scholarly journals Ketogenic diet effects on 52 children with pharmacoresistant epileptic encephalopathy: A clinical prospective study

2018 ◽  
Vol 8 (5) ◽  
pp. e00973 ◽  
Author(s):  
Qiong Wu ◽  
Hua Wang ◽  
Yu Ying Fan ◽  
Jun Mei Zhang ◽  
Xue Yan Liu ◽  
...  
2003 ◽  
Vol 103 (6) ◽  
pp. 707-712 ◽  
Author(s):  
Yeou-Mei Christiana Liu ◽  
Shelley Williams ◽  
Carlota Basualdo-Hammond ◽  
Derek Stephens ◽  
Rosalind Curtis

2021 ◽  
Vol 23 (5) ◽  
pp. 739-743
Author(s):  
Silvia Schiavoni ◽  
Carlotta Spagnoli ◽  
Susanna Rizzi ◽  
Grazia Gabriella Salerno ◽  
Daniele Frattini ◽  
...  

Seizure ◽  
2018 ◽  
Vol 60 ◽  
pp. 144-148 ◽  
Author(s):  
Ni Yan ◽  
Wang Xin-Hua ◽  
Zhang Lin-Mei ◽  
Chai Yi-Ming ◽  
Li Wen-Hui ◽  
...  

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

2018 ◽  
Vol 07 (04) ◽  
pp. 148-151 ◽  
Author(s):  
Fahad A. Bashiri ◽  
Abrar Hudairi ◽  
Malak Al Ghamdi ◽  
Adel A. Mahmoud

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.


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

2021 ◽  
Vol 62 (4) ◽  
pp. 370
Author(s):  
Xiaoyu Tian ◽  
Yange Zhang ◽  
Jinhong Zhang ◽  
Yan Lu ◽  
Xinyi Men ◽  
...  

2015 ◽  
Vol 51 ◽  
pp. 261-266 ◽  
Author(s):  
Danielle A.J.E. Lambrechts ◽  
Reina J.A. de Kinderen ◽  
Hans S.H. Vles ◽  
Anton J. de Louw ◽  
Albert P. Aldenkamp ◽  
...  

2017 ◽  
Vol 06 (03) ◽  
pp. 149-155
Author(s):  
Raffaele Falsaperla ◽  
Robinson Gutierrez ◽  
Gabriela Valenzuela ◽  
Piero Pavone ◽  
Sebastian Fortini ◽  
...  

Objective We evaluated the electroclinical features, etiology, treatment, and outcome of 16 patients with single-epileptic spasms (ESs) with or without hypsarrhythmia (WoH). Methods Nine boys and seven girls had single-ESs. ESs were considered as single epileptic spasm variants when no other spasm occurred for 1 minute before and after each spasm. Age at the onset of ESs was between 2 and 84 months, with a mean age of 11 months. Results We recognized a group of 15 patients with single-ESs as the main type of seizure; 6 patients with WoH and 9 patients with hypsarrhythmia, respectively. Nine of these 15 patients had other types of seizures before the onset of single-ESs, and 12 patients had other types of seizures during the period in which the ESs occurred. Nine of 15 patients had a structural and seven had an unknown etiology. In 10 cases, the ESs were refractory to antiepileptic drugs, while 4 patients responded well to adrenocorticotropic hormone (ACTH), 1 to pyridoxine, and 2 to the ketogenic diet (KD). The remaining patient (patient.16) had single-ESs and electroclinical features of Lennox–Gastaut syndrome (LGS). Conclusion In this article, we present a series of infants who had daily single-ESs with or WoH. Those with single-ESs with hypsarrhythmia evolved to an epileptic encephalopathy. Video-electroencephalogram (EEG) and polygraphic-EEG recordings are crucial to identify the single-ESs.


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