title: Low glycemic index treatment for seizure control in Angelman syndrome: A case series from the Center for Dietary Therapy of Epilepsy at the Massachusetts General Hospital.
abstract: The low glycemic index treatment, a dietary therapy that focuses on glycemic index and reduced carbohydrate intake, has been successful in reducing seizure frequency in the general epilepsy population. Epilepsy is a common feature of Angelman syndrome and seizures are often refractory to multiple medications, especially in those with maternal deletions. Dietary therapy has become a more frequently used option for treating epilepsy, often in combination with other antiepileptic drugs, due to its efficacy and favorable side effect profile. This study aimed to assess the effectiveness of the low glycemic index treatment for seizure control in Angelman syndrome. Through a retrospective medical record review of 23 subjects who utilized the low glycemic index treatment at the Clinic and Center for Dietary Therapy of Epilepsy at the Massachusetts General Hospital, we found that the high level of seizure control and favorable side effect profile make the low glycemic index treatment a viable treatment for seizures in Angelman syndrome. The majority of subjects in our cohort experienced some level of seizure reduction after initiating the diet, 5 (22%) maintained complete seizure freedom, 10 (43%) maintained seizure freedom except in the setting of illness or non-convulsive status epilepticus, 7 (30%) had a decrease in seizure frequency, and only 1 (4%) did not have enough information to determine seizure control post-initiation. The low glycemic index treatment monotherapy was successful for some subjects in our cohort but most subjects used an antiepileptic drug concurrently. Some subjects were able to maintain the same level of seizure control on a liberalized version of the low glycemic index treatment which included a larger amount of low glycemic carbohydrates. No correlation between the level of carbohydrate restriction and level of seizure control was found. Few subjects experienced side effects and those that did found them to be mild and easily treated. The efficacy of the low glycemic index treatment and its favorable side effect profile make it an excellent alternative or supplement to antiepileptic drug therapy for the treatment of seizures in Angelman syndrome.
authors.affiliation: Angelman Syndrome Clinic and Center for Dietary Therapy of Epilepsy, Massachusetts General Hospital, Boston, MA, United States.
authors.forename: Olivia R
authors.initials: OR
authors.lastname: Grocott
authors.affiliation: Angelman Syndrome Clinic and Center for Dietary Therapy of Epilepsy, Massachusetts General Hospital, Boston, MA, United States.
authors.forename: Katherine S
authors.initials: KS
authors.lastname: Herrington
authors.affiliation: Angelman Syndrome Clinic and Center for Dietary Therapy of Epilepsy, Massachusetts General Hospital, Boston, MA, United States.
authors.forename: Heidi H
authors.initials: HH
authors.lastname: Pfeifer
authors.affiliation: Angelman Syndrome Clinic and Center for Dietary Therapy of Epilepsy, Massachusetts General Hospital, Boston, MA, United States.
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Thiele
authors.affiliation: Angelman Syndrome Clinic and Center for Dietary Therapy of Epilepsy, Massachusetts General Hospital, Boston, MA, United States. Electronic address: rthibert@mgh.harvard.edu.
authors.forename: Ronald L
authors.initials: RL
authors.lastname: Thibert
mesh_terms: D000293:Adolescent; D000328:Adult; D017204:Angelman Syndrome / Q000150:complications / Q000178:diet therapy*; D002648:Child; D002675:Child, Preschool; D055423:Diet, Ketogenic; D005260:Female; D038321:Glycemic Index; D006801:Humans; D008297:Male; D008404:Massachusetts; D012189:Retrospective Studies; D012640:Seizures / Q000178:diet therapy* / Q000209:etiology; D016896:Treatment Outcome; D055815:Young Adult
keywords: Angelman syndrome; Epilepsy; LGIT; Low glycemic index treatment; Seizures