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Children with Tuberous Sclerosis Complex (TSC) on Ketogenic diet therapies: Outcome of a retrospective cohort

Objective: Tuberous sclerosis complex (TSC) is a neurocutaneous syndrome often associated with intractable epilepsy. Though vigabatrin is the drug of choice for epileptic spasms associated with this condition it usually progresses to drug refractory epilepsy. Ketogenic diet has been proven as an effective treatment modality for drug refractory epilepsies. We studied the the role of ketogenic diet therapies and their efficacy in TSC.

Methods: Cases with Tuberous Sclerosis complex with drug refractory epilepsy aged 2-15 years on ketogenic diet therapies, at a tertiary care teaching hospital in north India between Jan 2017 to Dec 2021 were retrospectively reviewed.

Results: Thirteen children were identified, two (15.3%) were on classical KD, seven (53.8%) were on Modified Atkin Diet (MAD), five (38.4 %) were on low glycemic index therapy (LGIT). Out of these 13 patients, 2 (15.3%) achieved complete seizure freedom, 6 (45.9%) had  50% seizure reduction at one year of follow up. There was no change in seizure frequency noted in 5 (38.4%) patients. None of the patient experienced increase in seizure frequency.

Conclusions: Ketogenic diet therapies show promising results in this cohort. A trial of ketogenic diet therapies should be considered in a child diagnosed as Tuberous sclerosis complex with drug refractory epilepsy.
Keywords: Tuberous Sclerosis, Ketogenic diet, Modified Atkin Diet, Low Glycemic therapy