Thrombectomy for cerebral infarction caused by carotid muscle fiber dysplasia: a case report

Thrombectomy for cerebral infarction caused by carotid muscle fiber dysplasia: a case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Thrombectomy for cerebral infarction caused by carotid muscle fiber dysplasia: a case report Liu Xiaodong, XU Ping This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4336295/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background Fibromuscular dysplasia (FMD) associated with cerebrovascular diseases represents a rare etiology of stroke in adolescents. The initial symptoms of FMD lack specificity, and the clinical comprehension of cerebral vascular FMD remains inadequate, leading to potential misdiagnosis or underdiagnosis. Therefore, early diagnosis coupled with comprehensive cerebrovascular imaging holds paramount importance. Case presentation The present report delineates a case of acute-onset stroke-like symptoms in a 26-year-old female adult patient. Notably, the patient exhibited no pre-existing risk factors for cerebral hemopathy and was devoid of any inducing factors such as drug abuse, long-term hormone use, or neck massage. Additionally, there was no history of fever or related conditions. Acute cerebral infarction was confirmed through head MRI findings. Subsequent improved cerebral angiography revealed occlusion of the right carotid artery and beaded changes in the left carotid artery, leading to the diagnosis of muscle fiber dysplasia (FMD) followed by right arterial thrombectomy. Conclusion Carotid myofibrous dysplasia (FMD), some of which may present with stroke-like symptoms, typically exhibits atypical manifestations and is prone to misdiagnosis. Therefore, it is crucial to integrate the patient's overall condition, clinical symptoms, and appropriate imaging diagnosis in the early stages to expedite triage. In cases of carotid artery occlusion caused by FMD, emergency thrombectomy can be performed for prompt vascular recanalization. Fibromuscular dysplasia Cerebral infarction Thrombectomy of cerebral artery Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Introduction Fibromuscular dysplasia (FMD) is a distinctive lesion of idiopathic, segmental non-inflammatory and non-atherosclerotic diseases, characterized by aberrant cellular proliferation and distortion of arterial wall structure [ 1 ].The etiology remains unclear, with genetic factors, environmental influences (such as smoking), and estrogen being the primary contributors to its pathogenesis [ 2 ], [ 3 ].The main pathological manifestations encompass multifocal or focal lesions in medium-sized and small arteries, which may involve vessel stenosis, aneurysm formation, and dissection [ 4 ]. The clinical manifestations of fibromuscular dysplasia (FMD) vary depending on the specific vascular bed affected and the severity of vascular stenosis. A previous study reported that approximately 17.9% of patients experience transient ischemic attack (TIA) or symptoms resembling an ischemic stroke as their initial presentation [ 5 ]. Here, we present a case report describing carotid artery occlusion resulting from FMD and subsequent administration of emergency thrombolysis. Case The 26-year-old female patient was admitted to the hospital on August 26, 2022, presenting with sudden slurred speech accompanied by left limb weakness persisting for a duration of 10 hours. Ten hours prior to admission, she experienced abrupt onset of dysarthria and expressive communication difficulties. She exhibited partial compliance with motor instructions but did not present any hearing or comprehension impairments. Additionally, she displayed weakness in her left limb, inability to elevate the left upper limb, inability to stand or walk using the left lower limb, disturbances in consciousness, difficulty swallowing and coughing while drinking water. Furthermore, she reported symptoms such as headache, tinnitus, dizziness, chest tightness palpitations and a history of neck massage. The patient had no significant medical history and denied any family history of similar conditions. Upon physical examination upon admission: body temperature was measured at T36.3℃; heart rate at P68 beats per minute; respiratory rate at R20 breaths per minute; blood pressure at Bp103/61mmHg; oxygen saturation level (SPO2) recorded as 98%. Cardiopulmonary and abdominal examinations yielded negative findings. Neurological examination revealed clear mental status with dysarthria observed but normal higher cognitive function intact without detection of vascular murmurs over the abdomen or costal ridge angle.Systolic murmur is audible in the right internal carotid artery. There is left central facial paralysis, left limb muscle strength grade 0, and no bilateral pathological signs emerged. The NIHSS score is 11 points (4:2 points + 5a: 4 points + 6a: 4 points + 9:1 point), GCS score is 15 points, swallowing dysfunction rating is 4 points, and mRS Score is 0 points. Auxiliary tests including routine blood test, coagulation function test, liver and kidney function test, electrolyte test, myocardial enzyme test, BNP test, erythrocyte sedimentation rate test, female hormone level checkup, blood lipid level checkup, anti-streptococcal hemolysin "O" antibody detection, rheumatoid factor detection, C-reactive protein detection and autoantibody system column detection showed no obvious abnormalities found. Cervical vascular ultrasound revealed diffuse thickening of the longitudinal section of the middle and left common carotid artery wall with concentric ring thickening observed in transverse section causing local lumen stenosis with stenosis rates of approximately 73% (right) and 60% (left). Figure 1 a depicts acute infarction in the right basal ganglia and paraventricular area on head magnetic resonance imaging. Figure 1 b demonstrates occlusion of the right internal carotid artery and middle cerebral artery on MRA of the head. Emergency digital subtraction angiography (DSA) suggests occlusion of the right common carotid artery, which was not visualized, resulting in mTICI class 0 flow impairment in the right hemisphere. Brain artery imaging reveals beaded changes indicative of fibromuscular dysplasia in the left internal carotid artery. The left anterior traffic is patent, while partial compensation for supply to the right middle cerebral artery territory occurs through pIA meningeal arteries. The left external carotid artery is occluded with a blind end formation and absence of forward blood flow observed. Compensation for supply to the right internal carotid artery territory via occipital arteries is provided by the right vertebral artery, whereas compensation for supply to the left external carotid artery territory occurs through occipital arteries supplied by the left vertebral artery. Partial compensation for supply to the right middle cerebral artery territory via pial arteries is facilitated by the right posterior cerebral artery (Fig. 2 ). Further super-selective cerebral angiography was performed, followed by intracranial artery thrombolectomy. Under local anesthesia, the 8F support catheter was advanced to the occluded blind end of the right common carotid artery to restore blood flow. Due to difficulties in advancing the suction catheter under negative pressure and considering orthotopic stenosis, balloon dilation was employed using coaxial technology to reach the semi-released state in the C2 segment of the internal carotid artery. The black-red thrombus (20×5×5mm) was successfully aspirated using ADAPT technology, resulting in revascularization of the M1 segment. Considering severe stenosis at proximal carotid artery, balloon dilation was performed with a thrombus protection device. After a 10-minute observation period confirming maintained forward blood flow (Fig. 3 ), stent implantation was deemed unnecessary. Physical examination on the first postoperative day revealed stable vital signs, bilateral pupils of 3.0mm in diameter with intact light reflex, clear speech, absence of facial palsy, left limb muscle strength at level 5-, right limb muscle strength at level 5, normal muscle tension, and no evidence of bilateral pathological signs. The NIHSS score was 0 and GCS score was 15. Subsequent MRA reexamination showed a right embryonic posterior communicating artery. Acute lacunar infarction in the right basal ganglia was observed on head MRI (Fig. 4 a-c). Three months after surgery, a small lamellar encephalomalacia lesion adjacent to the right lateral ventricle was detected on head MRI (Fig. 4 a-c). The patient exhibited normal speech and limb function with an NIHSS score of 0 and mRS Score of 0. Six months later, head and neck vascular CT revealed soft plaques causing beaded stenosis in both proximal carotid arteries (severe stenosis on the right side and moderate stenosis on the left side) (Fig. 5 ). Discussion Myofibrous dysplasia (FMD) is a rare disease characterized by stenosis, occlusion of affected blood vessels, and the development of aneurysms or arterial dissection. FMD can involve small and medium arteries throughout the body, with renal artery involvement observed in approximately 90.7% of patients, carotid artery involvement in 25–30% of cases, and less frequent involvement of intracranial arteries (8.3%). Additionally, FMD may affect other arteries such as the mesenteric artery, iliac artery, and brachial artery [ 1 ]. The condition predominantly affects women aged between 20 and 60 years old, with a male to female ratio of about 1:9; however, men tend to have a less common but more aggressive course of FMD [ 6 ]. Due to challenges in obtaining pathology samples for diagnosis purposes, digital subtraction angiography (DSA) remains the gold standard diagnostic tool for FMD. Pathologically classified into mesial type, intimal type, and outer type changes; the mesial type accounts for approximately 75%-80%. DSA reveals "beaded changes" where the diameter of these beads exceeds that of normal blood vessels primarily affecting the middle and distal segments. The mesial type represents around 10%-15%, presenting fewer beads on DSA with smaller diameters than normal arteries often accompanied by abundant collateral circulation. Intimal DSA demonstrates smooth localized eccentric or centripetal vessel stenosis or long segment smooth stenosis resembling arteritis [ 7 ]. Based on patient's DSA examination findings it was determined that they had mesomembrane-type FMD. The common clinical manifestations of cerebrovascular fibromuscular dysplasia (FMD) include: (1) severe and/or chronic migraine, particularly when accompanied by other suggestive symptoms or signs; (2) pulsatile tinnitus characterized by a "whoosh" sound in the ear synchronized with the heartbeat; (3) presence of a neck murmur; (4) stroke-like symptoms such as hemiplegia, aphasia, limb sensory disorders, transient ischemic attack (TIA), or temporary loss of vision; unilateral head and neck pain or focal neurological manifestations like partial Horner syndrome with drooping eyelid and pupil constriction [ 8 ]. Headache that is not classified as chronic migraine or non-migraine type, non-throbbing tinnitus, mild vertigo-headache combination may indicate possible carotid artery dissection. According to the European FMD Consensus guidelines: (1) screening for FMD should be conducted in women under 50 years old who have hypertension; (2) imaging screening for head-neck vascular abnormalities should be performed in cases of pulsatile tinnitus, cervical artery or intracranial artery dissection, intracranial aneurysm, subarachnoid hemorrhage (SAH), and cerebral ischemic events; (3). Computed tomography angiography (CTA) or magnetic resonance angiography(MRA) are preferred imaging modalities; 4. When head-neck FMD is detected on imaging studies further evaluation for renal artery involvement should be considered. Cerebral artery imaging examination plays a crucial role in diagnosing FMD. The luminal imaging findings associated with FMD include: bead-like lumen changes,(2). segmental stenosis which is rare but can present as either short centripetal stenosis or longer tubular stenosis,(3)."web-like" filling defect.(4). Diverticular type. Currently known causes of fibromuscular dysplasia (FMD) include genetic factors, environmental factors such as smoking, and estrogen levels. In this particular case, the patient did not smoke and had normal estrogen levels; however, genetics cannot be ruled out as a potential cause. FMD susceptibility genes like PHACTR1, LRP1, ATP2B1, and LIMA1 can be further screened in patients with familial aggregation tendency [ 9 ]. FMD affects various parts of the cerebrovascular system and its associated symptoms vary accordingly. Patients without obvious symptoms should undergo regular monitoring. For FMD patients with endovascular lesions such as vascular stenosis, occlusion, dissection, aneurysm or malformation, appropriate endovascular treatment can be considered based on individual conditions. In the absence of contraindications, antiplatelet therapy (e.g., daily aspirin dose of 75-100mg) is recommended for preventing blood clots and thromboembolic complications in FMD patients [ 10 ]. Conclusion Currently, there is a paucity of literature on FMD, as the disease lacks specific clinical manifestations. The early symptoms fail to capture the attention of young patients, particularly those experiencing headaches, dizziness, tinnitus, and other related symptoms. Consequently, these symptoms are prone to misdiagnosis leading to severe cerebrovascular accidents. For young individuals suffering from strokes, it is crucial to obtain an accurate medical history and conduct appropriate imaging evaluations in order to expedite access to specialized care and achieve favorable prognoses. Declarations Ethics approval and consent to participate Written informed consent was obtained from the patients for the publication of any potentially identifiable images or data included in this article. Consent for publication The patient signed informed consent forms. Data and materials availability Data is provided within the manuscript. Competing interests The authors declare no competing interests. Funding No funding. Author Contribution All the authors contributed to this article.Liu was responsible for writing the article and collecting pictures, and Xu supervised and revised the manuscript. Acknowledgements We would like to thank the participants and their families for their contributions to this work. The authors did not receive funding for this research. References Bender MT, et al. Safety Assessment of Endovascular Treatment of Cerebral Aneurysms in Patients with Fibromuscular Dysplasia. Interv Neurol. 2018;7(1–2):110–7. Persu A, et al. Current progress in clinical, molecular, and genetic aspects of adult fibromuscular dysplasia. Cardiovasc Res. 2022;118(1):65–83. Gornik HL, et al. First International Consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med. 2019;24(2):164–89. Khoury MH, Gornik HL. Fibromuscular dysplasia (FMD). Vasc Med. 2017;22(3):248–52. De Groote M, et al. Fibromuscular dysplasia - results of a multicentre study in Flanders. Vasa. 2017;46(3):211–8. Olin JW, et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation. 2012;125(25):3182–90. First international consensus on the. diagnosis and management of fibromuscular dysplasia: Erratum. J Hypertens. 2021;39(9):1932. First international consensus on the. diagnosis and management of fibromuscular dysplasia: Erratum. J Hypertens. 2019;37(5):1098. Georges A, et al. Genetic investigation of fibromuscular dysplasia identifies risk loci and shared genetics with common cardiovascular diseases. Nat Commun. 2021;12(1):6031. Lun R, et al. Comparison of Ticagrelor vs Clopidogrel in Addition to Aspirin in Patients With Minor Ischemic Stroke and Transient Ischemic Attack: A Network Meta-analysis. JAMA Neurol. 2022;79(2):141–8. Additional Declarations No competing interests reported. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4336295","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":298946961,"identity":"8a11fa83-5f5a-4179-a9fb-9497c3162109","order_by":0,"name":"Liu Xiaodong","email":"","orcid":"","institution":"Zunyi Medical University","correspondingAuthor":false,"prefix":"","firstName":"Liu","middleName":"","lastName":"Xiaodong","suffix":""},{"id":298946963,"identity":"44e2540f-df2c-481f-9594-51f71d83e6d7","order_by":1,"name":"XU Ping","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA90lEQVRIiWNgGAWjYJCCAwwM/+TsjzeAGAw8xGo5YMxw5gAJWkC6EhtuJBCp1uBG7sHDBb/uMDbOfGN4uIChTsac/QDjh485uLVIzshLODyz7xkzs3SOweEZDId5LHsSmCVnbsOthV8CqJK3h5mNTTot4TAPwwEegwMJbMy8eLSwQbXw8EgeA2mp4zE4/wC/FrAtPD8OS0hIMB8AamHmMbhBwBbJnncJh3kb0gwMeJIPHJ4B1G5w42EzXr8YHM89/Jnnj039BvaDzZ8LKursDc4nH/zwEY8WcNwxtkGYzAwGIIqxAZ96iBaGPzAto2AUjIJRMAqwAABiplLvUMgOWAAAAABJRU5ErkJggg==","orcid":"","institution":"Zunyi Medical University","correspondingAuthor":true,"prefix":"","firstName":"XU","middleName":"","lastName":"Ping","suffix":""}],"badges":[],"createdAt":"2024-04-28 05:26:54","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4336295/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4336295/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":56123162,"identity":"849beb00-b6b1-4877-8a68-343b666bbf47","added_by":"auto","created_at":"2024-05-08 20:43:23","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":515460,"visible":true,"origin":"","legend":"\u003cp\u003eHead SWI+MRA \u003cstrong\u003ea. \u003c/strong\u003eThe head magnetic resonance imaging revealed acute infarction in the right basal ganglia and lateral ventricle, while the head magnetic resonance angiography demonstrated occlusion of both the right internal carotid artery and middle cerebral artery.\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-4336295/v1/b8b7a461c74e1f7b5bc987a4.png"},{"id":56123166,"identity":"355eec26-880d-47b2-90eb-ebfc887b040b","added_by":"auto","created_at":"2024-05-08 20:43:23","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":525105,"visible":true,"origin":"","legend":"\u003cp\u003ePreoperative DSA: The right common carotid artery was occluded, no forward blood flow was observed, mTICI grade was 0, no development was observed in the right middle cerebral artery and anterior cerebral artery, and the left internal carotid artery was fine with beaded changes, considering muscle fiber dysplasia, the left anterior traffic was open, and the right middle cerebral artery was partially compensated by the pIA meningeae artery, the left external carotid artery was occluded, and the blind end was formed. No forward flow was observed. The right vertebral artery compensated for the right internal carotid artery supply area via the occipital artery, the left vertebral artery compensated for the left external carotid artery via the occipital artery, and the right posterior cerebral artery partially compensated for the right middle cerebral artery supply area via the pia meningeal artery.\u003c/p\u003e","description":"","filename":"floatimage2.png","url":"https://assets-eu.researchsquare.com/files/rs-4336295/v1/c2e162ace3739e0f374a497e.png"},{"id":56123163,"identity":"9b7d6f5f-7270-4824-a0dd-9ca7e2f610c7","added_by":"auto","created_at":"2024-05-08 20:43:23","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":455711,"visible":true,"origin":"","legend":"\u003cp\u003ePostoperative DSA: The recirculation of the right middle cerebral artery was observed, indicating a well-developed vascular network encompassing the right middle cerebral artery and its branches. A long segment stenosis was identified in the right common carotid artery, while irregularities were noted in the endometrium. Approximately 50% residual stenosis was present with satisfactory forward flow. Furthermore, excellent development of the right internal carotid artery, middle cerebral artery, anterior cerebral artery and their respective branches was observed. The overall angiographic outcome can be classified as mTICI grade 3.\u003c/p\u003e","description":"","filename":"floatimage3.png","url":"https://assets-eu.researchsquare.com/files/rs-4336295/v1/6304a11c5df5f9bb75bd7c0f.png"},{"id":56123164,"identity":"f2b91358-e42f-44e6-a8ae-c3e985a63985","added_by":"auto","created_at":"2024-05-08 20:43:23","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":485447,"visible":true,"origin":"","legend":"\u003cp\u003eHead MRI was performed on the first day and at 3 months post-surgery. Images a-c depict acute lacunar cerebral infarction in the right basal ganglia immediately after surgery, while images d-f reveal small lamellar encephalomalacia in the right lateral ventricle three months later.\u003c/p\u003e","description":"","filename":"floatimage4.png","url":"https://assets-eu.researchsquare.com/files/rs-4336295/v1/eabc38d2c5fd83c800b9680e.png"},{"id":56123165,"identity":"8908fdde-22dd-491d-9402-13a37eb17125","added_by":"auto","created_at":"2024-05-08 20:43:23","extension":"png","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":127175,"visible":true,"origin":"","legend":"\u003cp\u003eHead and neck vascular CT scan conducted 6 months post-surgery revealed bilateral common carotid artery proximal soft plaque with beaded stenosis, characterized by severe stenosis on the right side and moderate stenosis on the left side.\u003c/p\u003e","description":"","filename":"floatimage5.png","url":"https://assets-eu.researchsquare.com/files/rs-4336295/v1/6009c35a96676c50901a4129.png"},{"id":64231878,"identity":"1d6c6d99-e8cf-4a8e-9825-b53d337f8fa2","added_by":"auto","created_at":"2024-09-10 15:16:56","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":3313903,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4336295/v1/5688cef7-f661-482b-9a71-27cb09855c14.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Thrombectomy for cerebral infarction caused by carotid muscle fiber dysplasia: a case report","fulltext":[{"header":"Introduction","content":"\u003cp\u003eFibromuscular dysplasia (FMD) is a distinctive lesion of idiopathic, segmental non-inflammatory and non-atherosclerotic diseases, characterized by aberrant cellular proliferation and distortion of arterial wall structure [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e].The etiology remains unclear, with genetic factors, environmental influences (such as smoking), and estrogen being the primary contributors to its pathogenesis [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e], [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].The main pathological manifestations encompass multifocal or focal lesions in medium-sized and small arteries, which may involve vessel stenosis, aneurysm formation, and dissection [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe clinical manifestations of fibromuscular dysplasia (FMD) vary depending on the specific vascular bed affected and the severity of vascular stenosis. A previous study reported that approximately 17.9% of patients experience transient ischemic attack (TIA) or symptoms resembling an ischemic stroke as their initial presentation [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Here, we present a case report describing carotid artery occlusion resulting from FMD and subsequent administration of emergency thrombolysis.\u003c/p\u003e "},{"header":"Case","content":"\u003cp\u003eThe 26-year-old female patient was admitted to the hospital on August 26, 2022, presenting with sudden slurred speech accompanied by left limb weakness persisting for a duration of 10 hours. Ten hours prior to admission, she experienced abrupt onset of dysarthria and expressive communication difficulties. She exhibited partial compliance with motor instructions but did not present any hearing or comprehension impairments. Additionally, she displayed weakness in her left limb, inability to elevate the left upper limb, inability to stand or walk using the left lower limb, disturbances in consciousness, difficulty swallowing and coughing while drinking water. Furthermore, she reported symptoms such as headache, tinnitus, dizziness, chest tightness palpitations and a history of neck massage. The patient had no significant medical history and denied any family history of similar conditions. Upon physical examination upon admission: body temperature was measured at T36.3℃; heart rate at P68 beats per minute; respiratory rate at R20 breaths per minute; blood pressure at Bp103/61mmHg; oxygen saturation level (SPO2) recorded as 98%. Cardiopulmonary and abdominal examinations yielded negative findings. Neurological examination revealed clear mental status with dysarthria observed but normal higher cognitive function intact without detection of vascular murmurs over the abdomen or costal ridge angle.Systolic murmur is audible in the right internal carotid artery. There is left central facial paralysis, left limb muscle strength grade 0, and no bilateral pathological signs emerged. The NIHSS score is 11 points (4:2 points + 5a: 4 points + 6a: 4 points + 9:1 point), GCS score is 15 points, swallowing dysfunction rating is 4 points, and mRS Score is 0 points. Auxiliary tests including routine blood test, coagulation function test, liver and kidney function test, electrolyte test, myocardial enzyme test, BNP test, erythrocyte sedimentation rate test, female hormone level checkup, blood lipid level checkup, anti-streptococcal hemolysin \"O\" antibody detection, rheumatoid factor detection, C-reactive protein detection and autoantibody system column detection showed no obvious abnormalities found. Cervical vascular ultrasound revealed diffuse thickening of the longitudinal section of the middle and left common carotid artery wall with concentric ring thickening observed in transverse section causing local lumen stenosis with stenosis rates of approximately 73% (right) and 60% (left). Figure\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003ea depicts acute infarction in the right basal ganglia and paraventricular area on head magnetic resonance imaging. Figure\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eb demonstrates occlusion of the right internal carotid artery and middle cerebral artery on MRA of the head. Emergency digital subtraction angiography (DSA) suggests occlusion of the right common carotid artery, which was not visualized, resulting in mTICI class 0 flow impairment in the right hemisphere. Brain artery imaging reveals beaded changes indicative of fibromuscular dysplasia in the left internal carotid artery. The left anterior traffic is patent, while partial compensation for supply to the right middle cerebral artery territory occurs through pIA meningeal arteries. The left external carotid artery is occluded with a blind end formation and absence of forward blood flow observed. Compensation for supply to the right internal carotid artery territory via occipital arteries is provided by the right vertebral artery, whereas compensation for supply to the left external carotid artery territory occurs through occipital arteries supplied by the left vertebral artery. Partial compensation for supply to the right middle cerebral artery territory via pial arteries is facilitated by the right posterior cerebral artery (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). Further super-selective cerebral angiography was performed, followed by intracranial artery thrombolectomy. Under local anesthesia, the 8F support catheter was advanced to the occluded blind end of the right common carotid artery to restore blood flow. Due to difficulties in advancing the suction catheter under negative pressure and considering orthotopic stenosis, balloon dilation was employed using coaxial technology to reach the semi-released state in the C2 segment of the internal carotid artery. The black-red thrombus (20×5×5mm) was successfully aspirated using ADAPT technology, resulting in revascularization of the M1 segment. Considering severe stenosis at proximal carotid artery, balloon dilation was performed with a thrombus protection device. After a 10-minute observation period confirming maintained forward blood flow (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e), stent implantation was deemed unnecessary.\u003c/p\u003e\u003cp\u003ePhysical examination on the first postoperative day revealed stable vital signs, bilateral pupils of 3.0mm in diameter with intact light reflex, clear speech, absence of facial palsy, left limb muscle strength at level 5-, right limb muscle strength at level 5, normal muscle tension, and no evidence of bilateral pathological signs. The NIHSS score was 0 and GCS score was 15. Subsequent MRA reexamination showed a right embryonic posterior communicating artery. Acute lacunar infarction in the right basal ganglia was observed on head MRI (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003ea-c). Three months after surgery, a small lamellar encephalomalacia lesion adjacent to the right lateral ventricle was detected on head MRI (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003ea-c). The patient exhibited normal speech and limb function with an NIHSS score of 0 and mRS Score of 0. Six months later, head and neck vascular CT revealed soft plaques causing beaded stenosis in both proximal carotid arteries (severe stenosis on the right side and moderate stenosis on the left side) (Fig.\u0026nbsp;\u003cspan refid=\"Fig5\" class=\"InternalRef\"\u003e5\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e \u003c/p\u003e\u003cp\u003e \u003c/p\u003e\u003cp\u003e \u003c/p\u003e\u003cp\u003e \u003c/p\u003e\u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eMyofibrous dysplasia (FMD) is a rare disease characterized by stenosis, occlusion of affected blood vessels, and the development of aneurysms or arterial dissection. FMD can involve small and medium arteries throughout the body, with renal artery involvement observed in approximately 90.7% of patients, carotid artery involvement in 25\u0026ndash;30% of cases, and less frequent involvement of intracranial arteries (8.3%). Additionally, FMD may affect other arteries such as the mesenteric artery, iliac artery, and brachial artery [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. The condition predominantly affects women aged between 20 and 60 years old, with a male to female ratio of about 1:9; however, men tend to have a less common but more aggressive course of FMD [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Due to challenges in obtaining pathology samples for diagnosis purposes, digital subtraction angiography (DSA) remains the gold standard diagnostic tool for FMD. Pathologically classified into mesial type, intimal type, and outer type changes; the mesial type accounts for approximately 75%-80%. DSA reveals \"beaded changes\" where the diameter of these beads exceeds that of normal blood vessels primarily affecting the middle and distal segments. The mesial type represents around 10%-15%, presenting fewer beads on DSA with smaller diameters than normal arteries often accompanied by abundant collateral circulation. Intimal DSA demonstrates smooth localized eccentric or centripetal vessel stenosis or long segment smooth stenosis resembling arteritis [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Based on patient's DSA examination findings it was determined that they had mesomembrane-type FMD.\u003c/p\u003e \u003cp\u003eThe common clinical manifestations of cerebrovascular fibromuscular dysplasia (FMD) include: (1) severe and/or chronic migraine, particularly when accompanied by other suggestive symptoms or signs; (2) pulsatile tinnitus characterized by a \"whoosh\" sound in the ear synchronized with the heartbeat; (3) presence of a neck murmur; (4) stroke-like symptoms such as hemiplegia, aphasia, limb sensory disorders, transient ischemic attack (TIA), or temporary loss of vision; unilateral head and neck pain or focal neurological manifestations like partial Horner syndrome with drooping eyelid and pupil constriction [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. Headache that is not classified as chronic migraine or non-migraine type, non-throbbing tinnitus, mild vertigo-headache combination may indicate possible carotid artery dissection. According to the European FMD Consensus guidelines: (1) screening for FMD should be conducted in women under 50 years old who have hypertension; (2) imaging screening for head-neck vascular abnormalities should be performed in cases of pulsatile tinnitus, cervical artery or intracranial artery dissection, intracranial aneurysm, subarachnoid hemorrhage (SAH), and cerebral ischemic events; (3). Computed tomography angiography (CTA) or magnetic resonance angiography(MRA) are preferred imaging modalities; 4. When head-neck FMD is detected on imaging studies further evaluation for renal artery involvement should be considered. Cerebral artery imaging examination plays a crucial role in diagnosing FMD. The luminal imaging findings associated with FMD include: bead-like lumen changes,(2). segmental stenosis which is rare but can present as either short centripetal stenosis or longer tubular stenosis,(3).\"web-like\" filling defect.(4). Diverticular type.\u003c/p\u003e \u003cp\u003eCurrently known causes of fibromuscular dysplasia (FMD) include genetic factors, environmental factors such as smoking, and estrogen levels. In this particular case, the patient did not smoke and had normal estrogen levels; however, genetics cannot be ruled out as a potential cause. FMD susceptibility genes like PHACTR1, LRP1, ATP2B1, and LIMA1 can be further screened in patients with familial aggregation tendency [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. FMD affects various parts of the cerebrovascular system and its associated symptoms vary accordingly. Patients without obvious symptoms should undergo regular monitoring. For FMD patients with endovascular lesions such as vascular stenosis, occlusion, dissection, aneurysm or malformation, appropriate endovascular treatment can be considered based on individual conditions. In the absence of contraindications, antiplatelet therapy (e.g., daily aspirin dose of 75-100mg) is recommended for preventing blood clots and thromboembolic complications in FMD patients [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e].\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eCurrently, there is a paucity of literature on FMD, as the disease lacks specific clinical manifestations. The early symptoms fail to capture the attention of young patients, particularly those experiencing headaches, dizziness, tinnitus, and other related symptoms. Consequently, these symptoms are prone to misdiagnosis leading to severe cerebrovascular accidents. For young individuals suffering from strokes, it is crucial to obtain an accurate medical history and conduct appropriate imaging evaluations in order to expedite access to specialized care and achieve favorable prognoses.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e \u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e \u003cp\u003e Written informed consent was obtained from the patients for the publication of any potentially identifiable images or data included in this article.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eConsent for publication\u003c/strong\u003e \u003cp\u003eThe patient signed informed consent forms.\u003c/p\u003e \u003c/p\u003e\u003cp\u003e \u003ch2\u003eData and materials availability\u003c/h2\u003e \u003cp\u003e Data is provided within the manuscript.\u003c/p\u003e \u003c/p\u003e\u003cp\u003e \u003ch2\u003eCompeting interests\u003c/h2\u003e \u003cp\u003eThe authors declare no competing interests.\u003c/p\u003e \u003c/p\u003e\u003ch2\u003eFunding\u003c/h2\u003e \u003cp\u003eNo funding.\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eAll the authors contributed to this article.Liu was responsible for writing the article and collecting pictures, and Xu supervised and revised the manuscript.\u003c/p\u003e\u003ch2\u003eAcknowledgements\u003c/h2\u003e \u003cp\u003eWe would like to thank the participants and their families for their contributions to this work. The authors did not receive funding for this research.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eBender MT, et al. Safety Assessment of Endovascular Treatment of Cerebral Aneurysms in Patients with Fibromuscular Dysplasia. Interv Neurol. 2018;7(1\u0026ndash;2):110\u0026ndash;7.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003ePersu A, et al. Current progress in clinical, molecular, and genetic aspects of adult fibromuscular dysplasia. Cardiovasc Res. 2022;118(1):65\u0026ndash;83.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eGornik HL, et al. First International Consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med. 2019;24(2):164\u0026ndash;89.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKhoury MH, Gornik HL. Fibromuscular dysplasia (FMD). Vasc Med. 2017;22(3):248\u0026ndash;52.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDe Groote M, et al. Fibromuscular dysplasia - results of a multicentre study in Flanders. Vasa. 2017;46(3):211\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eOlin JW, et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation. 2012;125(25):3182\u0026ndash;90.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFirst international consensus on the. diagnosis and management of fibromuscular dysplasia: Erratum. J Hypertens. 2021;39(9):1932.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFirst international consensus on the. diagnosis and management of fibromuscular dysplasia: Erratum. J Hypertens. 2019;37(5):1098.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eGeorges A, et al. Genetic investigation of fibromuscular dysplasia identifies risk loci and shared genetics with common cardiovascular diseases. Nat Commun. 2021;12(1):6031.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLun R, et al. Comparison of Ticagrelor vs Clopidogrel in Addition to Aspirin in Patients With Minor Ischemic Stroke and Transient Ischemic Attack: A Network Meta-analysis. JAMA Neurol. 2022;79(2):141\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Fibromuscular dysplasia, Cerebral infarction, Thrombectomy of cerebral artery","lastPublishedDoi":"10.21203/rs.3.rs-4336295/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4336295/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eFibromuscular dysplasia (FMD) associated with cerebrovascular diseases represents a rare etiology of stroke in adolescents. The initial symptoms of FMD lack specificity, and the clinical comprehension of cerebral vascular FMD remains inadequate, leading to potential misdiagnosis or underdiagnosis. Therefore, early diagnosis coupled with comprehensive cerebrovascular imaging holds paramount importance.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase presentation\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe present report delineates a case of acute-onset stroke-like symptoms in a 26-year-old female adult patient. Notably, the patient exhibited no pre-existing risk factors for cerebral hemopathy and was devoid of any inducing factors such as drug abuse, long-term hormone use, or neck massage. Additionally, there was no history of fever or related conditions. Acute cerebral infarction was confirmed through head MRI findings. Subsequent improved cerebral angiography revealed occlusion of the right carotid artery and beaded changes in the left carotid artery, leading to the diagnosis of muscle fiber dysplasia (FMD) followed by right arterial thrombectomy.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eCarotid myofibrous dysplasia (FMD), some of which may present with stroke-like symptoms, typically exhibits atypical manifestations and is prone to misdiagnosis. Therefore, it is crucial to integrate the patient's overall condition, clinical symptoms, and appropriate imaging diagnosis in the early stages to expedite triage. In cases of carotid artery occlusion caused by FMD, emergency thrombectomy can be performed for prompt vascular recanalization.\u003c/p\u003e","manuscriptTitle":"Thrombectomy for cerebral infarction caused by carotid muscle fiber dysplasia: a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-05-08 20:43:18","doi":"10.21203/rs.3.rs-4336295/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"c6b04f14-dfb6-4fdc-89cd-c8880513dac2","owner":[],"postedDate":"May 8th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-09-10T15:08:48+00:00","versionOfRecord":[],"versionCreatedAt":"2024-05-08 20:43:18","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-4336295","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4336295","identity":"rs-4336295","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}