The ketogenic diet is well tolerated and can be effective in patients with argininosuccinate lyase deficiency and refractory epilepsy

JIMD Reports

Volumn 5, Issue , 2012, Pages 127-130

  Peuscher, Rosanne ^a   Dijsselhof, Monique E ^b   Abeling, Nico G ^b   Van Rijn, Margreet ^c   Van Spronsen, Francjan J ^c   Bosch, Annet M ^a  

^a ACADEMIC MEDICAL CENTER   (Netherlands)

^b UNIVERSITY OF AMSTERDAM   (Netherlands)

^c UNIVERSITY MEDICAL CENTER GRONINGEN   (Netherlands)

Author keywords

Ammonia level; Ketogenic diet; Refractory epilepsy; Seizure frequency; Sodium benzoate

Indexed keywords

EID: 84894047384     PISSN: 21928304     EISSN: 21928312     Source Type: Book Series    
DOI: 10.1007/8904_2011_115     Document Type: Chapter

References (9)

1. Bough K (2008) Energy metabolism as part of the anticonvulsant mechanism of the ketogenic diet. Epilepsia 49(Suppl 8):s91–s93
2. Coppola G, Verrotti A, Ammendola E, Operto FF, Corte RD, Signoriello G, Pascotto A (2009) Ketogenic diet for the treatment of catastrophic epileptic encephalopathies in childhood. Eur J Paediatr Neurol 14(3):229–234
3. Erez A, Nagamani SCS, Lee B (2011) Argininosuccinate lyase deficiency Argininosuccinic aciduria and beyond. Am J Med Genet C Semin Med Genet 157:45–53
4. Ficicioglu C, Mandell R, Shih VE (2009) Argininosuccinate lyase deficiency: longterm outcome of 13 patients detected by newborn screening. Mol Genet Metab 98(3):273–277
5. Grioni D, Furlan F, Corbetta C, Barboni C, Lastrico A, Marzocchi GM, Contri M, Gamba A, Vizziello P, Parini R (2011) Epilepsy and argininosuccinic aciduria. Neuropediatrics 42(3):97–103
6. Klepper J (2008) Glucose transporter deficiency syndrome (GLUT1DS) and the ketogenic diet. Epilepsia 49(Suppl 8): s46–s49
7. Kossoff EH, Zupec-Kania BA, Amark PE et al (2009) Optimal clinical management of children receiving the ketogenic diet: recommendations of the international ketogenic diet study group. Epilepsia 50(2):304–317
8. Neal EG, Chaffe H, Schwartz RH et al (2008) The ketogenic diet for the treatment of childhood epilepsy: a randomised controlled trial. Lancet Neurol 7(6):500–506
9. Wilder RM (1921) The effect of ketonemia on the course of epilepsy. Mayo Clin Bulletin 2:307–308