Ketogenic Diet Treatment - Malaysian Experience Dr. Teik Beng Khoo Paediatric Institute Hospital Kuala Lumpur
The Beginning Ketogenic diet (KD) treatment was first started among a small group of Malaysian children with refractory epilepsy in our hospital in 2006. MALAYSIA Kossoff, E.H., McGrogan, J.R., Worldwide use of the ketogenic diet. Epilepsia 2005
1 st KD Workshop in 2009 Dr. Janak Nathan
IPHKL s Experience (2006-2013)
Khoo TB et al, Neurology Asia 2016 Efficacy & Retention rate 100% 90% 80% 70% 60% 50% 40% 30% 20% 10% 0% 3 month 6 month 9 month 1 year 2 year N= 30 discontinued Ineffective > 50% > 90% seizure free 40% By Intention to treat model and include only those who continued KD up to 2 year
Khoo TB et al, Neurology Asia 2016 Common Adverse Effects (n = 30) n % 1. Constipation 13 43 2. Hunger 7 23 3. Excessive weight gain or loss 6 20 4. Vomiting 3 10 5. Hyperuricaemia 9 30 6. Hypocalcaemia 6 20 7. Renal calculi / Nephrocalcinosis 4 13
Patients characteristic (2006-2018) 84 patients Male : Female 53: 31 Age : 2 months 19 years old (median : 5 yr) Indications:- Epilepsy syndromes n Epilepsy syndromes n 1. Early myoclonic encephalopathy 4 7. Lennox Gastaut syndrome 9 2. Ohtahara syndrome 6 8. Epilepsy with CSWS 1 3. Migrating partial seizure of infancy 6 9. Rasmussen syndrome 2 4. West syndrome 14 10. Other SGE 12 5. Dravet syndrome 4 11. Symptomatic focal epilepsy 10 6. Myoclonic-astatic epilepsy 4 12 F.I.R.E.S. 12 Type of KDs : Classic KD, MCT-KD, MAD
KD Treatment for FIRES (Febrile-Infection Related Epilepsy Syndrome) 12 patients Age: 2 11 years old Outcome at 3 months Duration of Treatment: 1.5 month 8 years Functional Outcome Modified Rankins Score Died (2) Seizure -free (3) 3 (1) 2 >90% (6) 1 Seizure-free Not effective > 90% Died 0 1 2 3 4 5
Case 1 : KD for FIRES Making peace with FIRES
Case 1 : KD for FIRES
KD for EIEE (Early Infantile Epileptic Encephalopathies) 8 7 6 5 4 3 2 1 0 3 months 6 months 9 months 12 months 24 months seizure free > 90% > 50% Ineffective discontinued Sumitha M, Khoo TB (Poster presentation, KETO 2018)
Epilepsy syndrome EEG KD for EIEE Genetic Mutation Outcome 1. Ohtahara Burst suppression STXBP1 > 50% seizure reduction Syndrome 2. EIMFS Migrating KCNT1 > 50% seizure reduction at 3 mo. 3. EIMFS Migrating ATP7A (Menkes) Seizure free at 3 months but passed away at 6 months (palliative) 4. EIMFS Migrating SLC12A5 Seizure free at 6 months, with only occasional breakthrough seizure 5. EIMFS Migrating TBCD124 >90% seizure reduction 6. West syndrome 7. West syndrome 8. West syndrome Hypsarrhythmia 1p36 deletion No change in seizure, but improve in development & alertness Hypsarrhythmia RARS2 > 50% seizure reduction Hypsarrthythmia CDKL5 Not effective
Case 2 : KD for EIMFS 5 months old boy Recurrent apneic episodes at one months old of life. Started to have tonic seizures of extremities at 2.5 months Cyanotic episodes & behavioural arrest at 5 months Migrating focal seizures involving the eye gaze, head deviation and focal clonic jerks of the limbs at 6 months
Case 2 : KD for EIMFS Diagnosis: Epilepsy of infancy with migrating focal seizures (EIMFS) EIEE panel: SLC12A5 mutation Treatment: Drug resistant (CZP, PB, PHT, VPA, LEV, TPM) Seizure-free 6 months after KD treatment except occasional breakthrough seizures Off KD treatment at 3 years old and remains seizure free
Case 2 : KD for EIMFS EEG (3 months after KD) Normal awake background with no epileptiform discharges Almost normal sleep background
Keto Formula
Samples of Keto Meals (past)
Local Resources MyKDKids
Challenges of KD Treatment Compliance and tolerability issues Low long term retention rate China USA (MAD) USA (LGIT) IPHKL 3 months 66% 95% 63% 85% 6 months 45% 74% 46% 57% 12 months 28% - 24% 42% 24 months 9% - - 35% Outpatient KD initiation with low and slow approach Zhonghua Er Ke Za Zhi 2013 Auvin S. Seizure 2012 Muzykewicz DA et al, Epilepsia 2009 Khoo TB et al, Neurol Asia 2016
Conclusion KD has established itself as one of the important Rx in drug resistant epilepsies in Malaysia Our experience with the use of KD so far has been positive and for some families very rewarding.
Acknowledgement Dr. Janak Nathan Dr. Sumitha Murugesu Dieticians Siti Mariam Sharifah Mariam Lai Jaan Jiar Noor Adzha Nur Sakinah Mohd Fatahudin Norfariza (keto nurse) My Keto kids and families Our Keto Team
www.aoccn2019.com
Thank You