Increased cerebral blood flow in MELAS shown by Tc-99m HMPAO brain SPECT

Peng, Nan‐Jing; Liu, R. S.; Li, J. Y.; Tsay, Daw Guey; Kong, Koon-Wah; Kwok, Cheni; Strauss, H. William

doi:10.1007/s002340050005

Cited by 19 publications

(15 citation statements)

References 6 publications

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“…In patients with an ND1 defect the dopamine transporter SPECT was normal 87 . In MELAS patients with a SLE, the cerebral blood flow has been found increased on Tc-99m HMPAO cerebral SPECT 88 . In acute SLLs hyperperfusion has been documented within the lesions 24 .…”

Section: Single Photon Emission Computed Tomography (Spect)mentioning

confidence: 94%

Central Nervous System Imaging in Mitochondrial Disorders

Finsterer

2009

Can. j. neurol. sci.

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Imaging of central-nervous-system (CNS) abnormalities is important in patients with mitochondrial disorders (MCDs) since the CNS is the organ second most frequently affected in MCDs and some of them are potentially treatable. Clinically relevant imaging techniques for visualization of CNS abnormalities in MCDs are computed tomography, magnetic resonance imaging, and MR-spectroscopy. The CNS abnormalities in MCDs visualized by imaging techniques include stroke-like lesions with cytotoxic or vasogenic edema, laminar cortical necrosis, basal ganglia necrosis, focal or diffuse white matter lesions, focal or diffuse atrophy, intra-cerebral calcifications, cysts, lacunas, hypometabolisation, lactacidosis, hemorrhages, cerebral hypo- or hyperperfusion, intra-cerebral artery stenoses, or moyamoya syndrome. The CNS lesions may proceed with or without clinical manifestations, why neuroimaging should be routinely carried out in all MCDs to assess the degree of CNS involvement. Some of these lesions may remain unchanged for years, some may show contiguous spread and progression, but some may even disappear, spontaneously or in response to medication. Dynamics of Stroke-like lesions may be positively influenced by L-arginine, dichloracetate, steroids, edavarone, or antiepileptics. Symptomatic treatment of CNS abnormalities in MCD patients may positively influence their outcome.

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Section: Single Photon Emission Computed Tomography (Spect)mentioning

confidence: 94%

Central Nervous System Imaging in Mitochondrial Disorders

Finsterer

2009

Can. j. neurol. sci.

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“…Recently there have been a number of radiological studies to determine the mechanisms contributing to the onset of a stroke‐like episode and also to monitor its progression as they can migrate to other regions of the brain. There is evidence that during preclinical or acute onset of a stroke‐like episode, focal hyperperfusion can be detected by either arterial spin labelling or SPECT and can manifest before any structural abnormalities might be seen on MRI . In addition to this, there are reports of vasodilation following magnetic resonance angiogram and impaired cerebrovascular reactivity .…”

Section: Neurological Symptoms and Neuropathologymentioning

confidence: 99%

Review: Central nervous system involvement in mitochondrial disease

Lax

Gorman

Turnbull

2016

Neuropathology Appl Neurobio

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Mitochondrial respiratory chain defects are an important cause of inherited disorders affecting approximately 1 in 5000 people in the UK population. Collectively these disorders are termed ‘mitochondrial diseases’ and they result from either mitochondrial DNA mutations or defects in nuclear DNA. Although they are frequently multisystem disorders, neurological deficits are particularly common, wide‐ranging and disabling for patients. This review details the manifold neurological impairments associated with mitochondrial disease, and describes the efforts to understand how they arise and progressively worsen in patients with mitochondrial disease. We describe advances in our understanding of disease pathogenesis through detailed neuropathological studies and how this has spurred the development of cellular and animal models of disease. We underscore the importance of continued clinical, molecular genetic, neuropathological and animal model studies to fully characterize mitochondrial diseases and understand mechanisms of neurodegeneration. These studies are instrumental for the next phase of mitochondrial research that has a particular emphasis on finding novel ways to treat mitochondrial disease to improve patient care and quality of life.

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“…In addition perfusion‐studies with MRI showed hyperintensity, indicating hyperperfusion in the early stages of the SLL [36]. There are also studies with xenon‐CT, showing increased cerebral blood flow in the subacute or chronic stage from 1 week to 8 months after onset of a SLE [9,31], being attributed to a local increase in lactic acid [45]. Focal hyperperfusion in the acute stage and diffuse hypoperfusion in the chronic stage of a SLL were also found on HMPAO‐SPECT.…”

Section: Imaging Of Sllsmentioning

confidence: 99%

Management of mitochondrial stroke‐like‐episodes

Finsterer

2009

Euro J of Neurology

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Aim of this review is to discuss recent findings concerning the management of stroke-like episodes (SLEs) in patients with mitochondrial disorders (MIDs). Various databases were searched for appropriate literature. SLEs are a dominant feature of MIDs and occur most frequently in MELAS-syndrome, less frequently in MERRF-syndrome, Kearns-Sayre-syndrome, or Leigh-syndrome. SLEs occur at all ages and are frequently accompanied by other cerebral abnormalities. Clinically, SLEs mimic ischemic stroke but not on imaging studies and concerning the management. The morphological equivalent on MRI is the stroke-like-lesion, representing a vasogenic edema (hyperintensity on T2, diffusion-weighted imaging and apparent diffusion coefficient in the acute and subacute or chronic stage, most frequently in the parieto-occipital region, surpassing vascular territories). For diagnostic and therapeutic reasons SLEs need to be clearly delineated from ischemic stroke and cerebral bleeding. Though there is no causal therapy available, symptomatic and general measures can help to resolve the clinical manifestations. In conclusion this review shows that SLEs are a dominant feature of some syndromic or non-syndromic MIDs. The most effective strategy for the treatment of SLEs appears to be the application of L-arginine, coenzyme-Q, steroids, edaravone, creatine-monohydrate, or dichloracetate.

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Increased cerebral blood flow in MELAS shown by Tc-99m HMPAO brain SPECT

Cited by 19 publications

References 6 publications

Central Nervous System Imaging in Mitochondrial Disorders

Central Nervous System Imaging in Mitochondrial Disorders

Review: Central nervous system involvement in mitochondrial disease

Management of mitochondrial stroke‐like‐episodes

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