Highlights
- •Segmental stenosis of major cerebral arteries was found at acute phase of MELAS.
- •SLE lesions were located within the stenotic arteries territory.
- •Dilation at distal portions of the stenotic arteries was also observed.
- •Stenosis was resolved after the acute stage of SLE.
- •Reversible constriction of cerebral arteries may participate in SLE of MELAS.
Abstract
Objective
The pathophysiology of stroke-like episode (SLE) in mitochondrial myopathy, encephalopathy,
lactic acidosis and stroke-like episodes (MELAS) was uncertain, though mitochondrial
metabolic crisis of cortical neurons and mitochondrial proliferation in small vessels
of brain have been considered. However, the involvement of major cerebral vessels
was debated. We aimed to investigate whether major cerebral vessels participate in
SLE.
Methods
We retrospectively collected the clinical and neuroimaging data of MELAS patients
diagnosed in our center. Through follow-up, the cases harboring reversible cerebral
artery constriction on brain magnetic resonance angiography (MRA) examination were
included in this study.
Results
There were 20 patients with intact brain MRA data at acute and non-acute phases. Only
3 cases with m.3243A > G mutation were enrolled. They suffered once or twice SLEs
manifesting headache, blurred vision, seizures or mental and behavior disorder. New
lesions were present in temporo-parietal and/or temporo-occipital regions. Segmental
stenosis at middle cerebral artery and/or posterior cerebral artery, proximal portions
in particular, was ipsilateral to the lesions at acute phase in all the 3 patients,
which was resolved during the subacute or chronic stages. Moreover, the SLEs lesions
were located within the stenotic arteries territory. In addition, dilation at distal
portions of the stenotic arteries was observed at acute phase as well in 2 patients.
Conclusion
Reversible constriction of cerebral arteries may contribute to SLE of MELAS. MELAS
should be a differential diagnosis when stenosis of major cerebral vessels is present
at acute phase of SLE.
Keywords
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Article info
Publication history
Published online: July 19, 2022
Accepted:
July 16,
2022
Received in revised form:
March 18,
2022
Received:
January 29,
2022
Identification
Copyright
© 2022 Elsevier B.V. All rights reserved.
