Effective Management of Chronic Intestinal Pseudo-Obstruction in MELAS Using Acotiamide: A Case Report.

IF 0.6 Q4 CLINICAL NEUROLOGY

Case Reports in Neurology Pub Date : 2024-09-14 eCollection Date: 2024-01-01 DOI:10.1159/000541012

Yuri Kawano, Akira Taniguchi, Yugo Narita, Ken Kagawa, Tetsuro Harada, Akihiro Shindo

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引用次数: 0

Abstract

Introduction: Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is reported to be a secondary cause of chronic intestinal pseudo-obstruction (CIPO). Although few case reports have described CIPO in MELAS, effective treatment for CIPO has not been established. Here, we present a case report of amelioration of CIPO symptoms using acotiamide in a patient with MELAS.

Case presentation: A 51-year-old Japanese female with a mitochondrial disorder with m.3243A>G mutation and a history of anorexia for 2 years presented to our hospital with a left temporal headache and acute paraphasia. A stroke-like episode of MELAS was suspected and combined therapy with arginine, edaravone, and levetiracetam was initiated. Although her symptoms improved, she presented with nausea and vomiting and abdominal distension 6 days following admission. Abdominal contrast-enhanced computed tomography revealed dilatation from the stomach to the intestine, particularly marked the stomach, with neither obstruction nor impaired blood flow in the intestine. CIPO exacerbation with MELAS was suspected, and the patient's symptoms gradually improved with acotiamide.

Conclusion: Patients with MELAS could possibly experience stroke-like episodes during CIPO. Since acetylcholine possibly plays an important role in the pathophysiology of CIPO and acotiamide possesses prokinetic activity by inhibiting acetylcholinesterase, acotiamide could possibly improve CIPO symptoms.

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使用阿考替酰胺有效治疗 MELAS 的慢性假性肠梗阻：病例报告。

导言：据报道，线粒体脑肌病、乳酸酸中毒和中风样发作（MELAS）是慢性肠假性梗阻（CIPO）的继发原因。尽管有少数病例报告描述了 MELAS 中的 CIPO，但 CIPO 的有效治疗方法尚未确立。在此，我们报告了一例使用阿考替酰胺改善 MELAS 患者 CIPO 症状的病例：一名 51 岁的日本女性线粒体疾病患者（m.3243A>G 基因突变）因左颞部头痛和急性肢体瘫痪到我院就诊。患者被怀疑是 MELAS 中风样发作，并开始接受精氨酸、依达拉奉和左乙拉西坦联合治疗。虽然她的症状有所改善，但入院 6 天后又出现恶心、呕吐和腹胀。腹部造影剂增强计算机断层扫描显示，从胃到肠都有扩张，胃部尤为明显，肠道既无梗阻也无血流障碍。患者的症状在服用阿考替胺后逐渐好转：结论：MELAS 患者在 CIPO 期间可能会出现中风样发作。由于乙酰胆碱可能在CIPO的病理生理学中起着重要作用，而阿考替胺通过抑制乙酰胆碱酯酶具有促动力活性，因此阿考替胺有可能改善CIPO症状。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Case Reports in Neurology Medicine-Neurology (clinical)

CiteScore

1.50

自引率

0.00%

发文量

审稿时长

14 weeks

期刊介绍： This new peer-reviewed online-only journal publishes original case reports covering the entire spectrum of neurology. Clinicians and researchers are given a tool to disseminate their personal experience to a wider public as well as to review interesting cases encountered by colleagues all over the world. To complement the contributions supplementary material is welcomed. The reports are searchable according to the key words supplied by the authors; it will thus be possible to search across the entire growing collection of case reports with universally used terms, further facilitating the retrieval of specific information. Following the open access principle, the entire contents can be retrieved at no charge, guaranteeing easy access to this valuable source of anecdotal information at all times.