Short communicationLow glycemic index treatment for epilepsy in tuberous sclerosis complex
Introduction
Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder characterized by lesions of the central nervous system, kidney, lungs, heart, and skin (Crino et al., 2006). The loss or reduction of TSC1/2, tumor suppressor kinase, leads to the hamartomatous pathology of TSC. Eighty-five percent of individuals with TSC develop epilepsy, 62% of whom are medically refractory (Chu-Shore et al., 2010).
The low glycemic index treatment (LGIT) was developed as a liberalized alternative to the ketogenic diet for seizure management (Pfeifer and Thiele, 2005). This dietary treatment implements a ratio of 1:1 grams of fat to grams of carbohydrates and protein and limits the daily carbohydrate intake to ∼10% (40–60 g) of total calories. Importantly, in the LGIT, foods are also limited to those with a glycemic index of less than 50 relative to glucose (Pfeifer and Thiele, 2005). Previous research has demonstrated the success of the LGIT as an epilepsy therapy (Muzykewicz et al., 2009). Additionally, the ketogenic diet (KD) has been proven highly effective in the treatment of the often medically refractory seizures associated with TSC (Kossoff et al., 2005). In this study, we evaluated the clinical utility and strength of the LGIT for patients with TSC-related epilepsy.
Section snippets
Methods
We performed a retrospective chart review of all patients who initiated the LGIT at the Herscot Center for TSC at Massachusetts General Hospital between January 2005 and January 2011. Dietary treatment was delivered according to the LGIT protocol (Muzykewicz et al., 2009). Data were collected on patient age, sex, and genetic mutation. Each patient's seizure history was reported including their age at seizure onset, as well as their current and past epilepsy treatments. Change in seizure
Results
Fifteen patients with TSC initiated the LGIT. Patients ranged in age from 1.8 to 20.9 years old (average of 8.5 years) with seven males and eight females. Two patients had a confirmed mutation in TSC1 and six had a confirmed mutation in TSC2. The average age at seizure onset was 8.2 months. This cohort had tried an average of 5.8 AEDs with incomplete seizure control prior to dietary therapy, and 14/15 (93%) patients were medically refractory. None of the patients started a new AED during their
Discussion
TSC-related epilepsy is responsive to LGIT dietary therapy. This treatment carries low risk to patients as there have been no significant side effects observed (Pfeifer and Thiele, 2005, Pfeifer et al., 2008, Muzykewicz et al., 2009). A greater percentage of patients responded to the LGIT in this TSC population compared to the pediatric epilepsy population previously studied (47% vs. 25%, >50% seizure frequency reduction at 6 months) (Muzykewicz et al., 2009). When comparing the efficacy of the
Conflicts of interest
None of the authors have any conflict of interest to disclose.
Acknowledgements
This study was supported by the Herscot Center for Tuberous Sclerosis Complex, the Tuberous Sclerosis Alliance, and NIH/NINDS P01 NS024279.
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2023, Diet and Nutrition in Neurological DisordersUpdated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Recommendations
2021, Pediatric NeurologyCitation Excerpt :Clinicians should be aware that both everolimus107 and cannabidiol108 have important drug interactions with other antiseizure medications, including with each other.109,110 Dietary interventions, including a ketogenic diet111,112 or low-glycemic index treatment,113 may be an effective nonpharmacological therapy for patients with TSC with intractable epilepsy, including infantile spasms refractory to vigabatrin and hormonal therapies. Epilepsy surgery should be considered for patients with refractory TSC, particularly after failing three medications.114-116