Progress in the treatment of progressive myoclonic epilepsies
10.3760/cma.j.cn113694-20220518-00399
- VernacularTitle:进行性肌阵挛癫痫的治疗进展
- Author:
Sujuan SUN
1
;
Tingtao LIU
;
Qi XIANG
;
Xuewu LIU
Author Information
1. 山东大学齐鲁医院神经内科 山东大学癫痫病学研究所,济南 250012
- Keywords:
Epilepsy;
Myoclonic epilepsies, progressive;
Antiseizure drugs;
Treatment
- From:
Chinese Journal of Neurology
2023;56(2):191-197
- CountryChina
- Language:Chinese
-
Abstract:
Progressive myoclonic epilepsies (PMEs) are a group of rare genetic diseases. Common clinical manifestations include action myoclonus often with generalized tonic-clonic seizures, cognitive impairment and other focal neurological deficits. PMEs generally respond poorly to antiseizure drugs and have a poor overall prognosis. Disorders that can cause PMEs include Unverricht-Lundborg disease, Lafora disease, neuronal ceroid lipofuscinosis, myoclonic epilepsy with fragmented red fiber syndrome, sialic acidosis, dentate erythronucleus pallidus Lewy body atrophy, etc. The current treatments for PMEs include drug therapy, neuromodulatory therapy, dietary therapy, anti-inflammatory and immunomodulatory therapy, enzyme replacement therapy and gene therapy. This article reviews the currently known treatments for PMEs, and provides ideas for better research and exploration of treatments for PMEs.