Acute management of a stroke-like episode in MELAS syndrome: What should we know?

González, Tirso González-Pinto; Velasco, Javier Almeida; Estébanez, A. Moreno; Beitia, Garazi Agirre; Martinez, Laura; Cuervo, Irene Díaz; Arroyo, A. Martínez

doi:10.1016/j.ensci.2020.100249

Cited by 5 publications

(8 citation statements)

References 6 publications

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“…We do not agree with the notion that the index patient is the first adult case with good clinical outcome after “quick implementation of this approach” [ 1 ]. Several adults receiving ASD in addition to NO-precursors have been reported which responded favourably under this regimen [ [4] , [5] , [6] ].…”

contrasting

confidence: 65%

“…With interest we read the article by González-Pinto González et al about the management of a first ever stroke-like episode (SLE), with a corresponding stroke-like lesion (SLL), which was hyperintense on fluid attenuation inversion recovery (FLAIR) and diffusion weighted imaging (DWI) but isointense on apparent diffusion coefficient (ADC) maps, in a 38 years old male, who was diagnosed with mitochondrial encephalopathy, lactic acidosis, and stroke-like episode (MELAS) syndrome at age 30 years upon the clinical presentation (hypoacusis, permanent hyper-creatine-kinase (CK)-emia), a positive family history for MELAS (maternal uncle), and the detection of the variant m.3243A > G [ 1 ]. The SLE occurred despite regular intake of l -carnitine, coenzyme-Q (CoQ), and L-arginine (21 g/d) starting at age 30 years [ 1 ].…”

mentioning

confidence: 99%

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Optimising therapeutic strategies for acute stroke-like lesions in MELAS

Finsterer

2020

eNeurologicalSci

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contrasting

confidence: 65%

mentioning

confidence: 99%

Optimising therapeutic strategies for acute stroke-like lesions in MELAS

Finsterer

2020

eNeurologicalSci

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“…Several pathogeneses, including mitochondrial angiopathy, metabolic defects, and neuronal hyperexcitability, have been considered to have a potential role in this clinical condition. 2 The mechanism of mitochondrial angiopathy could be associated with ischemia due to small vessel injury; it is rarely involved in the large vessel for SE in MELAS. 3 However, some cases of large vessels involvement in MELAS are reported, including This is the first case report of endovascular revascularization therapy for ICA dissection in a patient with MELAS.…”

Section: Discussionmentioning

confidence: 99%

Endovascular revascularization for internal carotid artery dissection during stroke‐like episode in MELAS: A case report

Mitsutake

Tsugawa

Kimura

et al. 2021

Neurology & Clinical Neurosc

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A 45-year-old woman was referred to our hospital after complaining of speech difficulty for the previous 24 hours. She had a medical history of migraine for 10 years, deafness since birth, type 2 diabetes mellitus for 5 years, and depression for 5 years.Her initial neurological findings showed mild Broca-type aphasia without motor or sensory deficits (NIH Stroke scale score of 1).Brain magnetic resonance imaging (MRI) at admission revealed a hyperintense lesion in the left parietal to temporal lobe cortex on diffusion-weighted imaging (DWI) (Figure 1A) and apparent diffusion coefficient (ADC). Severe stenosis or occlusion of the cerebral arteries was not observed on initial magnetic resonance angiography (MRA). Acute cerebral infarction was diagnosed based on the MRI findings and clinical features, and recanalization occurred after occlusion of the left middle cerebral artery. Intravenous heparin sodium injection of 15 000 units/day was started. On the second day of admission, she complained of a severe headache, and her sensory aphasia gradually worsened with a confused state. A brain MRI performed on the 9th day of admission showed expansion of the hyperintense area in the left temporal to the parietal and occipital cortices on DWI (Figure 1B). MRA revealed severe stenosis of the left internal carotid artery (ICA) (Figure 1C). On magnetic resonance (MR)/time of flight (TOF) imaging, a double lumen sign was observed at the bifurcation of the left common carotid artery (Figure 1D). These findings suggested a diagnosis of left ICA dissection. Since her neurological symptoms worsened and the ischemic area on MRI expanded, revascularization therapy for left ICA dissection was considered.Long tapered irregular stenosis in the cervical portion of the left internal carotid artery and congestion of the contrast medium were

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“…Patients are prescribed a mitochondrial "cocktail" of antioxidants and cofactors to enhance metabolism and reduce oxidative stress. Consensus guidelines now recommend the administration of high-dose intravenous L-arginine (a nitric oxide precursor) in patients with acute neurological symptoms consistent with stroke-like episodes (Gonz ález et al, 2020).…”

Section: Treatmentmentioning

confidence: 99%

Understanding the nomenclature of mitochondrial DNA mutations through examples of two specific disease entities: Mitochondrial encephalopathy with lactic acidosis and stroke-like episodes and Leber hereditary optic neuropathy

Heuer

Seibert²

2022

J Am Assoc Nurse Pract

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Mitochondrial diseases are genetic disorders that can arise either from maternally inherited mitochondrial DNA (mtDNA) or from mutations in nuclear DNA. This article is the second in a series of papers reviewing mitochondrial genetics and several of the disorders associated with mitochondrial gene variants. With a prevalence of 1:∼4,300 persons, mitochondrial disorders are diagnostic entities with which nurse practitioners should be familiar. In describing genetic mutations, numbering nucleotides (nuclear or mtDNA) is critical for communicating exactly where a variation has occurred in a stretch of nucleotides. This article discusses the nomenclature associated with mtDNA mutations, using the examples of mutations causing mitochondrial encephalopathy with lactic acidosis and stroke-like episodes and Leber hereditary optic neuropathy. Pathophysiology, symptoms, and treatment options for these disease entities are discussed.

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Acute management of a stroke-like episode in MELAS syndrome: What should we know?

Cited by 5 publications

References 6 publications

Optimising therapeutic strategies for acute stroke-like lesions in MELAS

Optimising therapeutic strategies for acute stroke-like lesions in MELAS

Endovascular revascularization for internal carotid artery dissection during stroke‐like episode in MELAS: A case report

Understanding the nomenclature of mitochondrial DNA mutations through examples of two specific disease entities: Mitochondrial encephalopathy with lactic acidosis and stroke-like episodes and Leber hereditary optic neuropathy

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