovement not

influenced by contention!influenced by contention!

-- + tonic component frequently+ tonic component frequently

-- Duration: minutes;Duration: minutes;

-- Depending on seizure overall durationDepending on seizure overall duration – – postictallypostictally:: rapisrapisrecovery/comatose/recovery/comatose/ confusionalconfusional statestate

EEG:EEG: sharpwavessharpwaves and spikes with low frequencyand spikes with low frequency

Focal clonus – localization value – to contralateral rolandic region !



TonicTonic--clonicclonic seizures:seizures:

““ClassicalClassical””, historical epileptic seizure, historical epileptic seizure

LOC, tonic flexionLOC, tonic flexion →→ tonic extension + apnea,tonic extension + apnea,

cyanosis, autonomic signs;cyanosis, autonomic signs; ClonicClonic phasephase – – clonicclonic repetitive movements, ,,repetitive movements, ,,

respiratory soundsrespiratory sounds”” ,, hypersalivationhypersalivation

+/+/-- sphincteriansphincterian emission,emission, postictalpostictal somnolencesomnolence



3.3. SyndromicSyndromic classificationclassification Syndrome:Syndrome:

a complex of signs and symptoms which appeara complex of signs and symptoms which appeartogether, repeatedly, at a certain agetogether, repeatedly, at a certain age

anatomical localization;anatomical localization;

Trigger factors; Trigger factors;

Circadian rhythm;Circadian rhythm;

etiology;etiology;

EEG;EEG;

Prognostic;Prognostic;



Value of syndrome classification Value of syndrome classification

A boy aged 8, no significant history, had a A boy aged 8, no significant history, had a

generalized tonicgeneralized tonic--clonicclonic seizure….seizure….

…too little information:…too little information: “solitary” seizure, OR “solitary” seizure, OR

Sleep deprivation seizure OR Sleep deprivation seizure OR

The first sign of childhood absence epilepsy OR The first sign of childhood absence epilepsy OR The first sign of a systemic disorder The first sign of a systemic disorder



EPILEPTIC SYNDROMESEPILEPTIC SYNDROMES

Idiopathic epilepsiesIdiopathic epilepsies (20%):(20%):

-- Normal brainNormal brain

-- Familial history + for epilepsy;Familial history + for epilepsy;

-- Normal children;Normal children;

-- Good response to treatment; some spontaneously cure;Good response to treatment; some spontaneously cure;

Symptomatic epilepsiesSymptomatic epilepsies(40%):(40%):-- Focal or diffuse cerebral lesions , fixed orFocal or diffuse cerebral lesions , fixed or evolutiveevolutive;;

-- Neurological deficits/Neurological deficits/ abnabn brain imaging;brain imaging;

Cryptogenic epilepsiesCryptogenic epilepsies (40%):(40%):

-- They are suppose to be symptomatic; They are suppose to be symptomatic;

-- The cerebral lesion can not be proved by available investigation The cerebral lesion can not be proved by available investigations;s;



EPILEPTIC SYNDROMESEPILEPTIC SYNDROMES

LocalizationLocalization--related (focal):related (focal):

Idiopathic: BCECTSIdiopathic: BCECTS

Symptomatic:Symptomatic: e.g e.g – – frontal lobe epilepsy frontal lobe epilepsy

CryptogenicCryptogenic

Generalized:Generalized:

Idiopathic: CAE, JMEIdiopathic: CAE, JME

Cryptogenic/symptomatic: LennoxCryptogenic/symptomatic: Lennox--GastautGastaut Symptomatic: specific or non specificSymptomatic: specific or non specific



EPILEPSYEPILEPSY -- DIAGNOSTICDIAGNOSTIC1.1. History History

witnesses/patient; witnesses/patient;

2. Seizure description2. Seizure description sudden, abrupt, unexpected;sudden, abrupt, unexpected;

short, stereotyped paroxysmal episode;short, stereotyped paroxysmal episode;

stertorstertor;;

postictalpostictal phenomena: fatigability,phenomena: fatigability, myalgiamyalgia, confusion, confusion

the occurring circumstances (awake/sleep);the occurring circumstances (awake/sleep);

3. EEG:3. EEG:

helps the + dg;helps the + dg; semiologicalsemiological – – sindromologicalsindromological classification ;classification ;

surveillance;surveillance;

4. Other investigations4. Other investigations: cerebral CT/RMN/SPECT/PET;: cerebral CT/RMN/SPECT/PET;



Diagnostic algorithmDiagnostic algorithm

Is it a epileptic seizure or aIs it a epileptic seizure or a nonepilepticnonepileptic paroxysmalparoxysmal

event?event?

Is it an acute symptomatic convulsion?Is it an acute symptomatic convulsion?

Which are the triggers? Which are the triggers?

Semiology Semiology of the seizure!of the seizure!

Epileptic syndrome!Epileptic syndrome!





GENERALIZGENERALIZEDED EPILEPSIEPILEPSIESES

CR CR Y Y PTOGENIC/SPTOGENIC/S Y Y MPTOMATICMPTOMATICWEST SWEST S Y Y NDROMNDROMEE

OnsetOnset:: infancy infancy (max(max 33--1212 momo ) ) – – peak at 5 mopeak at 5 mo

Clinic:Clinic: clusters of clusters of tonic flexitonic flexionon/extensi/extensionon,, at awakening at awakening – –

INFANTILE SPASMSINFANTILE SPASMS;;

Normal or delayed psychomotor development prior to onset ofNormal or delayed psychomotor development prior to onset ofseizures regression/ delayed development afterwardsseizures regression/ delayed development afterwards

EEG: interEEG: interictally ictally – – hypsarrhythmiahypsarrhythmia;;

Tr Treatmenteatment: corticot: corticothheraperapyy -- SynachtenSynachten,, valproate valproate ( ( VPA VPA ) ),, vigabatrin vigabatrin (VGB(VGB – – in Westin West sdrsdr in tuberous sclerosis),in tuberous sclerosis), topiramatetopiramate

( ( TPM TPM ) ),, benzodiazepine ( benzodiazepine ( BZDBZD ) );;

Poor developmental outcome (mental retardation, autism,Poor developmental outcome (mental retardation, autism,

hyperkinetic syndrome, Lennoxhyperkinetic syndrome, Lennox--GastautGastaut syndrome);syndrome);



WEST SYNDROMEWEST SYNDROME

TRIADTRIAD

1. CHARACTERISTIC CLINICAL APPEARANCE1. CHARACTERISTIC CLINICAL APPEARANCE – –

INFANTILE SPASMSINFANTILE SPASMS

2. HYPSARRHYTHMIA ON EEG

3. USUALLY POOR DEVELOPMENTAL3. USUALLY POOR DEVELOPMENTAL

OUTCOMEOUTCOME



1. EPILEPTIC SPASMS1. EPILEPTIC SPASMS Sudden movementsSudden movements

short lastingshort lasting 0,20,2--2sec2sec

clustersclusters -- 11--30/day, 1 cluster = 2030/day, 1 cluster = 20--150 spasms150 spasms

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

SymmetricSymmetric ( 1( 1--30% asymmetric, with head + eyes deviation,30% asymmetric, with head + eyes deviation, →→ cerebracerebral lesions)l lesions)

occuring occuring at awakeningat awakening ( mostly) or at falling asleep, rarely in non REM , exceptional( mostly) or at falling asleep, rarely in non REM , exceptionally in REMly in REMsleep stagesleep stage

LOC (LOC ( →→ 90 sec)90 sec)

Breathing problems, cry Breathing problems, cry

Very subtle expression: yawning, facial grimaces, Very subtle expression: yawning, facial grimaces, hiccuping hiccuping ……

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

admission admission

+ other+ other szsz



2. Severely abnormal EEG patternEEG pattern: disorganized, discontinuous, high

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

DIN COLECTIA CLINICII DE

NEUROLOGIE PEDIATRICA A SP

AL.OBREGIA

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