Epileptic seizures worsen the gait and motor abnormalities in adult patients with Dravet syndrome(with a case report and Literature Review).

Epilepsia open(2023)

引用 0|浏览1
暂无评分
摘要
Dravet syndrome (DS), previously known as severe myoclonic epilepsy in infancy (SMEI), is considered the most serious "epileptic encephalopathy". Here, we present a man with a de novo SCN1A mutation who was diagnosed with DS at the age of 29. In addition to pharmacoresistant seizures and cognitive delay, he also developed moderate to severe motor and gait problems, such as crouching gait and Pisa syndrome. Moreover, it deteriorated significantly following an epileptic seizure. The patient presented with severe flexion of the head and trunk in the sagittal plane and fulfilled the diagnostic criteria for camptocormia and antecollis. After a week, it spontaneously alleviated partially. We applied levodopa to the patient and had a good response. Functional Gait Assessment (FGA) was assessed at three different times: four days after the seizure, one week after the seizure, and after taking levodopa for two years. The results were 4, 12, and 19 points, respectively. We postulated that: (1) gait and motor deficits are somehow influenced by recurrent epileptic episodes;(2) the nigrostriatal dopamine system is involved. To our knowledge, we were the ones who first reported this phenomenon.
更多
查看译文
关键词
crouching gait, Dravet syndrome, gait disturbances, prognosis, seizure
AI 理解论文
溯源树
样例
生成溯源树,研究论文发展脉络
Chat Paper
正在生成论文摘要