EPILEPSIEPILEPSI

Prof.DR.Dr.H.Prof.DR.Dr.H.Ruslan Ruslan MuhyiMuhyi,SpA(K),SpA(K)

Devisi saraf anak bag ika fk Devisi saraf anak bag ika fk unlam/rsud ulin banjarmasinunlam/rsud ulin banjarmasin

Definisi

Epilepsi• Kejang tanpa pencetus yang terjadi 2x atau

lebih • Tanpa pencetus: tidak ada kelainan yg

bersamaan, trauma otakStatus epileptikus• Kejang terjadi >30 mnt atau kejadian kejang

berulang tanpa pulih sadar diantara kejang Nelson Textbook of pediatrics, 17th ed, 2004

Swaiman et al. Pediatric neurology: priciples & practice. 3 rd ed. St. Louis:Mosby;1999

Klasifikasi (Commission on Classification and Terminology of the International League Against Epilepsy, 1989)

Localization-related (local, focal, partial) epilepsies and syndrome

Idiopathic (with age-related onset)Benign childhood epilepsy with centrotemporal spikesChildhood epilepsy with occipital paroxymsPrimary reading epilepsy

SymptomaticChronic-progressive epilepsia partialis continuaSyndromes characterized by seizure with spesific modes of precipitationTemporal lobe epilepsiesFrontal lobe epilepsiesParietal lobe epilepsiesOccipital lobe epilepsiesCryptogenic

Generalized epilepsies and syndromesIdiopathic (with age-related onset)

Benign neonatal familial convulsionBenign neonatal convulsionsBenign myoclonic epilepsy in infancyChildhood absence epilepsyJuvenile myoclonic epilepsyEpilepsy with grand mal seizures on awakeningOther generalized idiophatic epilepsiesEpilepsies with seizures precipitated by specific modes of activation

Cryptogenic or symptomaticWest syndromeLennox-Gastaut syndromeEpilepsy with myoclonic-astatic seizuresEpilepsy with myoclonic seizures

SymptomaticNonspecific etiologyEarly myoclonic encephalopathyEarly infatile epileptic encephalpathy with suppression burstOther symptomatic generalized epilepsiesSpecific syndromesEpileptic seizure complicating other disease states

klasifikasi

klasifikasi

Epilepsies and syndromes undetermined whether focal or generalizedWith both generalized and focal seizures

Neonatal seizuresSevere myoclonic epilepcy of infancyApilesy with continuous spike waveds during slow wave sleepAcquired epileptic epilepsiesOther undetermined epilepsies

Without unequivocal generalized or focal seizureSpecial syndromeSituation-related seizuresFebrile convulsionsIsolated seizures or isolated status epilepticusSeizure occuring only with acute metabolic or tonic avents

Swaiman et al. Pediatric neurology: priciples & practice. 3 rd ed. St. Louis:Mosby;1999

Kelainan menyerupai epilepsi• Benign paroxysmal vertigo • Night terrors• Sleep walking• Breath-holding spells• Syncope• Paroxysmal kinesigenic choreoathetosis• Shuddering attacks• Benign paroxysmal torticollis of infancy• Hereditary chin trembling• Narcolepsy• Cataplexy• Episodic dyscontrol syndrome• Masturbation• Tic• Migrain• Pseudoseizures

Swaiman et al. Pediatric neurology: priciples & practice. 3 rd ed. St. Louis:Mosby;1999Nelson Textbook of pediatrics, 17th ed, 2004

Patofisiologi

• Gangguan ion channel kelainan gen (generalized epilepsy with febrile seizure, autosomal dominant nocturnal frontal lobe epilepsy, benign familial neonatal convulsions and episodic ataxia type I with partial

seizures)• Eksitasi dan sinkronisasi neuron meningkat • Hipereksitabilitas disebabkan perubahan

fungsi sinaps dan sifat intrinsik neuron; ketidakseimbangan antara sistim neurotransmiter glutamat & GABA

Engelborgh, et al. Pathophysiology of epilepsy. Acta Neurol Belg 2000;100:201-13

Faktor Risiko

• Retardasi mental (90%)• Palsi serebral (90%) • Infeksi SSP (56-77%)• Riwayat kejang demam

The national institute of neurological disorders and stroke: evolving directions in the management of epilepsy. Clinician 2002;20:1-32

Langkah pendekatan pd anak dgn kejang

Kejang?Tidak Ya

Kelainan menyerupai kejang

Kejang pertama

Kejang berulang

Metabolik; EEG? CT scan? MRI? LCS?

-Compliance obat?-Dosis tepat?-Obat tepat?-Ggn metabolik?-Lesi struktural?-Interaksi obat?-Peny. degeneratif?-Epilepsi intraktabel?

Abnormal

Kejang simtomatikTerapi penyebab dasarnyaOAE bila diperlukan

NormalEEG normal

Riw.keluarga (-)Terapi (-)Observasi ketat

Normal (kec.EEG)

Epilepsi idiopatik

KlasifikasiTerkontrolMonitor berkala, OAE, observasi ES obat, EEG

Tdk terkontrolPerawatan, EEG ulang, penyesuaian dosis, pencitraan, monitor berkala

Nelson Textbook of pediatrics, 17th ed, 2004

Pemeriksaan Penunjang

• EEG tidak menunjukkan kelainan pada 50% pasien dng epilepsi. Sensitifitas CT scan 30-50%. Sensitifitas MRI 50-79%

The national institute of neurological disorders and stroke: evolving directions in the management of epilepsy. Clinician 2002;20:1-32

• MRI merupakan pilihan pertama pem. penunjang. CT scan dilakukan bila pem. MRI tdk dapat dilakukan pd keadaan tertentu atau sbgi pem. tambahan bila kelainan tdk terdeteksi dgn MRI

Wieshman UC. Clinical application of neuroimaging in epilepsy. J Neurol Neurosurg Psychiatry 2003;74:466-70

pemeriksaan penunjang

• 2/3 pasien didiagnosis epilepsi berdasarkan klinis dan pemeriksaan EEG

Bassili, et al. Pattern of diagnostic and therapeutic care of childhood epilepsy in Alexandria, Egypt. Intl J Quality in Health Care 2002;14:277-84

• Pemeriksaan urin, darah, cairan serebrospinal, EEG, pencitraan

Nelson Textbook of pediatrics, 17th ed, 2004Buku ajar neurologi anak, 2000

Tata laksana

• Obat antiepilepsi (OAE)Prinsip pengobatan:- OAE diberikan setelah serangan kedua- Jenis OAE yg diberikan tergantung sifat

serangan epilepsi- Monoterapi politerapi- Mencari dosis optimal terendah

Buku ajar neurologi anak 2000 Swaiman et al. Pediatric neurology: priciples & practice. 3rd ed. St. Louis:Mosby;1999

tata laksana

• Diet ketogenikefektif menurunkan frekuensi kejang (>50%) Vining, et al. A multicenter study the efficacy of the ketogenic diet. Arch Neurol

1998;55:1433-7

• Bedah- Kejang berulang yg tdk dpt diatasi dgn OAE- Epilepsi intraktabel- Prosedur: lobektomi temporal,reseksi

nontemporal, corpus callosotomy, hemisherectomy, stimulasi n.vagus

Swaiman et al. Pediatric neurology: priciples & practice. 3rd ed. St. Louis:Mosby;1999Holmes G. Epilepsy surgery in children. Neurology 2002;58:12

• Edukasi

epilepsi, terapi, efek samping obat, pengaruh terhadap kehidupan sosial & akademik, pertolongan pertama bila terjadi kejang

Nelson Textbook of pediatrics, 17th ed, 2004

tata laksana