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 medically refractory IS •The clinical response is positive (measured by redu cessation of spasms) in most infants (7/8) and the tolerability good (8/8) •Reduction (6/8)/ interruption (2/8) of AEDs is an a positive effect •We can not conclude on EEG improvement in this stud [email protected] [email protected] Cur ren t Age (mo) Gen - der Diagnosis Epileps y onset (mo) Diet start (mo) EEG before KD 1 19 F Cryptogenic West Syndrome 4 10 Hypsarritmia 2 22 M Symptomatic West Syndrome (Deletion 22q13) 9 16 Hypsarritmia 3 18 M Symptomatic West Syndrome (Trisomy 21-Down S.) 5 12 Hypsarritmia 4 18 F Early Myoclonic Encephalopathy 6 12 Suppression-burst 5 18 M Cryptogenic West Syndrome 4 13 Multifocal 6 16 M Symptomatic West Syndrome (Multiple cerebral malformations) 2 12 Multifocal 7 25 M Cryptogenic West Syndrome 3 21 Hypsarritmia 8 18 M Cryptogenic West Syndrome 3 15 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 reductio n EEG at follow-up 1 80 18 11 10 88 % Multifocal 2 210 0 0 0 100 % n/a 3 20 19 18 out of diet 10 % Hypsarritmia 4 76 57 12 62 18 % Slight improvement 5 450 113 63 n/a 86 % n/a* 6 120 147 37 n/a 68 % n/a* 7 60 29 20 n/a 66 % n/a* 8 40 28 20 n/a 50 % 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 1 720 / 4:1 / 5,5 g 720 / 4:1 / 5,5 g 750 / 4:1 / 5,5 g 860 / 4:1 / 5,5 g 2 550 / 4:1 / 2 g 700 / 3,5:1 / 5 g 700 / 3,5:1 / 5 g 810 / 3,5:1 / 5 g 3 550 / 4:1 / 2,3 g 580 / 4:1 / 2,4 g 680 / 4:1 / 2,8 g out of diet 4 450 / 3:1 / 1,8 g 525 / 3:1 / 2,1 g 565 / 3:1 / 2,1 g 610 / 4:1 /2,3 g 5 600 / 3:1 / 4 g 600 / 3:1 / 4 g 600 / 4:1 / 4 g n/a* 6 700 / 4:1 / 4 g 600 / 4:1 / 3 g 450 / 4:1 / 3 g n/a* 7 700 / 4:1 / 5 g 700 / 4:1 / 5 g 700 / 4:1 / 5 g n/a* 8 565 / 3:1 / 3,5 g 605 / 3:1 / 3,5 g 617 / 4:1 / 2,5 g n/a* *n/a= non available References: ¹Cochrane Database Syst Rev 2002; ²Kossoff EH, Pediatrics 2002; ³Eun SH et al, Brain Dev 2006 Patient 1