scholarly journals The ketogenic diet is effective for refractory epilepsy associated with acquired structural epileptic encephalopathy

2018 ◽  
Vol 60 (7) ◽  
pp. 718-723 ◽  
Author(s):  
Mel Michel Villaluz ◽  
Lysa Boissé Lomax ◽  
Trupti Jadhav ◽  
J Helen Cross ◽  
Ingrid E Scheffer
Keyword(s):  
Ketogenic Diet ◽  
Refractory Epilepsy ◽  
Epileptic Encephalopathy

scholarly journals The modified ketogenic diet for adults with refractory epilepsy: An evaluation of a set up service

Seizure ◽  
2017 ◽  
Vol 52 ◽  
pp. 1-6 ◽  
Author(s):  
Kirsty J. Martin-McGill ◽  
Michael D. Jenkinson ◽  
Catrin Tudur Smith ◽  
Anthony G. Marson
Keyword(s):  
Ketogenic Diet ◽  
Refractory Epilepsy ◽  
Set Up ◽  
Modified Ketogenic Diet

Beneficial effects of the ketogenic diet on drug-resistant epileptic encephalopathy associated with a de novo NBEA pathogenic variant

2021 ◽  
Vol 23 (5) ◽  
pp. 739-743
Author(s):  
Silvia Schiavoni ◽  
Carlotta Spagnoli ◽  
Susanna Rizzi ◽  
Grazia Gabriella Salerno ◽  
Daniele Frattini ◽  
...  
Keyword(s):  
Ketogenic Diet ◽  
De Novo ◽  
Epileptic Encephalopathy ◽  
Pathogenic Variant ◽  
Drug Resistant ◽  
Beneficial Effects

scholarly journals Guiding Ketogenic Diet with Breath Acetone Sensors

Sensors ◽  
2018 ◽  
Vol 18 (11) ◽  
pp. 3655 ◽  
Author(s):  
Andreas T. Güntner ◽  
Julia F. Kompalla ◽  
Henning Landis ◽  
S. Theodore ◽  
Bettina Geidl ◽  
...  
Keyword(s):  
Ketogenic Diet ◽  
Standard Treatment ◽  
Refractory Epilepsy ◽  
Neurological Diseases ◽  
Endurance Performance ◽  
Response Characteristics ◽  
Si Doped ◽  
Breath Acetone ◽  
Relevant Range ◽  
Good Agreement

Ketogenic diet (KD; high fat, low carb) is a standard treatment for obesity, neurological diseases (e.g., refractory epilepsy) and a promising method for athletes to improve their endurance performance. Therein, the level of ketosis must be regulated tightly to ensure an effective therapy. Here, we introduce a compact and inexpensive breath sensor to monitor ketosis online and non-invasively. The sensor consists of Si-doped WO3 nanoparticles that detect breath acetone selectively with non-linear response characteristics in the relevant range of 1 to 66 ppm, as identified by mass spectrometry. When tested on eleven subjects (five women and six men) undergoing a 36-h KD based on the Johns Hopkins protocol, this sensor clearly recognizes the onset and progression of ketosis. This is in good agreement to capillary blood β-hydroxybutyrate (BOHB) measurements. Despite similar dieting conditions, strong inter-subject differences in ketosis dynamics were observed and correctly identified by the sensor. These even included breath acetone patterns that could be linked to low tolerance to that diet. As a result, this portable breath sensor represents an easily applicable and reliable technology to monitor KD, possibly during medical treatment of epilepsy and weight loss.

ATP1A3 -related epileptic encephalopathy responding to ketogenic diet

2018 ◽  
Vol 40 (5) ◽  
pp. 433-438 ◽  
Author(s):  
Tommaso Schirinzi ◽  
Federica Graziola ◽  
Raffaella Cusmai ◽  
Lucia Fusco ◽  
Francesco Nicita ◽  
...  
Keyword(s):  
Ketogenic Diet ◽  
Epileptic Encephalopathy

A Case of Neonatal Epileptic Encephalopathy due to SCN2A Mutation Responsive to a Ketogenic Diet

2018 ◽  
Vol 07 (04) ◽  
pp. 148-151 ◽  
Author(s):  
Fahad A. Bashiri ◽  
Abrar Hudairi ◽  
Malak Al Ghamdi ◽  
Adel A. Mahmoud
Keyword(s):  
Ketogenic Diet ◽  
Treatment Modality ◽  
Genetic Diagnosis ◽  
Febrile Seizures ◽  
Epileptic Encephalopathy ◽  
Intractable Seizures ◽  
Ohtahara Syndrome ◽  
Newborn Girl ◽  
Generalized Epilepsy ◽  
Febrile Seizures Plus

AbstractNeonatal seizures may have multiple causes including metabolic and genetic etiologies. If a genetic diagnosis is known, it can guide the physician to choose the most appropriate treatment modality. SCN2A mutation is a rare cause of epileptic encephalopathy in the neonatal age group. It has a wide phenotypic variation, ranging from benign familial epilepsy to a malignant form of epilepsy. This mutation has been associated with Ohtahara syndrome, migrating focal seizures of infancy, West syndrome, Lennox–Gastaut syndrome, and generalized epilepsy with febrile seizures plus. We present the case of a newborn girl who presented with multiple types of seizures, starting at the age of 3 days. Our initial investigations were not able to identify the etiology of her intractable seizures. Whole exome sequencing confirmed an SCN2A mutation. Various antiepileptic drugs (AEDs), including phenobarbitone, phenytoin, levetiracetam, topiramate, vigabatrin, carbamazepine, clonazepam, and mexiletine, were tried. However, none provided an optimal response. She ultimately showed a dramatic response to the ketogenic diet (KD). This report highlights the effectiveness of the KD as a treatment modality for SCN2A mutation-related epileptic encephalopathy, particularly when seizures are intractable and unresponsive to conventional AEDs.

scholarly journals A Prospective Study on Changes in Nutritional Status and Growth Following Two Years of Ketogenic Diet (KD) Therapy in Children with Refractory Epilepsy

Nutrients ◽  
2019 ◽  
Vol 11 (7) ◽  
pp. 1596 ◽  
Author(s):  
Marisa Armeno ◽  
Antonella Verini ◽  
Mariana del Pino ◽  
Maria Beatriz Araujo ◽  
Graciela Mestre ◽  
...  
Keyword(s):  
Nutritional Status ◽  
Prospective Study ◽  
Ketogenic Diet ◽  
Refractory Epilepsy ◽  
Growth Monitoring ◽  
Male Predominance ◽  
History Of ◽  
A Prospective Study ◽  
Growth Deceleration

Introduction: Epilepsy is a neurological disorder characterized by an increased susceptibility to seizures. The ketogenic diet (KD) is currently the most important alternative non-pharmacological treatment. Despite its long history of clinical use, it is not clear how this diet affects longitudinal growth in children. Methods: A prospective study was designed to evaluate growth and nutritional status in 45 children on KD. Growth was assessed by measuring weight, height, and body mass index (BMI). Standard deviation scores (SDS) were calculated for all measurement parameters at KD initiation and at a two-year follow-up. Results: Overall, 45 patients who completed 24 months on KD were enrolled. Median age was 6.6 years (0.8 to 17.3), with a male predominance (n = 23); 74% of the 45 patients were responders on seizure reduction at three months; 26% of patients were non-responders. In our study, using −1 SDS as a cut-off point, growth deceleration was observed in 9% (n: 4) of the patients; however, the nutritional status was maintained or even improved. No correlation with age, sex, or ambulatory status was found. Conclusions: The nutritional follow-up of these patients was helpful to improve overweight and thinness but could not avoid growth deceleration in some of them. These findings confirm that children with refractory epilepsy on KD treatment require careful growth monitoring.

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