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II. ANAMNESISDilakukan secara Autoanamnesis pada tanggal 11 Desember 2014 pukul 09.30 WIB.

Keluhan UtamaKejang berulang 1 minggu sebelum ke Poliklinik Rumah Sakit

Keluhan TambahanDaya Ingat MenurunGangguan Pendengaran

. DIAGNOSIS KERJAa. Diagnosis Klinis : Bangkitan umum epilepsi tipe tonik - klonikb. Diagnosis Topis : Korteks lobus temporalisc. Diagnosis Etiologi : Idiopatik

Diagnosis Banding :- Tumor otak- Gangguan metabolik / sistemikPENATALAKSANAAN1. Farmakologi- Carbamazepin- Clobazam- Metformin- Amlodipin- Mecocobalamine- Piracetam2. Non Farmakologi- Hindari aktifitas berlebihan-Hindari menonton terlalu lama- Hindari membawa kendaraan pribadi- Edukasi psikososialKonsul pada dokter spesialis THT untuk penanganan gangguan pendengaran nya lebih lanjut.

DefinitionSeizure (Convulsion)Clinical manifestation of synchronised electrical discharges of neurons EpilepsyPresent when 2 or more unprovoked seizures occur at an interval greater than 24 hours apartDefinitionProvoked seizuresSeizures induced by somatic disorders originating outside the brainE.g. fever, infection, syncope, head trauma, hypoxia, toxins, cardiac arrhythmiasDefinitionStatus epilepticus (SE) Continuous convulsion lasting longer than 30 minutes OR occurrence of serial convulsions between which there is no return of consciousnessIdiopathic SESeizure develops in the absence of an underlying CNS lesion/insultSymptomatic SESeizure occurs as a result of an underlying neurological disorder or a metabolic abnormalityAetiology of seizures EpilepticIdiopathic (70-80%)Cerebral tumorNeurodegenerative disordersNeurocutaneous syndromesSecondary toCerebral damage: e.g. congenital infections, HIE, intraventricular hemorrhageCerebral dysgenesis/malformation: e.g. hydrocephalusAetiology of seizuresNon-epilepticFebrile convulsionsMetabolicHypoglycemiaHypoCa, HypoMg, HyperNa, HypoNaHead traumaMeningitisEncephalitisPoisons/toxinsAetiology of Status Epilepticus Prolonged febrile seizureMost common causeIdiopathic status epilepticusNon-compliance to anti-convulsantsSudden withdrawal of anticonvulsantsSleep deprivationIntercurrent infectionSymptomatic status epilepticusAnoxic encephalopathyEncephalitis, meningitisCongenital malformations of the brainElectrolyte disturbances, drug/lead intoxication, extreme hyperpyrexia, brain tumorProlonged febrile seizure:-lasting for more than 30min, particularly in a child younger than 3, is the most common cause of SE

Sudden withdrawal of anticonvulsants:-Especially benzodiazepines and barbituratesPathophysiologyStill unknownSome proposals:Excitatory glutamatergic synapsesExcitatory amino acid neurotransmitter (glutamate, aspartate)Abnormal tissues tumor, AVM, dead areaGenetic factorsRole of substantia nigra and GABAPathophysiologyExcitatory glutamatageric synapsesAnd, excitatory amino acid neurotransmitter (glutamate, aspartate)These are for the neuronal excitation In rodent models of acquired epilepsy and in human temporal lobe epilepsy, there is evidence for enhanced functional efficacy of ionotropic N-methyl-D-aspartate (NMDA) and metabotropic (Group I) receptors

Chapman AG. Glutatmate and Epilepsy. J Nutr. 2000 Apr; 130(4S Suppl): 1043S-5SPathophysiologyAbnormal tissues tumor, AVM, dead areaThese regions of the brain may promote development of novel hyperexcitable synapses that can cause seizures

PathophysiologyGenetic factorsAt least 20 % Some examples Benign neonatal convulsions--20q and 8qJuvenile myoclonic epilepsy--6pProgressive myoclonic epilepsy--21q22.3Classification of seizures

Seizures PartialElectrical discharges in a relatively small group of dysfunctional neurones in one cerebral hemisphereAura may reflect site of origin+ / - LOC Generalized

Diffuse abnormal electrical discharges from both hemispheresSymmetrically involvedNo warningAlways LOCSimpleComplexPartial Seizures1. w/ motor signs

2. w/ somato-sensory symptoms

3. w/ autonomic symptoms

4. w/ psychic symptoms

1. simple partial --> loss of consciousness

2. w/ loss of consciousness at onsetSecondary generalized 1. simple partial --> generalized

2. complex partial--> generalized

3. simple partial --> complex partial--> generalizedSimple partialIn simple partial seizures, consciousness is not impaired.Patients can present with motor, somatosensory, special sensory, autonomic or psychic symptoms

complexA complex partial seizure describes a seizure where consciousness is impaired.A partial seizure may begin with a simple seizure, conversely, its onset may coincide with the impairment of consciousness.It may be presented with or without an aura. 2ndary generalizedPartial sezure evolve to secondarily generalized seizures May be gen. Tonic-clonis, tonico or clonicSimple partial seizureswith motor signs Focal motor w/o march Focal motor w/ march Versive Postural Phonatory

Focal motor may remain strictly focal ORthey may spread to contiguous cortical areas producing a sequential involvement of body parts in an epileptic march - Jacksonian seizure

Versive : head turning to one side usually contraversive to the discharge

Simple partial seizures with motor signsSudden onset from sleepVersion of trunkPosturalLeft arm bent Forcefully stretched fingersLooks at watchNote seizure

Simple partial seizurewith versive motor signs and postural motor signs

usually arising from sleep; version of trunk to RL arm bent at elbow, finger forcefully stretched R arm beats on arm of chair to warn the nursetonic contraction of face and eyesjerking of head and L shoulderturns back to normal positionsupport head with R handlook at watch for note seizure in calender

Simple partial seizures with sensory symptomsSomato-sensoryVisualAuditoryOlfactoryGustatoryVertiginousAreas of cortex subserving sensory function, described as pins and needles / numbness

Visual: flashing lights - structured visual hallucinatory phenomena e.g. persons, scenes.

Auditory: crude aud sensations - highly integrated functions e.g. music Olfactory: unpleasant odours

Gustatory sensations: pleasant / odious taste often described as metallic

Vertiginous: sensations of falling in space, floating

Simple partial seizures with sensory symptomsVertiginous symptomsSudden sensation of falling forward as in empty spaceNo LOCDuration: 5 minsSimple partial seizures with autonomic symptomsVomitingPallorFlushingSweatingPupil dilatationPiloerectionIncontinenceborborygmi

Simple partial seizures with autonomic symptomsStiffness in L cheekDifficulty in articulating R side of mouth is drySalivating on the L sideProgresses to tongue and back of throat

Simple partial seizures with psychic symptomsDysphasiaDysmnesicCognitiveAffectiveIllusionsStructured hallucinationsPsychic: disturbance of higher mental functionDysmnesic symptoms: deja-vu or jamais-vu, forced thinking - panoramic vision ( a rapid recollection of episodes from his/her past life)

Cognitive: dreamy states, distorsions of time sense. Detachment or depersonalisation

Affective: Anger and Fear which is apparently unprovoked and abates rapidly

Simple partial seizure with pyschic symptomsDysmnesic symptomsdj-vuAffective symptomsfear and panicCognitiveStructured hallucinationliving through a scene of her former life againComplex Partial SeizuresSimple partial onset followed by impaired consciousnesswith or without automatismWith impairment of consciousness at onsetwith impairment of consciousness onlywith automatisms

Simple Partial Seizures followed by Complex Partial SeizuresSeizure starts from awake stateImpairment of consciousnessAutomatismslip-smackingright legComplex Partial Seizures with impairment of consciousness at onset Suddenly sit upRoll about with vehement movementSimple Partial Seizures to Complex Partial Seizures to Generalised Seizures Initially unable to communicate but understandsAutomatismSmackingHand-rubbingAbolished communicationGeneralised tonic-clonic seizure Begins with an epigastric rising sensation, which the patient signalslater, unable to speak, waving her L hand, cant communicatefollowed by smacking and hand-rubGeneralized seizuresAbsenceMyoclonicClonicTonicTonic-clonicAtonic

Generalized seizure: Atonic1: lack of normal toneTonic1: under continuous tensionClonic1: rhythmic contractions and relaxations of a muscle in rapid successionMyoclonic1: single or repetitive sudden twitching or spasm of a muscle or a group of musclesAbsence seizuresSudden onsetInterruption of ongoing activitiesBlank stare Brief upward rotation of eyesDuration: a few seconds to 1/2 minuteEvaporates as rapidly as it started

Absence seizuresStops hyperventilatingMild eyelid clonusSlight loss of neck muscle toneOral automatisms

Slight impairment of mental performance:Heard what was spoken to her before the seizure stopped, but has probably not understood it qte right

are you there and is it there sound very close to each other in DanishMyoclonic seizuresSudden, brief, shock-likePredominantly around the hours of going to or awakening from sleepMay be exacerbated by volitional movement (action myoclonus)

Tonic-clonic seizures(grand mal)Tonic PhaseSudden sharp tonic contraction of respiratory muscle: stridor / moan FallsRespiratory inhibition cyanosisTongue bitingUrinary incontinenceClonic PhaseSmall gusts of grunting respirationFrothing of salivaDeep respirationMuscle relaxationRemains unconsciousGoes into deep sleepAwakens feeling sore, headaches

Category Localization-relatedGeneralized Idiopathic Benign epilepsy of childhood with centrotemporal spikes(benign rolandic epilepsy)Benign occipital epilepsy Benign myoclonic epilepsy in infancyChildhood absence epilepsyJuvenile absence epilepsyJuvenile myoclonic epilepsy

Symptomatic (of underlying structural disease)Temporal lobeFrontal lobeParietal lobeOccipital lobe Early myoclonic encephalopathyCortical dysgenesisMetabolic abnormalitiesWest syndromeLennox-Gastaut syndrome Cryptogenic Any occurrence of partial seizures without obvious pathology Epilepsy with myoclonic absencesWest syndrome (with unidentified pathology)Lennox-Gastaut syndrome (with unidentified pathology) Table 1.Modified ILAE Classification of Epilepsy Syndromes Special syndromesFebrile convulsionsSeizures occurring only with toxic or metabolic provoking factorsNeonatal seizures of any etiologyAcquired epileptic aphasia (Landau-Kleffner syndrome)Table 1.Modified ILAE Classification of Epilepsy Syndromes (cond)Diagnosis in epilepsyAims:Differentiate between events mimicking epileptic seizuresE.g. syncope, vertigo, migraine, psychogenic non-epileptic seizures (PNES)Confirm the diagnosis of seizure (or possibly associated syndrome) and the underlying etiologyDiagnosis in epilepsyApproach:History (from patient and witness)Physical examinationInvestigations

Physical ExaminationGeneralesp. syndromal or non-syndromal dysmorphic features, neurocutaneous featuresNeurologicalOther system as indicatedE.g. Febrile convulsion, infantile spasmInvestigationsI. Exclusion of differentials:Bedside: urinalysisHaematological: CBPBiochemical: U&Es, Calcium, glucose, ABGsRadiological: CXR, CT headToxicological: screenMicrobiological: LP(Always used with justification)

InvestigationsII. Confirmation of epilepsy:Dynamic investigations : result changes with attacksE.g. EEGStatic investigations : result same between and during attacksE.g. Brain scan

Electroencephalography (EEG)EEG indicated whenever epilepsy suspectedUses of EEG in epilepsyDiagnostic: support diagnosis, classify seizure, localize focus, quantifyPrognostic: adjust anti-epileptic treatmentInternational 10-20 System of Electrode Placement in EEG

Electroencephalography (EEG)EEG interpretation in epilepsyHemispheric or lobar asymmetriesPeriodic (regular, recurring)Background activity:Slow or fastFocal or generalizedParoxysmal activity:Epileptiform features spikes, sharp wavesInterictal or ictalSpontaneous or triggeredEducation & SupportInformation leaflets and information about support groupAvoidance of hazardous physical activitiesManagement of prolonged fits Recovery positionRectal diazepamSide effects of anticonvulsants

AnticonvulsantsSuppress repetitive action potentials in epileptic foci in the brainSodium channel blockadeGABA-related targetsCalcium channel blockadeOthers: neuronal membrane hyperpolarisation

Na channel blockade: phenytoin, carbamazepine, lamotrigine

GABA-related targets: benzodiazepines interact with specific receptors on GABAa receptor-chloride ion channel macromolecular complex

Ca channel blockade: ethosuxamide inhibits low-threshold Ca currents especially in thalamic neurons that act as pacemakers to generate rhythmic cortical discharge

Other mechanisms: Valproic acid - neuronal membrane hyperpolarisation possibly by enhancing K channel permeabilityAnticonvulsantsCabamazepinePhenytoinValproic acidTonic-clonic and partialEthosuximideValproic acidClonazepamAbsence seizuresValproic acidClonazepamMyoclonic seizuresDiazepamLorazepamShort term controlPhenytoinPhenobarbitalProlonged therapyStatus EpilepticusCorticotropinCorticosteroidsInfantile SpasmsDrugs used in seizure disordersInfantile spasms: corticotropin and corticosteroids are commonly used but cause cushingoid side effects.