Abstract
We aimed to describe all the published cases of patients with the previous diagnosis of bAVM that developed ALS at this time. An exploratory research for the describers (“brain arteriovenous malformation” and “amyotrophic lateral sclerosis”) selected four fully available publications. An additional publication was unearthed as a reference in the above-mentioned manuscripts. We found a total of 19 cases in the literature; 47.8% (9/19) were males. The mean age of bAVM diagnosis was 26 (± 2) years, and the time to ALS-related symptoms onset was 14 (± 0.6) years. Sixteen of 19 reported patients, submitted to a mean of 3.8 (± 0.3) embolizations, underwent at least one embolization procedure. Amyotrophic lateral sclerosis (ALS) is a rare, complex devastating neurodegenerative disease. ALS has a genetic component, but it is also associated with environmental factors. A neurovascular hypothesis has been linked to levels of vascular endothelial growth factor (VEGF) in ALS—which emphasizes some reported cases of stroke or brain arteriovenous malformation (bAVM) preceding ALS motor symptoms. Taking this group as a whole, and despite some discrepancies, we suppose that the number of embolizations, site of bAVM, and evidence of perinidal angiogenesis may be related to the development of ALS; however, the association between ALS and bAVM remains without evidence given the small number of published cases.
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Dr. Andrade’s visiting scholarship at Columbia University, New York City, is sponsored by the Capes Foundation, Ministry of Education, Brazil.
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Stefanis, L., de Andrade, J.B.C. & Mohr, J.P. Brain Arteriovenous Malformation and Amyotrophic Lateral Sclerosis: a Review Based on Published Cases. SN Compr. Clin. Med. 2, 392–396 (2020). https://doi.org/10.1007/s42399-020-00231-1
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DOI: https://doi.org/10.1007/s42399-020-00231-1