Neurologic Manifestations of Xeroderma Pigmentosum Medication

Updated: Mar 19, 2019
  • Author: Peter Hedera, MD, PhD; Chief Editor: Amy Kao, MD  more...
  • Print

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications.



Class Summary

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

Carbamazepine (Tegretol, Carbatrol, Epitol)

Treats complex partial seizures. Appears to act by reducing polysynaptic responses and blocking posttetanic potentiation. Major mechanism of action is to reduce sustained high-frequency repetitive neural firing.

Phenytoin (Dilantin)

May act in motor cortex where may inhibit spread of seizure activity. Activity of brain stem centers responsible for tonic phase of grand mal seizures also may be inhibited.

Dose should be individualized. Administer larger dose before retiring if dose cannot be divided equally. Phosphorylated formulation, fosphenytoin, available for parenteral use and may be given IM or IV.

Valproic acid (Depakote, Depakene, Depacon)

Chemically unrelated to other drugs that treat seizure disorders. Although mechanism of action not established, activity may be related to increased brain levels of GABA, or enhanced GABA action.

Also may potentiate postsynaptic GABA responses, affect potassium channel, or have direct membrane-stabilizing effect.

For conversion to monotherapy, concomitant AED dosage ordinarily can be reduced by approximately 25% every 2 wk. This reduction may start at initiation of therapy or be delayed by 1-2 wk if concern that seizures may occur with reduction. Monitor patients closely during this period for increased seizure frequency.

As adjunctive therapy, divalproex sodium may be added to patient's regimen at 10-15 mg/kg/d. May increase by 5-10 mg/kg/wk to achieve optimal clinical response. Ordinarily, optimal clinical response achieved at daily doses < 60 mg/kg/d.