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Condition: Mitochondrial Disease

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Total 851 results found since Jan 2013.

Comparison of magnetic resonance spectroscopy (MRS) with arterial spin labeling (ASL) in the differentiation between mitochondrial encephalomyopathy, lactic Acidosis, plus stroke-like episodes (MELAS) and acute ischemic stroke (AIS)
Publication date: Available online 18 June 2018Source: Journal of Clinical NeuroscienceAuthor(s): Xiaodi Li, Yuzhou Wang, Zhanhang Wang, Jianjun Lu, Yan Xu, Jinlong Ye, Zuying Kuang, Bo Li, Mengqiu Pan, Wenming Chen, Shuisheng Lu, Ziyang Zhou, Lina Cheng, Honghao WangAbstractTo compare the utility and limitation of magnetic resonance spectroscopy (MRS) and arterial spin labeling (ASL) in the differentiation between mitochondrial encephalomyopathy, lactic acidosis, plus stroke-like episodes (MELAS) and acute ischemic stroke (AIS), a retrospective review of 17 MELAS and 26 AIS patients were performed. In all patients both MR...
Source: Journal of Clinical Neuroscience - July 5, 2018 Category: Neuroscience Source Type: research

The Usefulness of Muscle Biopsy in Initial Diagnostic Evaluation of Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes.
CONCLUSION: The usefulness of muscle biopsy appears to be limited to an initial confirmative diagnostic evaluation of MELAS. Muscle biopsy can provide some information in MELAS patients with weakness not confirmed by genetic testing. PMID: 30554496 [PubMed - in process]
Source: Yonsei Medical Journal - December 18, 2018 Category: Universities & Medical Training Authors: Baek MS, Kim SH, Lee YM Tags: Yonsei Med J Source Type: research

Pramipexole prevents ischemic cell death via mitochondrial pathways in ischemic stroke RESEARCH ARTICLE
ABSTRACT A dopamine D2 receptor agonist, pramipexole, has been found to elicit neuroprotection in patients with Parkinson’s disease and restless leg syndrome. Recent evidence has shown that pramipexole mediates its neuroprotection through mitochondria. Considering this, we examined the possible mitochondrial role of pramipexole in promoting neuroprotection following an ischemic stroke of rat. Male Wistar rats underwent transient middle cerebral artery occlusion (tMCAO) and then received pramipexole (0.25 mg and 1 mg/kg body weight) at 1, 6, 12 and 18 h post-occlusion. A panel of neurological tests and ...
Source: DMM Disease Models and Mechanisms - August 28, 2019 Category: Biomedical Science Authors: Andrabi, S. S., Ali, M., Tabassum, H., Parveen, S., Parvez, S. Tags: Rat as a Disease Model RESEARCH ARTICLE Source Type: research

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes due to m.3243A   >  G mutation in a 76-year-old woman
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) with m.3243A  > G mutation is a common type of mitochondrial disease [1,2]. Most patients with MELAS develop stroke-like episodes before the age of 40, a criterion of MELAS [3]. However, increasing reports have demonstrated that elderly subjects (≥ 60 years old) may develop this mitochondrial encephalopathy [4,5]. Here, we report a 76-year-old woman who was previously healthy without any organ dysfunction related to mitochondrial abnormalities (except for a mild hearing disability) and developed encephalopathy accompanied by st...
Source: Journal of the Neurological Sciences - March 18, 2020 Category: Neurology Authors: Kana Ueki, Yoshinobu Wakisaka, Kuniyuki Nakamura, Yuji Shono, Shinichi Wada, Yoji Yoshikawa, Yuta Matsukuma, Takeshi Uchiumi, Dongchong Kang, Takanari Kitazono, Tetsuro Ago Tags: Letter to the Editor Source Type: research

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes due to m.3243A   >  G mutation in a 76-year-old woman
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) with m.3243A  > G mutation is a common type of mitochondrial disease [1,2]. Most patients with MELAS develop stroke-like episodes before the age of 40, a criterion of MELAS [3]. However, increasing reports have demonstrated that elderly subjects (≥ 60 years old) may develop this mitochondrial encephalopathy [4 ,5]. Here, we report a 76-year-old woman who was previously healthy without any organ dysfunction related to mitochondrial abnormalities (except for a mild hearing disability) and developed encephalopathy accompanied by stro...
Source: Journal of the Neurological Sciences - March 18, 2020 Category: Neurology Authors: Kana Ueki, Yoshinobu Wakisaka, Kuniyuki Nakamura, Yuji Shono, Shinichi Wada, Yoji Yoshikawa, Yuta Matsukuma, Takeshi Uchiumi, Dongchong Kang, Takanari Kitazono, Tetsuro Ago Tags: Letter to the Editor Source Type: research

Knowledge about the characteristics of stroke-like lesions is expandable
AbstractStroke-like episodes (SLEs) are a common phenotypic feature of various syndromic and non-syndromic  mitochondrial disorders (MIDs), particularly of mitochondrial encephalopathy, lactic acidosis, and stroke-like episode syndrome (MELAS). The morphological equivalent of a SLE is the stroke-like lesion (SLE), a dynamic lesion, which initially expands to regress after weeks or months. SLLs present with typical morphological and structural abnormalities on multimodal magnetic resonance imaging (MRI), magnetic resonance spectroscopy (MRS), and FDG-PET. It is crucial to clearly delineate SLLs from ischemic stroke, as...
Source: Metabolic Brain Disease - August 26, 2021 Category: Neurology Source Type: research

Stroke-like lesions in mitochondrial disease may resemble ischemic stroke
J Family Med Prim Care. 2021 Aug;10(8):3151-3153. doi: 10.4103/jfmpc.jfmpc_2314_20. Epub 2021 Aug 27.ABSTRACTThe patient is a 73-y-male who was referred after a fall without losing consciousness or secessus. Clinical exam revealed disorientation, ophthalmoparesis, hemianopia to the left, left hemineglect, hypoacusis, quadruparesis, general wasting, generally reduced tendon reflexes, mild rigor, occasional myoclonic jerks of the right lower limb, and ataxia of the left lower limb. Cerebral magnetic resonance imaging (MRI) showed a stroke-like lesion (SLL), generalized atrophy, white matter lesions, and ponsgliosis. The prev...
Source: Primary Care - October 18, 2021 Category: Primary Care Authors: Josef Finsterer Source Type: research

L-arginine and mitochondrial encephalomyopathy, lactic acidosis and stroke- like episodes (MELAS): a systematic review
Conclusion Evidence for the use of L-arginine in MELAS syndrome is poor, and is associated with a high risk of bias. yi.ng@ncl.ac.uk 19
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 27, 2022 Category: Neurosurgery Authors: Stefanetti, R., Ng, Y. S., Errington, L., Dlay, J., Turnbull, D., McFarland, R., Gorman, G. Tags: Inflammation [amp ] Neurogenetics: 04 Source Type: research

Crocins for Ischemic Stroke: A Review of Current Evidence
Crocins (CRs) and the related active constituents derived from Crocus sativus L. (Saffron) have demonstrated protective effects against cerebral ischemia and ischemic stroke, with various bioactivities including neuroprotection, anti-neuroinflammation, antioxidant, and cardiovascular protection. Among CRs, crocin (CR) has been shown to act on multiple mechanisms and signaling pathways involved in ischemic stroke, including mitochondrial apoptosis, nuclear factor kappa light chain enhancer of B cells pathway, S100 calcium-binding protein B, interleukin-6 and vascular endothelial growth factor-A. CR is generally safe and wel...
Source: Frontiers in Pharmacology - August 5, 2022 Category: Drugs & Pharmacology Source Type: research

New insights into the roles of oligodendrocytes regulation in ischemic stroke recovery
Neurobiol Dis. 2023 Jun 13:106200. doi: 10.1016/j.nbd.2023.106200. Online ahead of print.ABSTRACTOligodendrocytes (OLs), the myelin-forming cells of the central nervous system, are integral to axonal integrity and function. Hypoxia-ischemia episodes can cause severe damage to these vulnerable cells through excitotoxicity, oxidative stress, inflammation, and mitochondrial dysfunction, leading to axonal dystrophy, neuronal dysfunction, and neurological impairments. OLs damage can result in demyelination and myelination disorders, severely impacting axonal function, structure, metabolism, and survival. Adult-onset stroke, per...
Source: Neurobiology of Disease - June 15, 2023 Category: Neurology Authors: Shuangfeng Huang Changhong Ren Yumin Luo Yuchuan Ding Xunming Ji Sijie Li Source Type: research

Lipoprotein Receptor-Related Protein-6 Protects the Brain From Ischemic Injury Basic Sciences
Conclusions— LRP6 activates an endogenous neuroprotective pathway that acts independently of β-catenin by controlling GSK-3β activity and preventing its deleterious mitochondrial and proinflammatory effects. The findings raise the possibility that emerging treatment strategies for diseases attributable to LRP6 loss-of-function mutations could also lead to new therapeutic avenues for ischemic stroke.
Source: Stroke - July 22, 2013 Category: Neurology Authors: Abe, T., Zhou, P., Jackman, K., Capone, C., Casolla, B., Hochrainer, K., Kahles, T., Ross, M. E., Anrather, J., Iadecola, C. Tags: Animal models of human disease, Acute Cerebral Infarction Basic Sciences Source Type: research

Brain Herniation Due To Mitochondrial Encephalomyopathy, Lactic Acidosis, Stroke Like Events (MELAS) With 3256 Mutation Responsive To Decompressive Craniotomy (P6.257)
CONCLUSIONS:In our MELAS case, decompessive craniotomy is life saving for brain herniation due to SLE. Headache refractory to narcotics and increased lactic acid level are early warning signs of acute SLE since MRI can be normal. Steriod might be indicated for acute treatment of cortical edema.Disclosure: Dr. Cui has nothing to disclose. Dr. Schwendimann has received research support from Lundbeck.
Source: Neurology - April 9, 2014 Category: Neurology Authors: Cui, C., Schwendimann, R. Tags: Cerebrovascular Disease and Interventional Neurology: Case Reports Source Type: research