Efficacy and tolerability of the ketogenic diet in Dravet syndrome – Comparison with various standard antiepileptic drug regimen

Dressler, Anastasia; Trimmel-Schwahofer, Petra; Reithofer, Eva; Mühlebner, Angelika; Gröppel, Gudrun; Reiter-Fink, Edith; Benninger, Franz; Grassl, Roland; Feucht, Martha

doi:10.1016/j.eplepsyres.2014.10.014

Cited by 116 publications

(98 citation statements)

References 35 publications

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“…Good efficacy in DS was observed by several centers (65-66% responders and 0-6% seizure free), respectively (Laux and Blackford, 2013;Nabbout et al, 2011) and also by our group with 70% response and 10% seizure freedom (Dressler et al, 2015).…”

Section: Discussionmentioning

confidence: 87%

The ketogenic diet in infants – Advantages of early use

et al. 2015

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Section: Discussionmentioning

confidence: 87%

The ketogenic diet in infants – Advantages of early use

et al. 2015

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“…A metabolic approach is well established and represents a mechanistically new type of treatment for ASD. In epilepsy, KDs are as effectively anticonvulsant as the commonly-used anticonvulsant drugs [16,79–82]; KDs may prove to surpass medications in effectiveness against ASD core symptoms. Based on the present and prior research [27,51], KD feeding treats both seizures and ASD-like symptoms in the EL mouse model of comorbid epilepsy/ASD - therefore, KDs may merit special consideration as a treatment for autistic patients with this same comorbidity.…”

Section: Discussionmentioning

confidence: 99%

Ketogenic diets improve behaviors associated with autism spectrum disorder in a sex-specific manner in the EL mouse

Ruskin

Fortin

Bisnauth

et al. 2017

Physiology & Behavior

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The core symptoms of autism spectrum disorder are poorly treated with current medications. Symptoms of autism spectrum disorder are frequently comorbid with a diagnosis of epilepsy and vice versa. Medically-supervised ketogenic diets are remarkably effective nonpharmacological treatments for epilepsy, even in drug-refractory cases. There is accumulating evidence that supports the efficacy of ketogenic diets in treating the core symptoms of autism spectrum disorders in animal models as well as limited reports of benefits in patients. This study tests the behavioral effects of ketogenic diet feeding in the EL mouse, a model with behavioral characteristics of autism spectrum disorder and comorbid epilepsy. Male and female EL mice were fed control diet or one of two ketogenic diet formulas ad libitum starting at 5 weeks of age. Beginning at 8 weeks of age, diet protocols continued and performance of each group on tests of sociability and repetitive behavior was assessed. A ketogenic diet improved behavioral characteristics of autism spectrum disorder in a sex- and test-specific manner; ketogenic diet never worsened relevant behaviors. Ketogenic diet feeding improved multiple measures of sociability and reduced repetitive behavior in female mice, with limited effects in males. Additional experiments in female mice showed that a less strict, more clinically-relevant diet formula was equally effective in improving sociability and reducing repetitive behavior. Taken together these results add to the growing number of studies suggesting that ketogenic and related diets may provide significant relief from the core symptoms of autism spectrum disorder, and suggest that in some cases there may be increased efficacy in females.

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“…Lamotrigine can also cause seizure exacerbation,10 although has been reported to be beneficial in some cases 11. Effective treatment strategies include valproic acid, clobazam, topiramate, levetiracetam, fenfluramine and bromides,12, 13, 14 or dietary therapies (ketogenic or modified Atkins diet) 15. There are ongoing studies on the use of cannabidiol in children with DS 16.…”

Section: Introductionmentioning

confidence: 99%

Audit of use of stiripentol in adults with Dravet syndrome

Balestrini

Sisodiya

2016

Acta Neurol Scand

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ObjectivesThere are very few data available in the literature on the use of stiripentol in adults with Dravet syndrome (DS). DS cases are increasingly recognized in adulthood, and more children with DS now survive to adulthood. The aim of the study was to document the effectiveness and tolerability of stiripentol in adults with DS.Material and methodsWe conducted an observational clinical audit in the epilepsy service of the National Hospital for Neurology and Neurosurgery, London (UK).ResultsWe included 13 adult subjects with DS (eight females, five males). The responder (defined as more than 50% reduction in all seizure types) rate was 3/13 (23%) at 36 months. The following other outcomes were reported: seizure exacerbation (3/13, 23%), no change (3/13, 23%), less than 50% reduction in seizures (2/13, 15%), more than 50% reduction in generalized tonic‐clonic seizures but no other seizure types (1/13, 8%), undefined response (1/13, 8%). The retention rate was 62% after 1 year and 31% after 5 years. Adverse effects were reported in 7/13 (54%): the most frequent were anorexia, weight loss, unsteadiness and tiredness. Withdrawal due to adverse effects occurred in 3/13 (23%).ConclusionsCompared with previous studies on children with DS, our results show a lower responder rate and a similar tolerability profile. Stiripentol can be effective with a good tolerability profile. Our audit is small, but supports the use of stiripentol in adults with DS when first‐line treatments are ineffective or not tolerated, in keeping with published guidelines.

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Efficacy and tolerability of the ketogenic diet in Dravet syndrome – Comparison with various standard antiepileptic drug regimen

Cited by 116 publications

References 35 publications

The ketogenic diet in infants – Advantages of early use

The ketogenic diet in infants – Advantages of early use

Ketogenic diets improve behaviors associated with autism spectrum disorder in a sex-specific manner in the EL mouse

Audit of use of stiripentol in adults with Dravet syndrome

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