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Modified atkins diet in children with developmental and epileptic encephalopathy with spike-wave activation in sleep
Shagun Singh1, Arushi Gahlot Saini2, Deepika Puri3
1Department of Telemedicine, Postgraduate Institute of Medical Education & Research, Chandigarh 160012, India.
Insights
The modified Atkins diet (MAD) showed safety for treating D/EE-SWAS but limited short-term efficacy in EEG and cognition. While seizure control improved, poor adherence presented challenges for this severe childhood epilepsy.
Area of Science:
- Pediatric Neurology
- Epileptology
- Nutritional Therapy
Background:
- Developmental and Epileptic Encephalopathy with Spike-Wave Activation in Sleep (D/EE-SWAS) is a severe childhood epilepsy linked to cognitive and language regression.
- The ketogenic diet is effective for drug-resistant epilepsy, but its role in D/EE-SWAS is understudied.
Purpose of the Study:
- To assess the efficacy and tolerability of the modified Atkins diet (MAD) in children with D/EE-SWAS.
- To evaluate MAD's impact on EEG, seizure burden, cognition, and behavior in refractory cases.
Main Methods:
- Prospective, single-arm interventional study at a pediatric neurology unit.
- Enrolled children aged 2-16 with D/EE-SWAS, high SWI, and refractory to standard therapies.
- Primary outcome: change in SWI at 12 weeks; secondary: seizure burden, cognition, language, and behavior at 24 weeks.
Main Results:
- 33% showed EEG response (>50% SWI reduction) at 12 weeks; 1 achieved complete resolution at 24 weeks.
- Seizure remission rates reached 82%, 90%, and 100% at 4, 12, and 24 weeks.
- No significant cognitive change; 60% and 40% parent-reported language and behavior improvements; 54% discontinued MAD due to poor adherence.
Conclusions:
- MAD is safe for D/EE-SWAS but shows limited short-term efficacy on EEG and cognition.
- Improvements were primarily observed in seizure control.
- Poor adherence is a significant challenge for MAD in this population.
Purpose:
Developmental and Epileptic encephalopathy with spike-wave activation in sleep (D/EE-SWAS) is a rare and severe childhood epilepsy syndrome often associated with cognitive and language regression. While the ketogenic diet has shown efficacy in drug-resistant epilepsy, its role in D/EE-SWAS remains inadequately studied. This prospective study aimed to evaluate the efficacy and tolerability of the modified Atkins diet (MAD) in children with D/EE-SWAS refractory to standard therapies.
Methods:
This was a single-arm, prospective interventional study conducted at a tertiary pediatric neurology unit from January 2022 to June 2023. Children aged 2-16 years with a confirmed diagnosis of D/EE-SWAS, with SWI ≥50 % during NREM sleep on EEG, and clinical evidence of seizure or neurodevelopmental regression despite treatment with at least two ASMs and corticosteroids were enrolled. The primary outcome was the change in SWI at 12 weeks. Secondary outcomes included seizure burden, cognitive outcomes (social quotient), and parent-reported language and behaviour changes at 24 weeks.
Results:
Twenty-two children were enrolled; 10 completed 12 weeks and five completed 24 weeks of MAD. At 12 weeks, only 33 % showed a good EEG response (>50 % SWI reduction); one child achieved complete resolution at 24 weeks. Seizure remission (including maintenance of remission in those with no clinical seizures) was observed in 82 %, 90 %, and 100 % at 4, 12, and 24 weeks, respectively. No significant change in cognitive scores was seen. However, 60 % and 40 % of parents reported improvements in language and behaviour. Adverse effects were mostly mild, and compliance was a major barrier, with 54 % discontinuing the diet before 12 weeks.
Conclusion:
The MAD was safe but not effective in the treatment of D/EE-SWAS in the short-term, with improvements only seen in seizure control. Its impact on EEG and cognition appears limited in the short-term and poor adherence poses significant challenges.
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