Zonisamide for progressive myoclonus epilepsy: long-term observations in seven patients.

Progressive myoclonic epilepsy (PME) syndromes are intractable to most antiepileptic drugs (AED). The course of these diseases, results in almost total dependency due to continuous myoclonias, repeated episodes of status epilepticus, ataxia and dementia. The need for new treatment strategies is therefore imperative. Zonisamide has previously been reported to be effective in two patients with PME. Case reports of seven patients (ages 19-42) with Unverricht-Lundborgs disease (ULD) and one Lafora Body Disease are presented. Zonisamide was given at doses of 100-600 mg/day for a period of 2 to 3 years. Concomitant AEDs were usually valproate and a benzodiazepine. Zonisamide dramatically reduced the amount of myoclonias and generalized seizures. In three of the cases, the initial dramatic effect on myoclonias wore off after 2-4 years of treatment but patients still experienced moderate efficacy for generalized tonic-clonic seizures. The dramatic reduction of stimulus sensitivity for light, touch and startle by zonisamide was sustained in all patients with ULD. Zonisamide may be a useful agent in the treatment of PME. Controlled clinical trials are warranted to further investigate the antiepileptic effects of this drug, in difficult to treat epileptic syndromes.
AuthorsM Kyllerman, E Ben-Menachem
JournalEpilepsy research (Epilepsy Res) Vol. 29 Issue 2 Pg. 109-14 (Jan 1998) ISSN: 0920-1211 [Print] NETHERLANDS
PMID9477142 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Anticonvulsants
  • Isoxazoles
  • zonisamide
  • Adult
  • Anticonvulsants (therapeutic use)
  • Epilepsies, Myoclonic (drug therapy)
  • Female
  • Humans
  • Isoxazoles (therapeutic use)
  • Male
  • Middle Aged
  • Time Factors

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research network!

Choose Username:
Verify Password: