TY - JOUR
T1 - Ketogenic Diet Therapy for Epilepsy Associated With Aicardi Syndrome
AU - Sanchez, Miguel A.Ramirez
AU - Cervenka, Mackenzie C.
AU - Bessone, Stacey K.
AU - Kossoff, Eric H.
N1 - Funding Information:
The authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: EHK is on the scientific advisory board of Bloom Science, and Atkins Nutritionals and is a consultant to Vitaflo and Nutricia. SKB is a consultant to Nutricia. MCC has received support from the Epilepsy Foundation, the School of Medicine, Johns Hopkins University Clinician Scientist Award, Nutricia, Vitaflo, the Carson Harris Foundation, Owens Family Foundation, Elaine Freeman and Johns Hopkins University Department of Neurosurgery, NIH (NINDS R01NS075020), and Army Research Laboratory. None of the other authors have any financial disclosures.
Publisher Copyright:
© The Author(s) 2021.
PY - 2021/10
Y1 - 2021/10
N2 - Introduction: Aicardi syndrome is a rare neurodevelopmental disorder associated with epilepsy in females. Ketogenic diet therapy represents a possible nonpharmacologic treatment in Aicardi syndrome patients. Methods: All patients with Aicardi syndrome seen at Johns Hopkins Hospital (Baltimore, MD) and Johns Hopkins All Children’s Hospital (St Petersburg, FL) treated with ketogenic diet therapy since 1994 were evaluated retrospectively. Results: Fifteen patients, ages 4 months to 34 years, were identified. Ten (67%) patients experienced a ≥50% seizure reduction after 3 months, with 3 (20%) having a ≥90% reduction. Only 1 patient was seizure-free for a short period of time. The number of drugs tried prior to ketogenic diet therapy initiation was correlated with ≥50% seizure reduction at 3 months, 5.8 vs 2.6 in responders versus nonresponders (P =.01). In addition, the mean number of drugs actively received also correlated, 3.0 vs 1.2, P =.005. Ketogenic diet therapy was slightly more successful in those without infantile spasms, 78% vs 50%, P =.33. Conclusion: Ketogenic diet therapy was helpful in Aicardi syndrome, although seizure freedom was rare. It was especially helpful for those who were more drug-resistant and did not have infantile spasms at ketogenic diet therapy onset.
AB - Introduction: Aicardi syndrome is a rare neurodevelopmental disorder associated with epilepsy in females. Ketogenic diet therapy represents a possible nonpharmacologic treatment in Aicardi syndrome patients. Methods: All patients with Aicardi syndrome seen at Johns Hopkins Hospital (Baltimore, MD) and Johns Hopkins All Children’s Hospital (St Petersburg, FL) treated with ketogenic diet therapy since 1994 were evaluated retrospectively. Results: Fifteen patients, ages 4 months to 34 years, were identified. Ten (67%) patients experienced a ≥50% seizure reduction after 3 months, with 3 (20%) having a ≥90% reduction. Only 1 patient was seizure-free for a short period of time. The number of drugs tried prior to ketogenic diet therapy initiation was correlated with ≥50% seizure reduction at 3 months, 5.8 vs 2.6 in responders versus nonresponders (P =.01). In addition, the mean number of drugs actively received also correlated, 3.0 vs 1.2, P =.005. Ketogenic diet therapy was slightly more successful in those without infantile spasms, 78% vs 50%, P =.33. Conclusion: Ketogenic diet therapy was helpful in Aicardi syndrome, although seizure freedom was rare. It was especially helpful for those who were more drug-resistant and did not have infantile spasms at ketogenic diet therapy onset.
KW - Aicardi
KW - diet
KW - ketogenic
KW - ketosis
KW - seizures
UR - http://www.scopus.com/inward/record.url?scp=85109760011&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85109760011&partnerID=8YFLogxK
U2 - 10.1177/08830738211023335
DO - 10.1177/08830738211023335
M3 - Article
C2 - 34240629
AN - SCOPUS:85109760011
SN - 0883-0738
VL - 36
SP - 1007
EP - 1010
JO - Journal of child neurology
JF - Journal of child neurology
IS - 11
ER -