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Reversible Dilation of Cerebral Macrovascular Changes in MELAS Episodes

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Abstract

Purpose

To investigate the cerebral macrovascular changes as well as the relationship of large vessels and cerebral blood flow (CBF) in mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) using magnetic resonance angiography (MRA) and arterial spin labeling (ASL) perfusion MR imaging (MRI).

Methods

A total of 20 patients diagnosed with MELAS (12 males, 8 females; mean age, 23.3 years) underwent conventional MRI, time-of-flight (TOF) MRA and three dimensional ASL. Follow-up scans were performed in 10 patients. The changes of cerebral arteries and branches on MRA images from both acute and recovery patients were independently evaluated by two radiologists. Lesion distribution and CBF were observed on the integrated maps of MRA and ASL.

Results

In 14 patients with clinical onsets, increased CBF was observed in all stroke-like lesions. Dilations of a single artery (four middle cerebral arteries, two posterior cerebral arteries) were found in six patients. Dilations of multiple arteries (two anterior cerebral arteries, seven middle cerebral arteries, six posterior cerebral arteries) were found in seven patients. Normal angiography was shown in one acute patient. Cortical terminal branches feeding the lesion areas were more obviously dilated than the main trunks. The dilated vessels returned to normal on follow-up scans concurrently with decreased CBF in nine patients who were resuscitated from episode attacks. Vasodilation was even seen in one preclinical patient who suffered a recurrent episode 50 days later.

Conclusion

Reversible dilation of cerebral macrovascular changes could be a new feature of MELAS and a presumed reason for fluctuant CBF. It would shed new light on the mitochondrial angiopathy.

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Acknowledgements

We would like to thank all patients who participated in this study. We also acknowledge the grants from the National Natural Science Foundation of China (No. 81301203, 81401035) which supported this study.

Author information

Authors and Affiliations

  1. Department of Radiology, Huashan Hospital, Fudan University, 12 Middle Wulumuqi Road, Shanghai, China

    Yuxin Li, Weixingzi Xu, Jiawen Cao, Haiqing Li & Liqin Yang

  2. Institute of Functional and Molecular Medical Imaging, Fudan University, 12 Middle Wulumuqi Road, Shanghai, China

    Yuxin Li & Liqin Yang

  3. Department of Neurology, Huashan Hospital, Fudan University, 12 Middle Wulumuqi Road, Shanghai, China

    Chong Sun & Jie Lin

  4. Advanced Application, GE Healthcare, Shanghai, China

    Jianxun Qu

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  1. Yuxin Li
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  2. Weixingzi Xu
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  3. Chong Sun
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  4. Jie Lin
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Corresponding author

Correspondence to Yuxin Li.

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Conflict of interest

Y. Li, W. Xu, C. Sun, J. Lin, J. Qu, J. Cao, H. Li and L. Yang declare that they have no competing interests.

Ethical standards

All investigations performed on humans in this study were carried out in accordance with national law and the Helsinki Declaration from 1964 (in its current revised form). This study was approved by the ethics committee of our hospital. Informed consent was obtained from each patient.

Additional information

Dr. Weixingzi Xu and Dr. Chong Sun both participated in the project design and revised the manuscript. In addition, Dr. Xu analyzed the imaging data and Dr. Sun managed the patients.

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62_2018_662_MOESM1_ESM.pdf

ESM-Fig. 1 MRA images (inferior-superior view) of each patient with MELAS at the initial MR scans; Fig. 2 MRA images (inferior-superior view) of ten patients with MELAS at the follow-up MR scans

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Li, Y., Xu, W., Sun, C. et al. Reversible Dilation of Cerebral Macrovascular Changes in MELAS Episodes. Clin Neuroradiol 29, 321–329 (2019). https://doi.org/10.1007/s00062-018-0662-8

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  • DOI: https://doi.org/10.1007/s00062-018-0662-8

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