Progressive Myoclonic Epilepsy (PME): Response of Seizures to Zonisamide
Abstract number :
1.167
Submission category :
Year :
2001
Submission ID :
799
Source :
www.aesnet.org
Presentation date :
12/1/2001 12:00:00 AM
Published date :
Dec 1, 2001, 06:00 AM
Authors :
S.C. Reigle, BS, Neurology, Children[ssquote]s, Washington, DC; W.D. Gaillard, MD, Neurology, Children[ssquote]s, Washington, DC; P.L. Pearl, MD, Neurology, Children[ssquote]s, Washington, DC; S.L. Weinstein, MD, Neurology, Children[ssquote]s, Washington,
RATIONALE: Zonisamide (Zonegran[tm]) is a broad-spectrum antiepilepsy drug (AED) with both sodium and calcium channel blocking activities, approved for treatment of partial seizures. Efficacy in other seizure types has not been established. Progressive Myoclonic Epilepsies (PME) are a group of disorders characterized by myoclonic and mixed seizures and progressive neurologic decline. Myoclonic seizures, are refractory to most medications. We assessed the response of Zonisamide (ZNS) and various seizure types in children with PME.
METHODS: Eight patients (5 males, 3 females, mean age at diagnosis: 5.5 years), on an average of two baseline AEDs (3 on VPA, 2 on TPM, and 1 on Felbamate, Phenobarbital, Dilantin, Nitrazepam, VNS, and Ketogenic Diet respectively), were started on a daily dosage of 0.4 mg/kg/day of open-labeled, un-blinded, adjunctive ZNS, and titrated over an 8 week period. All patients had seizures and [dsquote]developmental delay[dsquote]. Seizure types at time of initiation were: myoclonic (7/8); tonic/tonic-clonic (4/8); partial (2/8); absence (2/8); and [dsquote]drops[dsquote] (4/8).
RESULTS: Parents maintained a daily seizure log. Four children had optimal response between 5-8 mg/kg/day, two at 1-4 mg/kg/day, and one at 10mg/kg/day (mean dosage was 5.7 mg/kg/day), with a mean follow-up of 7.25 months (range 3-10 months), and resulted in a reduction of various seizure types. Five out of 7 children had a [gt]90% reduction of myoclonic seizures, half had [gt]90% reduction of both tonic-clonic and [dsquote]drop[dsquote] seizures, and all patients with partial and absence seizures had [gt]90% reduction in seizure activity. One patient had an increase in seizure activity, which resulted in almost doubling his tonic seizures. Four patients with [gt]90% reduction of all of their seizure types were on an average of 2 anticonvulsants (range 1-3), before adding ZNS.
CONCLUSIONS: ZNS as adjunctive therapy was effective in reducing various seizure types in patients diagnosed with PME in this short term follow-up study.
Support: Elan Pharmaceuticals.
Disclosure: Grant - Elan Pharmaceuticals, Phase III Clinical Trials, Dr. Conry, PI. Consulting - Elan Pharmaceuticals, Dr. Conry.