Use of the ketogenic diet to manage refractory epilepsy in CDKL5 disorder: Experience of >100 patients

Epilepsia ◽  
2017 ◽  
Vol 58 (8) ◽  
pp. 1415-1422 ◽  
Author(s):  
Zhan Lim ◽  
Kingsley Wong ◽  
Heather E. Olson ◽  
Ann M. Bergin ◽  
Jenny Downs ◽  
...  
2017 ◽  
Vol 88 (5) ◽  
pp. e1.27-e1
Author(s):  
Zhan Lim ◽  
Jenny Downs ◽  
Kingsley Wong ◽  
Helen Leonard

Seizure ◽  
2017 ◽  
Vol 52 ◽  
pp. 1-6 ◽  
Author(s):  
Kirsty J. Martin-McGill ◽  
Michael D. Jenkinson ◽  
Catrin Tudur Smith ◽  
Anthony G. Marson

Sensors ◽  
2018 ◽  
Vol 18 (11) ◽  
pp. 3655 ◽  
Author(s):  
Andreas T. Güntner ◽  
Julia F. Kompalla ◽  
Henning Landis ◽  
S. Theodore ◽  
Bettina Geidl ◽  
...  

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.


Nutrients ◽  
2019 ◽  
Vol 11 (7) ◽  
pp. 1596 ◽  
Author(s):  
Marisa Armeno ◽  
Antonella Verini ◽  
Mariana del Pino ◽  
Maria Beatriz Araujo ◽  
Graciela Mestre ◽  
...  

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.


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

2010 ◽  
Vol 19 (4) ◽  
pp. 575-579 ◽  
Author(s):  
Pavel Klein ◽  
Jaromir Janousek ◽  
Arkady Barber ◽  
Randi Weissberger

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