{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T05:06:55Z","timestamp":1772168815352,"version":"3.50.1"},"update-to":[{"DOI":"10.12688\/f1000research.121554.1","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2022,10,13]],"date-time":"2022-10-13T00:00:00Z","timestamp":1665619200000}},{"DOI":"10.12688\/f1000research.121554.2","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2022,10,13]],"date-time":"2022-10-13T00:00:00Z","timestamp":1665619200000}}],"reference-count":12,"publisher":"F1000 Research Ltd","license":[{"start":{"date-parts":[[2022,10,13]],"date-time":"2022-10-13T00:00:00Z","timestamp":1665619200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["f1000research.com"],"crossmark-restriction":false},"short-container-title":["F1000Res"],"abstract":"<ns3:p>We report a rare case of a vertebro-vertebral arteriovenous fistula (VVAVF) manifesting as amyotrophic lateral sclerosis (ALS). A 76-year-old female patient presented with progressive weakness, muscle atrophy, fasciculation, and preserved deep tendon reflexes in the right upper limb. Electrophysiological testing showed lower motor neuron dysfunction. The patient was suspected to have ALS, but cervical magnetic resonance imaging (MRI) revealed enlarged blood vessels in the spinal canal, which compressed the cervical spinal cord and nerve roots. Angiography showed a shunt from the right vertebral artery to the right intervertebral vein and the vertebral venous plexus; therefore, the patient was diagnosed with VVAVF. Transarterial embolization was performed to obliterate the shunt, and weakness in the patient\u2019s right upper limb subsequently improved. It is worth considering VVAVF as a differential diagnosis of ALS-like diseases.<\/ns3:p>","DOI":"10.12688\/f1000research.121554.3","type":"journal-article","created":{"date-parts":[[2022,10,13]],"date-time":"2022-10-13T06:30:29Z","timestamp":1665642629000},"page":"546","update-policy":"https:\/\/doi.org\/10.12688\/f1000research.crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case Report: Vertebro-vertebral arteriovenous fistula showing symptoms mimicking ALS: Diagnostic imaging supports accurate differentiation between ALS and mimicking conditions"],"prefix":"10.12688","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9419-4077","authenticated-orcid":false,"given":"Hinako","family":"Kirikae","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8698-6218","authenticated-orcid":false,"given":"Ryuhei","family":"Harada","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsuhiko","family":"Hosaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsuro","family":"Misu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daisuke","family":"Ando","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hitoshi","family":"Warita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshiki","family":"Endo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3998-4612","authenticated-orcid":false,"given":"Shinya","family":"Sonobe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kuniyasu","family":"Niizuma","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masashi","family":"Aoki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2560","published-online":{"date-parts":[[2022,10,13]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"1617-1628","DOI":"10.1016\/j.mayocp.2018.04.007","article-title":"Amyotrophic Lateral Sclerosis: An Update for 2018.","volume":"93","author":"B Oskarsson","year":"2018","journal-title":"Mayo Clin. 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Spine."}],"container-title":["F1000Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/f1000research.com\/articles\/11-546\/v3\/xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/11-546\/v3\/pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/11-546\/v3\/iparadigms","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,9,14]],"date-time":"2024-09-14T00:55:33Z","timestamp":1726275333000},"score":1,"resource":{"primary":{"URL":"https:\/\/f1000research.com\/articles\/11-546\/v3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,10,13]]},"references-count":12,"URL":"https:\/\/doi.org\/10.12688\/f1000research.121554.3","relation":{"has-version":[{"id-type":"doi","id":"10.12688\/f1000research.121554.2","asserted-by":"subject"},{"id-type":"doi","id":"10.12688\/f1000research.121554.1","asserted-by":"subject"}],"has-review":[{"id-type":"doi","id":"10.5256\/f1000research.139731.r153200","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.139731.r262993","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.139731.r153200","asserted-by":"object"},{"id-type":"doi","id":"10.5256\/f1000research.139731.r262993","asserted-by":"object"}]},"ISSN":["2046-1402"],"issn-type":[{"value":"2046-1402","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,10,13]]},"assertion":[{"value":"Indexed","URL":"https:\/\/f1000research.com\/articles\/11-546\/v3#article-reports","order":0,"name":"referee-status","label":"Referee status","group":{"name":"current-referee-status","label":"Current Referee Status"}},{"value":"10.5256\/f1000research.133427.r142017, Masafumi Hiramatsu, Department of Neurological Surgery, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, 12 Jul 2022, version 1, 1 not approved","URL":"https:\/\/f1000research.com\/articles\/11-546\/v1#referee-response-142017","order":0,"name":"referee-response-142017","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"<b>Ryuhei Harada<\/b>; \n<i>Posted: 29 Jul 2022<\/i>; Dear Dr. Hiramatsu,  Thank you very much for the thoughtful and constructive feedback. We are thankful for the time and energy you expended. Our responses to your comments are as follow:  \n<b>Q1:<\/b> &quot;\n<i>The authors report a case of VVAVF (Vertebro-vertebral arteriovenous fistula) with ALS-like symptoms. The present case is a typical case of VVAVF and the authors suggest that novelty exists only because ALS was required for differential diagnosis. When there are few abnormal findings in imaging studies, it may be challenging to differentiate the case from ALS.&nbsp;On the other hand, in the present case, the abnormalities are evident in imaging studies, and it is questionable whether ALS should be listed as a differential diagnosis. In the setting of progressive paralysis and muscle atrophy in the upper extremities, imaging studies of cervical spine lesions are often performed before suspecting ALS.&quot;<\/i>  \n<b>A1\uff1a<\/b>Thank you for raising an important point. We agree that the diagnosis of VVAVF was clear from the imaging findings. However, in the progressive course, upper motor neuron signs and lower motor neuron signs were observed, especially muscle atrophy, weakness, and fasciculation, and the clinical findings were mimicking ALS. On the other hand, VVAVF is rare, and the present patient had no past medical history that may be involved in the development of VVAVF (more detailed description in Q2). From this point of view, we considered it to be an instructive case.  \n<b>Q2:<\/b>&nbsp;\n<i>&quot;The presence or absence of trauma or findings suspicious of connective tissue disorder should be noted.&quot;<\/i>  \n<b>A2\uff1a<\/b>Thank you for this suggestion. The patient had no history of trauma other than a history of a fracture of the left upper arm at the age of 68. The patient had no findings suggesting connective tissue diseases (e.g., tissue fragility, hyperextensibility of skin or joints, neurofibroma, elevated inflammatory markers, or collagen disease-related autoantibodies). We have reflected this comment by lines 8-11 from the top of the \u201cCase report\u201d section.  \n<b>Q3: <i>&quot;<\/i><\/b>\n<i>The presence or absence of bruit or findings suspicious of radiculopathy, such as pain and numbness, should be noted.&quot;<\/i>  \n<b>A3\uff1a<\/b>Thank you for this suggestion. After reviewing the imaging findings, a careful review of the physical examination revealed a bruit in the right neck and a slight tingling sensation in the radial side of the right forearm. Symptoms of pain were not present. We have reflected this comment by lines 29-31 from the top of the \u201cCase report\u201d section.  \n<b>Q4: <i>&quot;<\/i><\/b>\n<i>The authors should state whether the findings described in the case report are upper or lower motor neuron dysfunction.&quot;<\/i>  \n<b>A4\uff1a<\/b>Thank you for this suggestion. Preserved reflexes in the right upper extremity with muscle atrophy reflect upper motor neuron dysfunction. Muscle atrophy and fasciculation in the right upper extremity reflect lower motor neuron dysfunction. We have reflected this comment by lines 13-15 from the top of the \u201cCase report\u201d section.  \n<b>Q5:<\/b>&nbsp;&quot;The CTA shows red coiling vessels suggesting intradural reflux of shunt flow. Is there reflux on angiography? Intradural reflux may cause progressive congestive myelopathy. How about the presence of myelopathy on MR imaging and clinical examination?&quot;  \n<b>A5\uff1a<\/b>Thank you for providing these discussions. The red coiling vessels of figure 1C represent anterior and posterior spinal arteries. Angiography did not reveal reflux into them, and their involvement in the arteriovenous shunt was not apparent. We have not observed clinical or imaging findings suggestive of congestive myelopathy. We have reflected this comment by figure legend 1C and lines 36-37 from the top of the \u201cCase report\u201d section.  \n<b>Q6:<\/b>&nbsp;\n<i>&quot;Right vertebral angiography shows the steal phenomenon, but is there any symptom of stealing, such as vertebra-basilar ischemia?&quot;<\/i>  \n<b>A6\uff1a<\/b>Thank you for this suggestion. The patient had no symptoms suggestive of vertebra-basilar ischemia (e.g., vertigo, dizziness, loss of vision, nausea, vomiting, and dysarthria). We have reflected this comment by lines 34-36 from the top of the \u201cCase report\u201d section.  \n<b>Q7:<i> &quot;<\/i><\/b>\n<i>On treatment, the coil mass appears to protrude into the VA; if the coil protrudes into the VA, there is a risk of thrombosis and stroke. It would be better to reconstruct the VA with a stent or occlude the VA completely.&quot;<\/i>  \n<b>A7\uff1a<\/b>Thank you for the important advice. Follow-up angiography after 3 months of treatment showed residual protrusion of the coil mass into the VA, although the blood flow in the VA main trunk was maintained. Therefore, we agree that careful follow-up is needed.  \n<b>Q8:<\/b>&nbsp;\n<i>&quot;What do the authors believe is causing the hyperintensity signal in the anterior horn at the C6\/7 level, spinal cord compression, or myelopathy due to intradural reflux of abnormal shunt flow?&quot;<\/i>  \n<b>A8\uff1a<\/b>Thank you for this suggestion. You have raised an important question. We consider the hyperintensity signal in the anterior horn at the C6\/7 level to be caused primarily by spinal cord compression by dilated blood vessels. The level of intramedullary hyperintensity signal coincidences with the site of the most intense spinal cord compression. It is a finding that supports the mechanism of spinal cord compression. On the other hand, the intramedullary lesion appears too localized to be considered congestive myelopathy. As noted above, there is also no apparent reflux of the anterior and posterior spinal arteries, nor is there apparent regurgitation in the spinal canal. Please see lines 13-23 from the top of the \u201cDiscussion\u201d section.  \n<b>Q9:<\/b>&nbsp;\n<i>&quot;The authors state that the damaged anterior horn leads to partial posttreatment improvement, but I think the mass effect of the coil is the cause of the limitation of the symptom improvement.&quot;<\/i>  \n<b>A9: <\/b>Thank you for providing these insights.  Indeed, I agree with the point that the mass effect of the coils may offset the improvement in spinal cord compression due to the reduction of dilated vessels. However, we have observed improvement in clinical symptoms and imaging findings of spinal cord compression after endovascular treatment.  Again, thank you for giving us the opportunity to strengthen our manuscript with your valuable comments and queries. We have worked hard to incorporate your feedback and hope that these revisions persuade you to accept our submission.  Sincerely, Ryuhei Harada Department of Neurology, Tohoku University Graduate School of Medicine","URL":"https:\/\/f1000research.com\/articles\/11-546\/v3#referee-comment-8580","order":1,"name":"referee-comment-8580","label":"Referee Comment","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.139731.r153200, Corrado Angelini, Neuromuscular Laboratory, Department of Neurosciences, University of Padova, Padua, 17 Nov 2022, version 3, indexed","URL":"https:\/\/f1000research.com\/articles\/11-546\/v3#referee-response-153200","order":2,"name":"referee-response-153200","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.139731.r262993, Matias Costa, Swedish Medical Center, Seattle, 14 Sep 2024, version 3, indexed","URL":"https:\/\/f1000research.com\/articles\/11-546\/v3#referee-response-262993","order":3,"name":"referee-response-262993","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"The author(s) declared that no grants were involved in supporting this work","order":4,"name":"grant-information","label":"Grant Information"},{"value":"This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.","order":0,"name":"copyright-info","label":"Copyright"}]}}