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Major cerebral vessels involvement in patients with MELAS syndrome: Worth a scan? A systematic review
Laura Ludovica Gramegna1, Irene Cortesi2, Micaela Mitolo1
1IRCCS Istituto delle Scienze Neurologiche di Bologna, Functional and Molecular Neuroimaging Unit, Bologna, Italy; Department of Biomedical and NeuroMotor Sciences, University of Bologna, Bologna, Italy.
Abstract:
Major cerebral vessels have been proposed as a target of defective mitochondrial metabolism in patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome (MELAS). Cerebral angiographic techniques are not routinely performed in MELAS patients. A systematic literature review was performed to identify studies describing major vessel caliber alterations in MELAS. Twenty-three studies reporting on 46 MELAS patients were included. Alterations in major caliber vessels were present in 59% (27/46) of patients. Dilation occurred in 37% (17/46) of patients, and in 88% (15/17) of them during a stroke-like episode (SLE). Stenosis was reported in 24% (11/46) of patients: 36% (4/11) related to an SLE and 64% (7/11) to dissections or degenerative changes. During an SLE, identification of intracranial vessels dilation or stenosis could be a selection tool for new treatment protocols. Outside SLE, identification of major cerebral vessels dissections and degenerative changes may help to prevent subsequent complications.
Insights
Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) patients frequently exhibit cerebral vessel caliber changes. Dilation often occurs during stroke-like episodes, while stenosis may indicate dissections or degenerative issues.
Area of Science:
- Neurology
- Mitochondrial Diseases
- Vascular Medicine
Background:
- Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a mitochondrial disorder.
- Defective mitochondrial metabolism is implicated in MELAS, potentially affecting cerebral vessels.
- Cerebral angiography is not standard in MELAS patient care.
Purpose of the Study:
- To systematically review literature for major cerebral vessel caliber alterations in MELAS patients.
- To correlate vessel changes with clinical events like stroke-like episodes (SLEs).
Main Methods:
- Systematic literature review.
- Inclusion of 23 studies with 46 MELAS patients.
- Analysis of reported major vessel caliber alterations.
Main Results:
- 59% of MELAS patients showed major vessel caliber alterations.
- Dilation occurred in 37% of patients, often during SLEs (88%).
- Stenosis was found in 24%, linked to SLEs (36%) or dissections/degeneration (64%).
Conclusions:
- Cerebral vessel dilation or stenosis during SLEs in MELAS could guide new treatment protocols.
- Identifying dissections and degenerative changes outside SLEs may prevent complications.

