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线粒体脑肌病伴乳酸酸中毒和卒中样发作(MELAS)综合征延迟就诊继发的复发性卒中事件

Recurrent Stroke Events Secondary to a Late Presentation of Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-Like Symptoms (MELAS) Syndrome.

作者信息

Jameel Ihab, Sreh Abuajela, Das Partha

机构信息

Internal Medicine, University Hospitals of Derby and Burton NHS Foundation Trust, Derby, GBR.

Gastroenterology, Barnsley Hospital NHS Foundation Trust, Barnsley, GBR.

出版信息

Cureus. 2020 Dec 2;12(12):e11839. doi: 10.7759/cureus.11839.

Abstract

Mitochondrial encephalomyopathy with lactic acidosis and stroke-like symptoms (MELAS) is a rare mitochondrial disorder that typically presents before the age of 40 with most patients diagnosed before the age of 20. Symptoms and signs typically include mitochondrial myopathy, encephalopathy with stroke-like episodes, seizures and/or dementia, and lactic acidosis. We present a case of a 56-year-old lady presenting with recurrent ischaemic strokes and seizures associated with non-territorial low attenuation areas on brain imaging. Together with a raised serum lactate and background history of Syndrome of Inappropriate secretion of Anti-Diuretic Hormone (SIADH), genetic analysis was carried out that confirmed the presence of the most common mutation associated with MELAS syndrome which is m.3243A>G mutation. This case raises the importance of considering a diagnosis of inherited mitochondrial disorder when faced with recurrent atypical stroke-like episodes, when neuro-imaging is inconsistent with ischemic infarction, even in adults or elderly individuals. It also highlights the importance of background history and associated conditions that should be put into consideration when thinking about differential diagnosis.

摘要

伴有乳酸性酸中毒和卒中样症状的线粒体脑肌病(MELAS)是一种罕见的线粒体疾病,通常在40岁之前发病,大多数患者在20岁之前被诊断出来。症状和体征通常包括线粒体肌病、伴有卒中样发作的脑病、癫痫和/或痴呆以及乳酸性酸中毒。我们报告一例56岁女性病例,该患者出现复发性缺血性卒中以及癫痫发作,脑部影像学检查显示非脑叶性低衰减区域。结合血清乳酸水平升高以及抗利尿激素不适当分泌综合征(SIADH)的背景病史,进行了基因分析,证实存在与MELAS综合征相关的最常见突变,即m.3243A>G突变。该病例凸显了在面对复发性非典型卒中样发作、神经影像学表现与缺血性梗死不一致时,即使是成人或老年人,考虑遗传性线粒体疾病诊断的重要性。它还强调了在考虑鉴别诊断时应考虑背景病史和相关疾病的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8422/7714735/cbc38ce86ff0/cureus-0012-00000011839-i01.jpg

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