An unfortunate challenge: Ketogenic diet for the treatment of Lennox–Gastaut syndrome in tyrosinemia type 1

doi:10.1016/j.ejpn.2016.02.015

European Journal of Paediatric Neurology

Volume 20, Issue 4, July 2016, Pages 674-677

https://doi.org/10.1016/j.ejpn.2016.02.015 Get rights and content

Highlights

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Ketogenic diet can be conducted in a patient with an additional dietary requirement.
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Ketogenic diet should not be considered as a last chance treatment even in more complex situation.
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Ketogenic diet can be helpful in the management of Lennox–Gastaut syndrome.

Abstract

The ketogenic diet is an evidence-based treatment for resistant epilepsy including Lennox–Gastaut syndrome. This diet is based on low carbohydrate-high fat intakes. Dietary treatment is also therapeutic for inborn errors of metabolism such as aminoacdiopathies. We report a child with both Lennox–Gastaut syndrome and tyrosinemia type 1. This epilepsy syndrome resulted form a porencephalic cyst secondary to brain abscesses that occurred during the management of malnutrition due to untreated tyrosinemia type 1. We used a ketogenic diet as treatment for Lennox–Gastaut syndrome taking into account dietary requirements for tyrosinemia type 1. The patient was transiently responder during a 6-month period. This report illustrates that ketogenic diet remains a therapeutic option even when additional dietary requirements are needed.

Section snippets

Case report

A 17-month-old girl was brought to our attention for multiple bleeding (epistaxis, otorrhagia, rectorragie) secondary to liver failure with a previous history of growth and development delays.

She was born from non-consanguineous parents, the last of 4 siblings, without prenatal or birth significant history. She had delayed developmental milestones, demonstrating head control at 5 months of age, sitting with support at 9 months, walking and language not yet acquired.

On examination she had

Discussion

This report illustrates that KD could be used taken into account an additional diet requirement for a metabolic disorder. Because of the pharmacoresistance of a Lennox–Gastaut syndrome in a patient with tyrosinemia type 1, we started tailored KD. We used a KD with restricted phenylalanine and tyrosine intakes. Our aim was to maintain plasma tyrosine levels below 400 μmol/l. This treatment was proposed after the failure of various AED combinations with a particular attention with AEDs known to

Conflict of interests

The authors have stated that they had no interests which might be perceived as posing a conflict or bias.

References (11)

A. Arzimanoglou et al.
Lennox–gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology
Lancet Neurol
(2009)
E.G. Neal et al.
The ketogenic diet for the treatment of childhood epilepsy: a randomised controlled trial
Lancet Neurol
(2008)
N.M. Aylward et al.
The ketogenic diet for the treatment of myoclonic astatic epilepsy in a child with type 1 diabetes mellitus
Can J Diabetes
(2014)
J. Klepper
Glut1 deficiency syndrome in clinical practice
Epilepsy Res
(2012)
E.C. Hancock et al.
Treatment of lennox–gastaut syndrome
Cochrane Database Syst Rev
(2013)

There are more references available in the full text version of this article.

Cited by (4)

Ketogenic diet for infants with epilepsy: A literature review
2020, Epilepsy and Behavior
Citation Excerpt :
We analyzed four RCTs [39–42], among which one studied the economic evaluation of KD versus usual care in children and adolescents with intractable epilepsy; one analyzed patients with refractory epilepsy aged 1–18 years; one studied the efficacy of the classic KD compared with modified Atkins diets in the refractory childhood epilepsy, and another demonstrated a positive impact of the KD on behavioral and cognitive functioning in children and adolescents with refractory epilepsy. Twelve publications were clinical cases [43–54]: among these, one paper studied the use of KD as a broad-spectrum treatment for super-refractory pediatric status epilepticus in patients aged 9 weeks to 13,5 years. Among the 4 clinical reviews [55–58] we obtained from PubMed, 1/4 showed the results of a project group of 5 experts, performed in 2015, in order to create a consensus statement regarding the clinical management of the KD in infancy; 1/4 analyzed the cost-effectiveness of the KD and VNS for the treatment of children with intractable epilepsy; 1/4 performed the analysis of all nonpharmacological medical treatments in pediatric epilepsies, and the last offered an overview of various diets, comparing their efficacy.
The ketogenic diet (KD) is an established, nonpharmacological treatment for drug-resistant epilepsy (DRE). Actually, KD and its variants have been shown to be elective and resolute for patients with glucose transporter type 1 (GLUT1) deficiency. The aim of this review was to study the use of KD and its variants in infancy, including the neonatal age, and demonstrate the safety and efficacy of this treatment in patients with the age of 0–23 months affected by DRE already subjected to pharmacological approach attempts.
A literature search was conducted using PubMed as the medical database source. We used the age limit of 0–23 months, and we considered only articles published between the years 2015 and 2018, in light of increasing interest worldwide in the use of KD and its variants to manage DRE. We included 52 publications: 1 Cochrane study, 22 retrospective studies, 9 prospective studies, 4 randomized controlled trials (RCTs), 12 clinical cases, and 4 clinical reviews.
Literature data showed that KD and its variants are safe and useful in patients with the age of 0–23 months with DRE. Classical KD is of first choice in the treatment of GLUT1 deficiency. Earlier introduction of KD in GLUT1 promises a better outcome and a decrease in seizure frequency in these patients.
Ketogenic Diets in the Management of Lennox-Gastaut Syndrome—Review of Literature
2022, Nutrients
Ketogenic dietary therapy: principles of implementation and application for inborn disorders of metabolism
2020, Monatsschrift fur Kinderheilkunde
Ketogenic diet for refractory childhood epilepsy: Beyond seizures control, the experience of a Portuguese pediatric centre
2019, Acta Medica Portuguesa

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Case StudyAn unfortunate challenge: Ketogenic diet for the treatment of Lennox–Gastaut syndrome in tyrosinemia type 1

Highlights

Abstract

Section snippets

Case report

Discussion

Conflict of interests

Lancet Neurol

Lancet Neurol

Can J Diabetes

Epilepsy Res

Treatment of lennox–gastaut syndrome

Cochrane Database Syst Rev

Case Study
An unfortunate challenge: Ketogenic diet for the treatment of Lennox–Gastaut syndrome in tyrosinemia type 1