1. Ketogenic Diet for Treating Infantile Spasms: ½ year experience Mortensen M¹, Nielsen H¹, Povlsen JH¹, Johansen D¹, Daneman K¹, Miranda MJ¹ ¹Danish Epilepsy Centre, Dianalund, Denmark Introduction Infantile spasms (IS) are a severe epilepsy syndrome in early childhood with a variety of aetiologies IS have a high morbidity and are difficult to treat¹ Cessation of spasms by early and appropriate treatment is essential for improving outcome Ketogenic Diet (KD) has been successfully used for treating medically refractory IS² ³ Aim To evaluate the effectiveness of KD for treating medically refractory IS, regarding tolerability and seizure reduction (retrospective study). Patients and methods Retrospective study of the first 8 infants treated with KD All infants had previously been treated with Vigabatrin, Hydrocortisone and a mean of 5 (3-7) antiepileptic drugs (AEDs) (VPA, TPM, LEV, Zonisamide, benzodiazepines). Table 2 - Results Table 1 - Patient data Table 3 - The Ketogenic Diet r References Conclusions KD has shown to be an effective and safe treatment for medically refractory IS The clinical response is positive (measured by reduction or cessation of spasms) in most infants (7/8) and the tolerability good (8/8) Reduction (6/8)/ interruption (2/8) of AEDs is an additional positive effect We can not conclude on EEG improvement in this study metmor@vestamt.dk marimn@vestamt.dk Cur rent Age (mo) Gen -der DiagnosisEpilepsy onset (mo) Diet start (mo) EEG before KD 119F Cryptogenic West Syndrome 410 Hypsarritmia 222M Symptomatic West Syndrome (Deletion 22q13) 916 Hypsarritmia 318M Symptomatic West Syndrome (Trisomy 21-Down S.) 512 Hypsarritmia 418F Early Myoclonic Encephalopathy 612 Suppression-burst 518M Cryptogenic West Syndrome 413 Multifocal 616M Symptomatic West Syndrome (Multiple cerebral malformations) 212 Multifocal 725M Cryptogenic West Syndrome 321 Hypsarritmia 818M Cryptogenic West Syndrome 315 Hypsarritmia Seizures before KD (mean/week) Seizures after 1 mo (mean/week) Seizures after 3 mo (mean/week) Seizures after 6 mo (mean/week) Total Seizure reduction EEG at follow-up 18018111088 % Multifocal 2210000100 % n/a 3201918out of diet10 % Hypsarritmia 47657126218 % Slight improvement 545011363n/a86 % n/a* 612014737n/a68 % n/a* 7602920n/a66 % n/a* 8402820n/a50 % Multifocal Diet start Kcal / Ratio / Carbsxday Diet after 1 mo Kcal / Ratio / Carbsx day Diet after 3 mo Kcal / Ratio / Carbsxday Diet after 6 mo Kcal / Ratio / Carbsxday 1720 / 4:1 / 5,5 g 750 / 4:1 / 5,5 g860 / 4:1 / 5,5 g 2550 / 4:1 / 2 g700 / 3,5:1 / 5 g 810 / 3,5:1 / 5 g 3550 / 4:1 / 2,3 g580 / 4:1 / 2,4 g680 / 4:1 / 2,8 gout of diet 4450 / 3:1 / 1,8 g525 / 3:1 / 2,1 g565 / 3:1 / 2,1 g610 / 4:1 /2,3 g 5600 / 3:1 / 4 g 600 / 4:1 / 4 gn/a* 6700 / 4:1 / 4 g600 / 4:1 / 3 g450 / 4:1 / 3 gn/a* 7700 / 4:1 / 5 g n/a* 8565 / 3:1 / 3,5 g605 / 3:1 / 3,5 g617 / 4:1 / 2,5 gn/a* *n/a= non available References: ¹Cochrane Database Syst Rev 2002; ²Kossoff EH, Pediatrics 2002; ³Eun SH et al, Brain Dev 2006 Patient 1