{"title":"[A case of amyotrophic lateral sclerosis complicated by syringomyelia associated with Chiari type I malformation].","authors":"Miki Aikawa, Tetsuo Ando, Hidehiro Shibayama, Motoo Kubota, Masahiro Sonoo, Toshio Fukutake","doi":"10.5692/clinicalneurol.cn-002045","DOIUrl":null,"url":null,"abstract":"<p><p>The patient was a 78-year-old woman. She underwent foramen magnum decompression for syringomyelia associated with Chiari type I malformation, which had developed with difficulty in raising the left upper limb and muscle weakness in both upper limbs. One year after surgery, weight loss of 20 kg, progressive muscle atrophy and weakness in the extremities, paralytic dysarthria, and fasciculation in the bilateral anterior thighs were observed, and needle electromyography showed acute denervation and chronic denervation in the medial vastus muscle. The rapid postoperative progression of symptoms and lower motor neuron symptoms in the lower extremities could not be explained by syringomyelia associated with Chiari type I malformation and were considered a possible complication of amyotrophic lateral sclerosis (ALS). It is possible that the surgery may have caused ALS progression, and attention to the rate of progression of neurologic symptoms may be important in the diagnosis of ALS complications.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2025-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Neurology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.5692/clinicalneurol.cn-002045","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
摘要
患者是一名 78 岁的女性。她因脊髓脊膜膨出症伴有 Chiari I 型畸形而接受了枕骨大孔减压术,术后出现左上肢抬高困难和双上肢肌无力。术后一年,患者体重下降 20 kg,四肢肌肉进行性萎缩和无力,构音障碍,双侧大腿前部出现筋束,针刺肌电图显示内侧阔肌急性变性和慢性变性。术后症状和下肢下运动神经元症状的快速发展无法用奇拉氏Ⅰ型畸形相关的鞘膜积液来解释,因此被认为可能是肌萎缩性脊髓侧索硬化症(ALS)的并发症。手术有可能导致肌萎缩性脊髓侧索硬化症恶化,因此关注神经症状的恶化速度对诊断肌萎缩性脊髓侧索硬化症并发症非常重要。
[A case of amyotrophic lateral sclerosis complicated by syringomyelia associated with Chiari type I malformation].
The patient was a 78-year-old woman. She underwent foramen magnum decompression for syringomyelia associated with Chiari type I malformation, which had developed with difficulty in raising the left upper limb and muscle weakness in both upper limbs. One year after surgery, weight loss of 20 kg, progressive muscle atrophy and weakness in the extremities, paralytic dysarthria, and fasciculation in the bilateral anterior thighs were observed, and needle electromyography showed acute denervation and chronic denervation in the medial vastus muscle. The rapid postoperative progression of symptoms and lower motor neuron symptoms in the lower extremities could not be explained by syringomyelia associated with Chiari type I malformation and were considered a possible complication of amyotrophic lateral sclerosis (ALS). It is possible that the surgery may have caused ALS progression, and attention to the rate of progression of neurologic symptoms may be important in the diagnosis of ALS complications.