Abstract
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.
Highlights
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) is a maternally inherited disorder caused by mitochondrial DNA or nuclear DNA mutations in a diffuse multisystemic fashion
We review the conventional findings and the latest application of Magnetic resonance imaging (MRI) in MELAS syndrome (Table 1)
Earlier reports suggested that the ADC value of stroke-like lesions (SLLs) was normal or increased [20, 21], but most studies have found that the ADC signals alternately change or mix in different periods [18, 22–24] (Figure 1F)
Summary
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) is a maternally inherited disorder caused by mitochondrial DNA (mtDNA) or nuclear DNA (nDNA) mutations in a diffuse multisystemic fashion. Part of cortical lesions show patchy or linear enhancement on T1-weighted postcontrast images (Figure 1C), due to local exudation or circulation disorders caused by the breakdown of the blood-brain barrier and increased regional cerebral blood flow in the affected areas [11].
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