CT and MRI imaging of the brain in MELAS syndrome

Pauli, Wojciech; Zarzycki, Artur; Krzyształowski, A; Walecka, Anna

doi:10.12659/pjr.884010

Cited by 31 publications

(14 citation statements)

References 13 publications

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“…Focal hypodensities with atrophy are commonly seen on CT scans of childhood-onset MELAS syndrome [16], [17], [18]. Global atrophy and calcification of the basal ganglia are sometimes present [16].…”

Section: Discussionmentioning

confidence: 99%

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Migratory stroke-like lesions in a case of adult-onset mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome and a review of imaging findings

Cai

Coelln

Kouo

2016

Radiology Case Reports

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Imaging findings of adult-onset mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is poorly documented. The authors present a 48-year-old woman with subacute onset of word-finding difficulties and right arm stiffness. Magnetic resonance imaging performed 2 weeks prior revealed left temporal lobe diffusion and fluid-attenuated inversion recovery hyperintensity predominantly involving the cortex. The apparent diffusion coefficient map showed preserved signal in the temporal cortex. Subsequent magnetic resonance imagings demonstrated a new diffusion signal abnormality extending to the left parietal cortex and occipital cortex with resolving diffusion hyperintensity in the temporal lobe. MR spectroscopy showed scattered areas of lactate deposition. Diagnosis of MELAS syndrome was confirmed by genetic analysis. Fluctuating, migratory stroke-like lesions with a predilection for the parietal, temporal, and occipital cortex that do not conform to a vascular territory and a lactate spike at 1.3 ppm on MR spectroscopy are characteristic of MELAS syndrome. Preserved signal intensity on apparent diffusion coefficient is useful to distinguish MELAS syndrome from ischemic infarction where the signal is typically reduced.

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Section: Discussionmentioning

confidence: 99%

“…These diffusion signal abnormalities are likely to resolve over time, paralleling clinical improvement, while new lesions may develop with or without new symptoms. This migratory, waxing and waning pattern of stroke-like lesions on imaging is a cardinal feature of MELAS syndrome [16], [18], [23]. Pattern of ADC mapping is variable.…”

Section: Discussionmentioning

confidence: 99%

Migratory stroke-like lesions in a case of adult-onset mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome and a review of imaging findings

Cai

Coelln

Kouo

2016

Radiology Case Reports

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“…149 The characteristics of mitochondrial strokes are different from those of thrombotic events, primarily in that they do not conform to vascular territories. [150][151][152][153][154][227][228][229][230] MELAS syndrome (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes) typically presents in a previously normal child with a history of migrainous headaches and vomiting. [230][231][232] Children with MELAS often have short stature and a history of seizures.…”

Section: Systemic Causes: Inflammatory and Genetic/metabolicmentioning

confidence: 99%

Management of Stroke in Neonates and Children: A Scientific Statement From the American Heart Association/American Stroke Association

Ferriero¹,

Fullerton²,

Bernard³

et al. 2019

Stroke

526

515

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Purpose-Much has transpired since the last scientific statement on pediatric stroke was published 10 years ago. Although stroke has long been recognized as an adult health problem causing substantial morbidity and mortality, it is also an important cause of acquired brain injury in young patients, occurring most commonly in the neonate and throughout childhood. This scientific statement represents a synthesis of data and a consensus of the leading experts in childhood cardiovascular disease and stroke. Methods-Members of the writing group were appointed by the American Heart Association Stroke Council's Scientific Statement Oversight Committee and the American Heart Association's Manuscript Oversight Committee and were chosen to reflect the expertise of the subject matter. The writers used systematic literature reviews, references to published clinical and epidemiology studies, morbidity and mortality reports, clinical and public health guidelines, authoritative statements, personal files, and expert opinion to summarize existing evidence and to indicate gaps in current knowledge. This scientific statement is based on expert consensus considerations for clinical practice. Results-Annualized pediatric stroke incidence rates, including both neonatal and later childhood stroke and both ischemic and hemorrhagic stroke, range from 3 to 25 per 100 000 children in developed countries. Newborns have the highest risk ratio: 1 in 4000 live births. Stroke is a clinical syndrome. Delays in diagnosis are common in both perinatal and childhood stroke but for different reasons. To develop new strategies for prevention and treatment, disease processes and risk factors that lead to pediatric stroke are discussed here to aid the clinician in rapid diagnosis and treatment. The many important differences that affect the pathophysiology and treatment of childhood stroke are discussed in each section. Conclusions-Here we provide updates on perinatal and childhood stroke with a focus on the subtypes, including arterial ischemic, venous thrombotic, and hemorrhagic stroke, and updates in regard to areas of childhood stroke that have not received close attention such as sickle cell disease. Each section is highlighted with considerations for clinical practice, attendant controversies, and knowledge gaps. This statement provides the practicing provider with much-needed updated information in this field. (Stroke. 2019;50:e00-e00.

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“…Yoshida et al, reported a patient with 2 episodes of status epilepticus (similar to the patient in the current report) and suggested that a more protracted course of seizures with a persistent altered mental status was concomitant with larger brain lesions ( Yoshida et al, 2013 ). On the other hand, stroke-like episodes as the most common clinical features, and the corresponding changes in brain imaging are very helpful to guide clinicians to correctly diagnose MELAS syndrome ( Pauli, Zarzycki, Krzysztalowski, & Walecka, 2013 ).…”

Section: Discussionmentioning

confidence: 99%

A Mitochondrial Disorder in a Middle Age Iranian Patient: Report of a Rare Case

Almasi

Motamed

Mehrpour

et al. 2017

BCN

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Introduction:Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) can involve multiple systems and cause stroke-like episodes and status epilepticus.Case Presentation:A 48-year-old female with history of early fatigability, migraine-type headaches, and bilateral sensory-neural hearing loss presented 3 episodes of serial seizures. On admission she was affected by Wernicke aphasia and, then, right hemiparesis. Investigations showed elevated arterial lactate and ragged red fibers on muscle biopsy.Conclusion:Though more commonly diagnosed during childhood, some cases of adult-onset MELAS syndrome are reported. This syndrome should be considered in patients with stroke-like events in adults without cerebrovascular risk factors and difficult-to-treat seizures.

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CT and MRI imaging of the brain in MELAS syndrome

Cited by 31 publications

References 13 publications

Migratory stroke-like lesions in a case of adult-onset mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome and a review of imaging findings

Migratory stroke-like lesions in a case of adult-onset mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome and a review of imaging findings

Management of Stroke in Neonates and Children: A Scientific Statement From the American Heart Association/American Stroke Association

A Mitochondrial Disorder in a Middle Age Iranian Patient: Report of a Rare Case

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