EPILEPSI

Ruslan Muhyi

Definisi
Epilepsi Kejang tanpa pencetus yang terjadi 2x atau lebih Tanpa pencetus: tidak ada kelainan yg bersamaan, trauma otak Status 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. 3rd 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 sundrome Idiopathic (with age-related onset)
Benign childhood epilepsy with centrotemporal spikes Childhood epilepsy with occipital paroxyms Primary reading epilepsy

Symptomatic
Chronic-progressive epilepsia partialis continua Syndromes characterized by seizure with spesific modes of precipitation Temporal lobe epilepsies Frontal lobe epilepsies Parietal lobe epilepsies Occipital lobe epilepsies Cryptogenic

klasifikasi
Generalized epilepsies and syndromes Idiopathic (with age-related onset)
Benign neonatal familial convulsion Benign neonatal convulsions Benign myoclonic epilepsy in infancy Childhood absence epilepsy Juvenile myoclonic epilepsy Epilepsy with grand mal seizures on awakening Other generalized idiophatic epilepsies Epilepsies with seizures precipitated by specific modes of activation

Cryptogenic or symptomatic
West syndrome Lennox-Gastaut syndrome Epilepsy with myoclonic-astatic seizures Epilepsy with myoclonic seizures

Symptomatic
Nonspecific etiology Early myoclonic encephalopathy Early infatile epileptic encephalpathy with suppression burst Other symptomatic generalized epilepsies Specific syndromes Epileptic seizure complicating other disease states

klasifikasi
Epilepsies and syndromes undetermined whether focal or generalized With both generalized and focal seizures
Neonatal seizures Severe myoclonic epilepcy of infancy Apilesy with continuous spike waveds during slow wave sleep Acquired epileptic epilepsies Other undetermined epilepsies

Without unequivocal generalized or focal seizure

Special syndrome Situation-related seizures Febrile convulsions Isolated seizures or isolated status epilepticus Seizure occuring only with acute metabolic or tonic avents
Swaiman et al. Pediatric neurology: priciples & practice. 3rd 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. 3rd ed. St. Louis:Mosby;1999 Nelson 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

Tidak
Kelainan menyerupai kejang

Kejang?
Kejang pertama Metabolik; EEG? CT scan? MRI? LCS?

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

Abnormal
Kejang simtomatik Terapi penyebab dasarnya OAE bila diperlukan

Normal EEG normal

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

Normal (kec.EEG)
Epilepsi idiopatik Terkontrol Monitor berkala, OAE, observasi ES obat, EEG

Klasifikasi

Nelson Textbook of pediatrics, 17th ed, 2004

Tdk terkontrol Perawatan, EEG ulang, penyesuaian dosis, pencitraan, monitor berkala

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, 2004 Buku 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 ketogenik

efektif 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;1999 Holmes G. Epilepsy surgery in children. Neurology 2002;58:12

tata laksana Edukasi epilepsi, terapi, efek samping obat, pengaruh terhadap kehidupan sosial & akademik, pertolongan pertama bila terjadi kejang
Nelson Textbook of pediatrics, 17th ed, 2004