Inappropriate intracranial hemodynamics in the natural course of MELAS

Nishioka, Junko; Akita, Yukihiro; Yatsuga, Shuichi; Katayama, Koujyu; Matsuishi, Toyojiro; Ishibashi, Masatoshi; Koga, Yasutoshi

doi:10.1016/j.braindev.2007.06.008

Cited by 32 publications

(19 citation statements)

References 9 publications

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“…However, the territories of the affected pial and intracerebral arterioles do not correspond to the distribution of SLLs . Furthermore, focal hyperemia in affected areas is frequently observed during the acute and progressive stage . These observations suggest that the ischemia due to mitochondrial angiopathy in arterioles is not, in itself, sufficient to cause SLLs .…”

Section: Discussionmentioning

confidence: 92%

Increased number of mitochondria in capillaries distributed in stroke‐like lesions of two patients with MELAS

et al. 2019

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We investigated two autopsy cases of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes (MELAS) using immunohistochemical staining with an anti‐mitochondrial antibody against translocase of the outer membrane 20 (TOMM20). In case 1, the patient was a 42‐year‐old man with a disease duration of 53 days, and in case 2, the patient was a 62‐year‐old woman with a disease duration of 27 months. In both the cases autopsy revealed moderate atrophy of the cerebrum and cerebellum and multifocal necrotizing lesions, irrespective of the vascular territory. Case 1 showed multiple areas with total necrosis in the cortex, accompanied by increases in number of protoplasmic astrocytes and acidophilic neurons as well as axonal swelling, suggestive of acute or subacute stage stroke‐like lesions (SLLs). In case 2, most of the SLLs displayed laminar spongy change in a rarefied cortex, and were considered to be at the chronic stage. In both the cases, capillary proliferation was noted within the SLLs, particularly in the acute phase. Endothelial cells of proliferating capillaries were strongly positive for TOMM20. In the cortex outside the SLLs, microvessels displayed only a fine granular immunoreactivity, as is seen in the controls. Although smooth muscle cells and endothelial cells in pial arteries and arterioles were also strongly positive for TOMM20, the territories of the affected pial arteries and arterioles did not correlate with the distribution of the SLLs. Although MELAS is characterized by recurrent stroke‐like episodes (SLEs), the pathogenetic relationship between SLEs and mitochondrial angiopathy remains unknown. An aberrant increase of mitochondria in the capillary endothelial cells of SLLs may disturb endothelial function, thus playing a role in the formation or development of SLLs.

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Section: Discussionmentioning

confidence: 92%

Increased number of mitochondria in capillaries distributed in stroke‐like lesions of two patients with MELAS

et al. 2019

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“…Most investigators have reported a relative hyperemia in the affected brain region. 17,18 The two studies that have reported decreased regional perfusion during stroke-like episode 14,19 relied on SPECT scans. Retention of the tracer used in these studies is dependent on the metabolic state of the tissue and the stability of the blood-brain barrier.…”

Section: Discussionmentioning

confidence: 99%

Mitochondrial Encephalomyopathy Lactic Acidosis and Stroke-Like Episodes (MELAS): A Case Report and Critical Reappraisal of Treatment Options

Fryer

Bain

Vivo

2016

Pediatric Neurology

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IMPORTANCE-Stroke-like episodes signal progression and significant disability in the mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes (MELAS) syndrome. Arginine is widely used as a treatment for stroke-like episode, although there is little evidence for this intervention. We discuss the management of a patient with MELAS who presented with a stroke-like episode.OBSERVATION-During a seizure, which triggers the stroke-like episode, neurons are forced to utilize glycolysis as a source of adenosine triphosphate (ATP). Glycolytic byproducts are damaging to the neuron. Breakdown of the blood-brain barrier leads to vasogenic edema.CONCLUSION-Treatment of stroke-like episode should include anticonvulsants interictally to prevent seizures and dexamethasone ictally to help repair the blood-brain barrier.

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“…At the onset, cognitive impairment may be partial, appearing with specific cognitive deficits, particularly in abstract reasoning, verbal memory, visual memory, language (naming and fluency), executive or constructive functions, calculation, attention (attention deficit disorder and decreased attention span), or visuospatial functions [9–12]. Cognitive functions and intellectual abilities may decline from initially focal cognitive impairment to dementia [9–17]. Dementia is defined as chronic and disabling memory impairment, with involvement of at least one other cognitive function, resulting in reduced competence to judge or to reflect [18].…”

Section: Cognitive Impairment In Mitochondrial Disordersmentioning

confidence: 99%

Mitochondria, Cognitive Impairment, and Alzheimer's Disease

Mancuso

Calsolaro

Orsucci

et al. 2009

International Journal of Alzheimer's Disease

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To date, the beta amyloid (Aβ) cascade hypothesis remains the main pathogenetic model of Alzheimer's disease (AD), but its role in the majority of sporadic AD cases is unclear. The “mitochondrial cascade hypothesis” could explain many of the biochemical, genetic, and pathological features of sporadic AD. Somatic mutations in mitochondrial DNA (mtDNA) could cause energy failure, increased oxidative stress, and accumulation of Aβ, which in a vicious cycle reinforce the mtDNA damage and the oxidative stress. Despite the evidence of mitochondrial dysfunction in AD, no causative mutations in the mtDNA have been detected so far. Indeed, results of studies on the role of mtDNA haplogroups in AD are controversial. In this review we discuss the role of the mitochondria, and especially of the mtDNA, in the cascade of events leading to neurodegeneration, dementia, and AD.

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Inappropriate intracranial hemodynamics in the natural course of MELAS

Cited by 32 publications

References 9 publications

Increased number of mitochondria in capillaries distributed in stroke‐like lesions of two patients with MELAS

Increased number of mitochondria in capillaries distributed in stroke‐like lesions of two patients with MELAS

Mitochondrial Encephalomyopathy Lactic Acidosis and Stroke-Like Episodes (MELAS): A Case Report and Critical Reappraisal of Treatment Options

Mitochondria, Cognitive Impairment, and Alzheimer's Disease

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