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Usefulness of Ketogenic diet, Cannabidiol, and Quinidine in KCNT1 epilepsies

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Created On: 05/12/2024

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Key take away

Ketogenic diet, Cannabidiol, and Quinidine could be effective treatments for drug-resistant seizures linked to KCNT1 gene mutations, offering hope where conventional medications often fail.

Background

KCNT1-related epilepsies include three major types: [1] Epilepsy of infancy with migrating focal seizures (EIMFS), [2] Autosomal dominant or sporadic sleep-related hypermotor epilepsy (AD SHE), and [3] Various developmental and epileptic encephalopathies (DEE).

A systematic review sought to determine the effectiveness of various treatments for patients with KCNT1-related epilepsies, highlighting their potential benefits and addressing the urgent need for alternative treatments due to drug-resistant seizures.

Method

The study conducted a thorough review of studies from PubMed and Embase, focusing on the effectiveness of the anti-seizure medications, ketogenic diet (KD, a medical diet high in fat, with adequate protein and very low carbohydrates), Cannabidiol (CBD), and Quinidine (QUIN) for treating epilepsies. Researchers evaluated how these treatments impacted seizure frequency, intensity, and quality of life across the three patient groups.

Result

In 43 studies involving 197 patients, the following results were observed:

EIMFS Patients (135 patients, 32 studies): KD was beneficial for 62.5% (25/40), CBD for 50% (6/12), and QUIN for 44.6% (25/56).
AD SHE Patients (32 patients, 10 studies): Only one KD trial was reported that showed no benefit, and QUIN depicted no reported benefits in 8 patients.
DEE Patients (30 patients, 10 studies): KD benefited 4/7, CBD helped 1/2, and QUIN was effective for 6/9.

Additionally, conventional anti-seizure medications were beneficial in just 5%-25% of cases.

Conclusion

KD, CBD, and QUIN offer promising alternatives for managing KCNT1-related epilepsies, particularly where conventional medications fall short. However, more research is needed to fine-tune therapeutic modalities and determine which patients will benefit most.