Unverricht-Lundborg disease (EPM1) is the most common form of progressive myoclonus epilepsy. Symptoms stabilize in adulthood but chronic myoclonus frequently remains the primary cause of disability. We carried out a pragmatic, pilot, open-label trial to evaluate the tolerability and the antimyoclonic efficacy of Zonisamide (ZNS) in 12 patients with EPM1. The ZNS target dose was 300-500 mg/day. ZNS add-on was associated with clinical improvement of myoclonus and lowering of mean scores on all Unified Myoclonus Rating Scale (UMRS) sections except section 6 (physician's assessment of disability). ZNS was generally well tolerated and only two patients withdrew during follow-up phase due to mild adverse effects.
|Translated title of the contribution||Efficacy and tolerability of Zonisamide (Zonegran™) in ULD: A pilot open-label trial|
|Number of pages||2|
|Journal||Bollettino - Lega Italiana contro l'Epilessia|
|Publication status||Published - Apr 2011|
ASJC Scopus subject areas
- Clinical Neurology