Antimyoclonic Effect of Levetiracetam and Clonazepam Combined Treatment on Myoclonic Epilepsy with Ragged-Red Fiber Syndrome with m.8344A>G Mutation
Background: Treatment of myoclonic seizures in myoclonic epilepsy with ragged-red fibers (MERRFs) has been empirical and ineffective. Guideline on this disease is not available. Additional trials must be conducted to find more suitable treatments for it. In this study, the antimyoclonic effects of m...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer
2018-01-01
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| Series: | Chinese Medical Journal |
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| Online Access: | http://www.cmj.org/article.asp?issn=0366-6999;year=2018;volume=131;issue=20;spage=2433;epage=2438;aulast=Su |
| _version_ | 1831766854624870400 |
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| author | Li-Jun Su Yu-Liang Wang Tao Han Shan Qiao Ke-Jun Zang Huai-Kuan Wu Yong-Xin Su Ling-Ling Liu Xue-Wu Liu |
| author_facet | Li-Jun Su Yu-Liang Wang Tao Han Shan Qiao Ke-Jun Zang Huai-Kuan Wu Yong-Xin Su Ling-Ling Liu Xue-Wu Liu |
| author_sort | Li-Jun Su |
| collection | DOAJ |
| description | Background: Treatment of myoclonic seizures in myoclonic epilepsy with ragged-red fibers (MERRFs) has been empirical and ineffective. Guideline on this disease is not available. Additional trials must be conducted to find more suitable treatments for it. In this study, the antimyoclonic effects of monotherapies, including levetiracetam (LEV), clonazepam (CZP), valproic acid (VPA), and topiramate (TPM) compared to combination therapy group with LEV and CZP on MERRF, were evaluated to find a more advantageous approach on the treatment of myoclonic seizures.
Methods: Treatments of myoclonic seizures with VPA, LEV, CZP, and TPM were reported as monotherapies in 17 MERRF patients from Qilu Hospital between 2003 and 2016, who were diagnosed through clinical data and genetic testing. After 1–4 months of follow-up (mean: 82.9 ± 28.1 days), 12 patients that exhibited poor responses to monotherapy were given a combined treatment consisting of LEV and CZP subsequently. The follow-up period was 4–144 months (mean: 66.3 ± 45.3 months), the effective rates of monotherapy group (17 patients) and combination therapy group (12 patients) were analyzed by Chi-square test.
Results: The m.8344 A>G mutation was detected in all patients. There were four patients with partial response (4/17, two in the CZP group and two in the LEV group), ten patients with stable disease (10/17, six in the CZP group, three in the LEV group, and one in the TPM group), and three patients with progressive disease (3/18, two in the VPA group and one in the TPM group). Twelve of the patients with LEV combined with CZP showed a positive effect and good tolerance (12/12), eight of them demonstrated improved cognition and coordination. There was a significant difference between the monotherapy group and combination therapy group in the efficacy of antimyoclonic seizures (χ2 = 13.7, P < 0.001).
Conclusions: LEV in combination with CZP is an efficient and safe treatment for myoclonic seizures in patients with this disease exhibiting the m.8344A>G mutation. |
| first_indexed | 2024-12-22T06:30:37Z |
| format | Article |
| id | doaj.art-57885e3953b640a09fd33507b72df459 |
| institution | Directory Open Access Journal |
| issn | 0366-6999 |
| language | English |
| last_indexed | 2024-12-22T06:30:37Z |
| publishDate | 2018-01-01 |
| publisher | Wolters Kluwer |
| record_format | Article |
| series | Chinese Medical Journal |
| spelling | doaj.art-57885e3953b640a09fd33507b72df4592022-12-21T18:35:43ZengWolters KluwerChinese Medical Journal0366-69992018-01-01131202433243810.4103/0366-6999.243568Antimyoclonic Effect of Levetiracetam and Clonazepam Combined Treatment on Myoclonic Epilepsy with Ragged-Red Fiber Syndrome with m.8344A>G MutationLi-Jun SuYu-Liang WangTao HanShan QiaoKe-Jun ZangHuai-Kuan WuYong-Xin SuLing-Ling LiuXue-Wu LiuBackground: Treatment of myoclonic seizures in myoclonic epilepsy with ragged-red fibers (MERRFs) has been empirical and ineffective. Guideline on this disease is not available. Additional trials must be conducted to find more suitable treatments for it. In this study, the antimyoclonic effects of monotherapies, including levetiracetam (LEV), clonazepam (CZP), valproic acid (VPA), and topiramate (TPM) compared to combination therapy group with LEV and CZP on MERRF, were evaluated to find a more advantageous approach on the treatment of myoclonic seizures. Methods: Treatments of myoclonic seizures with VPA, LEV, CZP, and TPM were reported as monotherapies in 17 MERRF patients from Qilu Hospital between 2003 and 2016, who were diagnosed through clinical data and genetic testing. After 1–4 months of follow-up (mean: 82.9 ± 28.1 days), 12 patients that exhibited poor responses to monotherapy were given a combined treatment consisting of LEV and CZP subsequently. The follow-up period was 4–144 months (mean: 66.3 ± 45.3 months), the effective rates of monotherapy group (17 patients) and combination therapy group (12 patients) were analyzed by Chi-square test. Results: The m.8344 A>G mutation was detected in all patients. There were four patients with partial response (4/17, two in the CZP group and two in the LEV group), ten patients with stable disease (10/17, six in the CZP group, three in the LEV group, and one in the TPM group), and three patients with progressive disease (3/18, two in the VPA group and one in the TPM group). Twelve of the patients with LEV combined with CZP showed a positive effect and good tolerance (12/12), eight of them demonstrated improved cognition and coordination. There was a significant difference between the monotherapy group and combination therapy group in the efficacy of antimyoclonic seizures (χ2 = 13.7, P < 0.001). Conclusions: LEV in combination with CZP is an efficient and safe treatment for myoclonic seizures in patients with this disease exhibiting the m.8344A>G mutation.http://www.cmj.org/article.asp?issn=0366-6999;year=2018;volume=131;issue=20;spage=2433;epage=2438;aulast=SuClonazepam; Levetiracetam; Myoclonic Epilepsy with Ragged-Red Fibers; Myoclonic Seizures |
| spellingShingle | Li-Jun Su Yu-Liang Wang Tao Han Shan Qiao Ke-Jun Zang Huai-Kuan Wu Yong-Xin Su Ling-Ling Liu Xue-Wu Liu Antimyoclonic Effect of Levetiracetam and Clonazepam Combined Treatment on Myoclonic Epilepsy with Ragged-Red Fiber Syndrome with m.8344A>G Mutation Chinese Medical Journal Clonazepam; Levetiracetam; Myoclonic Epilepsy with Ragged-Red Fibers; Myoclonic Seizures |
| title | Antimyoclonic Effect of Levetiracetam and Clonazepam Combined Treatment on Myoclonic Epilepsy with Ragged-Red Fiber Syndrome with m.8344A>G Mutation |
| title_full | Antimyoclonic Effect of Levetiracetam and Clonazepam Combined Treatment on Myoclonic Epilepsy with Ragged-Red Fiber Syndrome with m.8344A>G Mutation |
| title_fullStr | Antimyoclonic Effect of Levetiracetam and Clonazepam Combined Treatment on Myoclonic Epilepsy with Ragged-Red Fiber Syndrome with m.8344A>G Mutation |
| title_full_unstemmed | Antimyoclonic Effect of Levetiracetam and Clonazepam Combined Treatment on Myoclonic Epilepsy with Ragged-Red Fiber Syndrome with m.8344A>G Mutation |
| title_short | Antimyoclonic Effect of Levetiracetam and Clonazepam Combined Treatment on Myoclonic Epilepsy with Ragged-Red Fiber Syndrome with m.8344A>G Mutation |
| title_sort | antimyoclonic effect of levetiracetam and clonazepam combined treatment on myoclonic epilepsy with ragged red fiber syndrome with m 8344a g mutation |
| topic | Clonazepam; Levetiracetam; Myoclonic Epilepsy with Ragged-Red Fibers; Myoclonic Seizures |
| url | http://www.cmj.org/article.asp?issn=0366-6999;year=2018;volume=131;issue=20;spage=2433;epage=2438;aulast=Su |
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