TATALAKSANA DIET KETOGENIK PADA ANAK DENGAN SINDROM LENNOX GASTAUT DAN CONDUCT DISORDER

LEONIRMA TENGGUNA, Prof. dr. Sunartini, Ph.D, SpAK; dr. Sasmita Nugraha, Sp.AK

2022 | Tesis-Spesialis | ILMU KESEHATAN ANAK

Latar belakang Sindrom Lennox Gastaut (Lennox Gastaut Disorder/LGS) merupakan tipe epilepsi intraktabel yang terdapat pada 1-2% kasus epilepsi anak. Diagnosis dan tatalaksana LGS tidak mudah serta prognosis kurang memuaskan. Conduct disorder adalah kelainan psikiatri yang dapat ditemukan pada epilepsi dengan jumlah sebanyak 1 dari 24 kasus. Selain obat anti epilepsi, diet ketogenik merupakan tatalaksana alternatif yang dapat dipertimbangkan pada LGS. Kasus Pemantauan jangka panjang dan intervensi multidisiplin dilakukan terhadap anak laki-laki berusia 11 tahun 8 bulan dengan LGS dan conduct disorder. Penelitian ini mengamati luaran klinis serangan kejang, rehospitalisasi akibat status epileptikus, fungsi kognitif, perbaikan perilaku conduct disorder, EKG, EEG, profil lipid, kalsium darah, dan efek samping intervensi farmakologi dan non-farmakologi. Selama 12 bulan pengamatan, luaran yang tercapai yaitu penurunan frekuensi serangan kejang sebanyak >75%, tidak ada rehospitalisasi akibat status epileptikus, perbaikan perilaku conduct disorder, serta EKG dan kalsium darah dalam batas normal. Luaran yang belum tercapai yaitu perbaikan fungsi kognitif dan EEG. Efek samping tatalaksana yaitu hiperplasia ginggiva akibat fenitoin, penurunan fungsi kognitif terutama akibat fenobarbital, dan dislipidemia akibat diet ketogenik. Kesimpulan Diet ketogenik dapat dipertimbangkan sebagai tatalaksana alternatif pada pasien LGS dengan conduct disorder.

Background Lennox Gastaut syndrome (LGS) is a type of intractable epilepsy that presents in 1-2% of pediatric epilepsy cases. The diagnosis and management of LGS is not easy with poor prognosis. Conduct disorder is a psychiatric disorder that can be found in epilepsy in as many as 1 in 24 cases. In addition to antiepileptic drugs, ketogenic diet is an alternative treatment that can be considered in LGS. Case Presentation Long-term monitoring and multidisciplinary intervention were carried out on an 11 years 8 months old boy with LGS and conduct disorder. This study looked at the clinical outcomes of seizures, rehospitalization due to status epilepticus, cognitive function, improvement in conduct disorder behavior, ECG, EEG, lipid profile, serum calcium, and side effects of pharmacological and non-pharmacological interventions. During 12 months of observation, the outcomes achieved were decrease in the frequency of seizures by >75%, no rehospitalization due to status epilepticus, improvement in conduct disorder behavior, and normal ECG and blood calcium level. Outcomes that have not been achieved are the improvement of cognitive function and EEG. Side effects of treatment are gingival hyperplasia due to phenytoin, and decreased cognitive function due to phenobarbital. Conclusion Ketogenic diet can be considered as an alternative treatment for LGS patients with conduct disorder.

Kata Kunci : Lennox Gastaut syndrome, LGS, conduct disorder, epilepsy, ketogenic diet