Embed Size (px)
Citation preview
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
rReferences
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
[email protected]@vestamt.dk
Current Age(mo)
Gen-der
Diagnosis Epilepsy
onset(mo)
Diet
start(mo)
EEG
before KD
1 19 FCryptogenic West
Syndrome 4 10 Hypsarritmia
2 22 MSymptomatic West
Syndrome
(Deletion 22q13)9 16 Hypsarritmia
3 18 MSymptomatic West
Syndrome
(Trisomy 21-Down S.)5 12
Hypsarritmia
4 18 FEarly Myoclonic Encephalopathy 6 12 Suppression-burst
5 18 MCryptogenic West
Syndrome 4 13 Multifocal
6 16 MSymptomatic West Syndrome (Multiple
cerebral malformations)2 12 Multifocal
7 25 MCryptogenic West
Syndrome 3 21 Hypsarritmia
8 18 MCryptogenic 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
reduction
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
