Which medications in the drug class Anticonvulsants are used in the treatment of Tuberous Sclerosis?

Updated: Aug 21, 2018
  • Author: David Neal Franz, MD; Chief Editor: Amy Kao, MD  more...
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Answer

Anticonvulsants

These agents prevent seizure recurrence and terminate clinical and electrical seizure activity.

Vigabatrin (Sabril)

Irreversible inhibitor of GABA transaminase, approved in the summer of 2009 by the US FDA as an orphan drug for the treatment of infantile spasms. Vigabatrin is considered to be standard of care for infants with infantile spasms (West syndrome) due to tuberous sclerosis complex. Due to the potential for irreversible peripheral visual field loss with vigabatrin, careful ophthalmologic follow-up is a condition to obtaining the drug through approved US pharmacies. Vigabatrin may also cause reversible areas of T2 hyperintensity on MRI, which, like the fluctuating signal changes seen on MRI scans of neurofibromatosis 1 patients, are of uncertain clinical significance.

Valproic acid (Depakote, Depakene, Depacon)

Considered effective first-line AED therapy against infantile spasms (West syndrome) and other seizure types seen in patients with TSC.

Lamotrigine (Lamictal)

Inhibits release of glutamate and inhibits voltage-sensitive sodium channels, leading to stabilization of neuronal membrane. Effectiveness in patients with TSC has been investigated in open-label study with promising results.

Initial dose, maintenance dose, titration intervals, and titration increments depend on concomitant medications.

Topiramate (Topamax, Qudexy XR, Trokendi XR)

Sulfamate-substituted monosaccharide with broad spectrum of antiepileptic activity that may have state-dependent sodium channel blocking action, potentiates inhibitory activity of neurotransmitter GABA. May block glutamate activity. Effectiveness in TSC has been investigated in one open-label study with promising results.

Carbamazepine (Tegretol, Carbatrol, Epitol)

Drug of choice for partial onset seizures in children and adults. Some investigators believe carbamazepine can aggravate certain seizure types in young children with TSC.


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