Significant Improvements of EEG and Clinical Findings With Oral Lacosamide in a Patient With Ring Chromosome 20

Abstract

To the Editor, Lacosamide (LCM) was recently approved as an adjunctive therapy for partial-onset seizures with a different mechanism of action that may underly for its effectiveness in intractable epilepsy. 1 In animal models, its utility to reduce the cumilative seizure time of self-sustaining status epilepticus and hippocampal neuronal damage was shown. 2 In 3 randomized doubleblind, placebo-controlled, multicenter trials in more than 1200 patients, efficiency of LCM was studied and they reported a significant reduction in seizure frequency with doses of 200 to 600 mg/d. [3][4][5] Additionally, as a result of new clinical studies on status epilepticus (SE), LCM remarked compared with other new antiepileptic drugs (AED). 2 Here, we present a very rare patient with ring chromosome (R20) who was admitted for refractory epilepsy with frequent secondary generalized seizures (SGTCS) and nonconvulsive status epilepticus (NCSE) episodes. Interestingly, her frequent seizures and EEG findings with NCSE have been significantly improved with an add on treatment of oral lacosamide. Case ReportA 25-year-old woman was hospitalized in our video-EEG monitoring unit 8 years ago due to her refractory seizures and learning problems. Her brother also had learning and behavioral problems whose EEG findings included generalized epileptiform discharges. Our patient had daily complex partial seizures and secondary generalized seizures. Also her interictal EEG showed very frequent generalized epileptiform activities. She was apathic and confused. Despite the usage of lots of AEDs, including carbamazepine, valproic acide, levetiracetam, intravenously or orally, she had frequent seizures, she was confused and her EEG was not recovered. Her brain magnetic resonance imaging was unremarkable. Because of her progressive secondary generalized seizures, learning problems and EEG findings showing NCSE patterns occuring particularly during sleep period, she was investigated for R20. After diagnosis of R20 mosaicism, we decided to vagus nerve stimulation treatment but that was not available because of her social problems.Her family reported daily seizures and she was apathic with frequent epileptiform discharges on EEG at the last follow-up visiting in our outpatient clinic, 7 months ago. Although she has been undertreatment of carbamazepine 600 mg/d, valproic acid 1500 mg/d, levetiracetam 2000 mg/d, and clonazepam 3 g/d, she had 4 to 5 SGTCSs in a day. On neurological examination, she was confused and unable to walk alone. Routine laboratory investigations including full blood count and biochemistry were normal. Serum drug levels for carbamazepine (6 mg/L) and valproic acid (68 µg/mL) were in theraupatic ranges. Routine EEG showed backround activity slowing with 3.5-to 4-Hz waves. Generalized slow and sharp characterized slow waves and 2-to 2.5-Hz spike-wave complexes were reported in anterior parts of hemispheres. These generalized epileptiform discharges appeared for 40 to 60 seconds with not any clinical findings (Figure 1 A and B).Oral LCM wa...

Praxis‐induced reflex seizures in two Japanese cases with ring chromosome 20 syndrome

“…This combination should also be considered as patients grow older because NCSE usually increases in frequency in patients with R20 when they reach adolescence. In addition, only one case of R20 was reported to benefit from lacosamide (Onder and Tezer, 2016). In the current cases, lacosamide and gabapentin were effective in each patient, respectively.…”

Ring Chromosome 20 Syndrome: Genetics, Clinical Characteristics, and Overlapping Phenotypes

“…In our experience, valproic acid and lamotrigine, often in combination, are generally the most effective antiepileptic drugs (AEDs) for treating seizures in r(20) (20). However, many patients continue to have drug-resistant epilepsy, and other AEDs have been reported to be effective, such as lacosamide (31,79), ezogabine (80), and lithium (34). Some affected individuals have found beneficial effects from alternative treatments, such as ketogenic diet (81), while the efficacy of vagal nerve stimulation is controversial (26,32,67,82).…”

Electroclinical Improvement in a Patient with Ring Chromosome 20 Syndrome Treated with Zonisamide: A Case Report

“…2 3 4 In recent years, add-on treatment with lacosamide and ezogabine showed a favorable outcome. 8 9 Moreover, improvement with vagus nerve stimulation and ketogenic diet has been described. 4 5 6 7 8 9 10…”

“…8 9 Moreover, improvement with vagus nerve stimulation and ketogenic diet has been described. 4 5 6 7 8 9 10…”

“…[2][3][4] In recent years, add-on treatment with lacosamide and ezogabine showed a favorable outcome. 8,9 Moreover, improvement with vagus nerve stimulation and ketogenic diet has been described. [4][5][6][7][8][9][10] We report the case of a 13-year-old female with mosaic r (20) syndrome, who showed a significant improvement after an add-on treatment with oral zonisamide.…”