Reversible Dilation of Cerebral Macrovascular Changes in MELAS Episodes

Li, Yuxin; Xu, Wei; Sun, Chong; Lin, Jie; Qu, Jianxun; Cao, Jiawen; Li, Haiqing; Yang, Li

doi:10.1007/s00062-018-0662-8

Cited by 17 publications

(15 citation statements)

References 31 publications

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“…MRA has not been routinely performed in MELAS in the past, because major cerebral vessels were considered to be the target of mitochondrial metabolism defects in these patients. However, more and more studies have found major cerebral vessels dilation ( 39 , 44 , 45 ) or stenosis ( 46 , 47 ) on MRA in MELAS in the acute and chronic stages of the disease. Gramegna et al ( 48 ) found that the proportion of cerebral major vessels dilation and stenosis was 40 and 19%, respectively, on MRA, and the middle cerebral artery was the most commonly involved.…”

Section: Funtional Mri Findingsmentioning

confidence: 99%

MRI Features of Stroke-Like Episodes in Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-Like Episodes

Cheng

Zhang

2022

Front. Neurol.

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Mitochondrial myopathy encephalopathy lactic acidosis and stroke-like episodes (MELAS) is an important cause of stroke-mimicking diseases that predominantly affect patients before 40 years of age. MELAS results from gene mutations in either mitochondrial DNA (mtDNA) or nuclear DNA (nDNA) responsible for the wide spectrum of clinical symptoms and imaging findings. Neurological manifestations can present with stroke-like episodes (the cardinal features of MELAS), epilepsy, cognitive and mental disorders, or recurrent headaches. Magnetic resonance imaging (MRI) is an important tool for detecting stroke-like lesions, accurate recognition of imaging findings is important in guiding clinical decision making in MELAS patients. With the development of neuroimaging technologies, MRI plays an increasingly important role in course monitoring and efficacy assessment of the disease. In this article, we provide an overview of the neuroimaging features and the application of novel MRI techniques in MELAS syndrome.

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Section: Funtional Mri Findingsmentioning

confidence: 99%

MRI Features of Stroke-Like Episodes in Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-Like Episodes

Cheng

Zhang

2022

Front. Neurol.

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“…In patient 8, who had bilateral SLLs in the parietal lobes, matching dilation of the MCA and dilatation of the right ACA were found [1]. We do not agree that the right ACA matches with a right parietal SLL.…”

mentioning

confidence: 58%

“…In a recent article by Li et al [1], a study of cerebral perfusion in 20 patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) by MRI/MRA was presented. We have the following comments/concerns.…”

Section: Letter To Editormentioning

confidence: 99%

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Cerebral Hyper-perfusion in Stroke-like Lesions may be Secondary and not Pathogenic

Finsterer¹,

Rudolfstiftung²,

Sinda³

2018

J Clin Cases Rep

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“…SLEs are characterized by recurrent attacks followed by symptomatic remission, eventually resulting in the progressive accumulation of neurological deficits in MELAS 4 . Evidence from neuroimaging, such as the alterations of cerebral diffusion, perfusion, and metabolism, 5–7 has revealed the different characteristics between the acute SLEs stage and chronic stage in patients with MELAS. However, the neuropathological mechanisms of MELAS remain largely unknown and need to be further explored.…”

mentioning

confidence: 99%

Altered Dynamic Functional Connectivity in Patients With Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke‐Like Episodes (MELAS) at Acute and Chronic Stages: Shared and Specific Brain Connectivity Abnormalities

Wang

Sun

Lin

et al. 2020

Magnetic Resonance Imaging

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BackgroundMitochondrial encephalomyopathy with lactic acidosis and stroke‐like episodes (MELAS) is a rare maternally inherited genetic disease; however, little is known about its underlying brain basis. Furthermore, the dynamic functional connectivity (dFC) of brain networks in MELAS has not been explored.PurposeTo investigate the abnormalities of dFC in patients with MELAS at the acute and chronic stages, and to determine the possible relations between dynamic connectivity alterations and volumes of stroke‐like lesions (SLLs).Study TypeProspective.SubjectsTwenty‐two MELAS patients at the acute stage, 23 MELAS patients at the chronic stage, and 22 healthy controls.Field Strength/SequenceSingle‐shot gradient‐recalled echo planar imaging (EPI) sequence at 3T.AssessmentDynamic FC states were estimated using the sliding window approach and k‐means clustering analyses. Combined with graph theory, the topological properties of the dFC network were also accessed.Statistical TestsPermutation test, Pearson correlation coefficient, and false discovery rate correction.ResultsWe identified four dFC states and found that MELAS patients (especially at the acute stage) spent more time in a state with weaker connectivity (state 1) and less time in states with stronger connectivity. In addition, volumes of acute SLLs were positively correlated with mean dwell time in state 1 (r = 0.539, P < 0.05) and negatively correlated with the number of transitions (r = −0.520, P < 0.05). Furthermore, MELAS patients at the acute stage exhibited significantly increased global efficiency (P < 0.01) and decreased local efficiency (P < 0.001) compared to the controls and the patients at the chronic stage. Patients at the chronic stage only showed significantly (P < 0.001) decreased local efficiency compared to the controls.Data ConclusionOur findings suggest similar and distinct dFC alterations in MELAS patents at the acute and chronic stages, providing novel insights for understanding the neuropathological mechanisms of MELAS.Level of Evidence 2Technical Efficacy Stage Stage 2J. MAGN. RESON. IMAGING 2021;53:427–436.

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Reversible Dilation of Cerebral Macrovascular Changes in MELAS Episodes

Abstract: Reversible dilation of cerebral macrovascular changes could be a new feature of MELAS and a presumed reason for fluctuant CBF. It would shed new light on the mitochondrial angiopathy.

Cited by 17 publications

References 31 publications

MRI Features of Stroke-Like Episodes in Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-Like Episodes

MRI Features of Stroke-Like Episodes in Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-Like Episodes

Cerebral Hyper-perfusion in Stroke-like Lesions may be Secondary and not Pathogenic

Altered Dynamic Functional Connectivity in Patients With Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke‐Like Episodes (MELAS) at Acute and Chronic Stages: Shared and Specific Brain Connectivity Abnormalities

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