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Neurology India, Vol. 54, No. 2, April-June, 2006, pp. 189 Invited Comments Invited Comments Kothare SanjeevV Division of Neurology, Department of Pediatrics, St Christopher's Hospital for Children, Drexel University College of Medicine, Erie Avenue on Front Street, Philadelphia, PA 19134 Code Number: ni06055 Related article: ni06054 Typically presenting in the early teenager years, Juvenile Myoclonic Epilepsy (JME) is a common epilepsy syndrome with a prevalence of 4-10% of all patients with epilepsy.[1] Patients with JME present with multiple seizure types including generalized tonic-clonic, absence and myoclonic jerks. At least 40% of patients with JME report a family history of JME. Genetic studies have indicated several potential loci, with the most commonly accepted site identified as chromosome 6p12-p11.[2] The mainstay of therapy for JME is valproate (VPA), with 80% of patients achieving seizure freedom and up to 90% achieving > 50% reduction in seizure frequency.[3] In this issue, the authors describe their experience using VPA in 46 patients with sporadic JME, as compared to 24 patients with familial JME.[4] The authors prospectively evaluated a cohort of 70 patients with JME treated with VPA for one year. They obtained blood VPA levels and clinical responses and they defined excellent responders as those who were seizure free on treatment. They found no statistical difference in the number of patients who were excellent responders with sporadic JME (76.1%) as compared to those with familial JME (66.7%) with a family history of JME in a first degree relative. Mean drug level of VPA and mg/kg/day of dosage was also similar. History of absence seizures and increased age at the onset of grand mal seizures decreased the number of excellent responders (response rates) in both groups. This is in slight contrast to other reports which have shown poor responders to have all three sub-types of seizures, abnormal photo-paroxysmal response and psychiatric co-morbidities.[3] This study has some limitations. Most importantly, the authors do not provide information as to whether the patients recruited in the study had new-onset seizures and were drug-naïve or if the patients were previously treated. In addition, no information is provided on how seizure counts were monitored in the sub-types with absence seizures. A 24-hour ambulatory EEG is far more sensitive in assessing success to treatment in patients with absence seizures, since clinical observations may be misleading. Also, seizure freedom as an indicator of successful response to therapy may be misleading; a 50% reduction in seizure frequency may be a more accepted way to assess efficacy of therapy. Interestingly, the mean dose of medication in both groups (12-13 mg/kg/day) is significantly lower than what is used in clinical practice. Overall, this study provides valuable information. In spite of JME being both clinically and genetically heterogeneous, both groups responded equally to VPA therapy. Further research is warranted to investigate whether similar observations occur in other epilepsy syndromes and neurological conditions with a genetic predisposition. References
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