DOCSLIB.ORG

My Experience with Fibromuscular Dysplasia and Stroke

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
My Experience with Fibromuscular Dysplasia and Stroke 42 PERSONAL ESSAY SEPTEMBER 2007 worsening. The ER was backed up with slowly moved all the way down my face. My Experience with trauma cases, so I approached the triage I was scared to death. nurse to ask how long the wait would The nurse then took me right back. be. I explained that I was a registered My blood pressure was 210/130. I had Fibromuscular nurse and that my headache was getting never had high blood pressure. After worse. I asked her to just look at my being sent for a CT scan that came out pupils. She started explaining that they negative, a young resident presented Dysplasia and Stroke were backed up because of all of the me with discharge papers and a diag- traumas and as I again told her I was a nosis of unequal pupils. Mind you, my Pam Mace, RN nurse (which wasn’t getting me any- blood pressure was still elevated and President, The Fibromuscular Dysplasia Society of America where), my face started to go numb. The my pupils were unequal and I just had numbness started at my left temple and a TIA (small stroke). A Brief Review of Fibromuscular Dysplasia Jeffrey W. Olin, DO Professor of Medicine, Mount Sinai School of Medicine New York, New York What is Fibromuscular Dysplasia? Fibromuscular dysplasia (FMD) is a disease that causes one or more arteries in the body to have abnormal cell development in the artery wall. The disease typically occurs in young women and most commonly presents with hypertension, transient ischemic attack, stroke, or an asymptomatic cervical bruit. In 60% to 75% of cases, the renal artery is affected. The carotid and vertebral arteries are affect- ed in about 25% to 30% of cases. Although renal, carotid and vertebral arteries are most affected, FMD can be present in any arterial bed. FMD is a nonatherosclerotic and noninflammatory disease. There are several different types of FMD, but medial fibroplasia, producing the appearance of a “string of beads,” is the most common type encountered. Many physicians consider fibromuscular dysplasia to be rare, but it is not that uncommon; it is often simply overlooked or misdiagnosed.1 y name is Pam Mace and at the age of 37, I had a tran- Possible Causes of FMD Msient ischemic attack (TIA). The cause of fibromuscular dysplasia remains unknown. A variety of genetic, mechanical and hor- It started with a headache. I woke monal factors have been proposed, but further study is required to better understand the cause of this one morning with a dull headache. condition. Over the course of the day, my head became tender to touch. I thought I Diagnosing FMD might feel better after going for a run. The most important aspect is recognition of the signs and symptoms that may occur with fibromus- As I went to leave, my granddaughter, cular dysplasia. The most common presentations are hypertension or an asymptomatic carotid bruit in Ashley, asked if she could ride her bike a young person, usually a woman. Other not uncommon presentations include neck pain, Horner’s syn- alongside, and although I knew it drome, transient ischemic attack, stroke, headache, pulsitile tinnitus, claudication, mesenteric ischemia would slow me down, I agreed. Thank and renal insufficiency. Patients with FMD are more likely to experience a dissection of an artery. God, I did! Since Ashley was with me, While the gold standard for diagnosis remains catheter-based angiography, duplex ultrasound, CT I had to stop at every intersection, gas angiography and MR angiography have all been used with some success. station and fast food restaurant we The “string of beads” appearance as visualized on the angiogram and MR angiogram (in the accom- came upon to help her with her bike. panying article by Pam Mace, RN) is characteristic of the medial type of FMD. Intimal disease, peri- After returning home and shower- medial disease and other less common types have a different angiographic appearance. ing, I noticed my pupils were unequal. Patients with carotid artery FMD should undergo a MRA of the intracranial circulation because about My headache wasn’t any worse but 7-12% of such patients have an associated intracranial aneurysm. my left pupil was very tiny and my right was huge. Being an emergency Treatment of FMD room nurse, I concluded that these Treatment depends on the artery affected and the associated signs or symptoms. In a young per- were neurological symptoms. If some- son with FMD of the renal arteries and hypertension, the treatment of choice is percutaneous balloon one arrived at the ER looking as I did, angioplasty. Stent implantation is not necessary as the results with angioplasty alone are excellent. I would think something bad was FMD of the carotid arteries likewise responds very well to angioplasty. Angioplasty should be reserved going on, like a stroke or brain tumor. for patients with symptoms (TIA or stroke). Asymptomatic carotid FMD (detected by hearing a bruit) I called a close friend of mine, an should be treated with aspirin 81 mg daily. No intervention is required or recommended. If a dissection ER physician with whom I’ve worked, occurs, the patient should initially be treated with anticoagulation. If that fails, or blood supply is seri- and described what was happening to ously compromised, a stent should be placed. me. She urged me to get to the ER right away. I felt really stupid. I References wouldn’t describe it as the worst 1. Olin JW. Recognizing and Managing Fibromuscular Dysplasia. Cleve Clinic J Med 2007;74:273-282. headache of my life… at least not yet. 2. Slovut DP, Olin JW. Fibromuscular Dysplasia. N Engl J Med 2004;350:1862-1871. After arriving at the emergency room, the pressure in my head was More information is also available at www.fmdsa.org. SEPTEMBER 2007 PERSONAL ESSAY 43 The next day, I ended up in another The rest of my story involves decided they should do an angiogram carotid. Since the second stent, I have ER where I was given a spinal tap and almost being sent home again. Being a to look at the arteries. It showed areas been doing great. The stents really an MRI. The MRI showed that I had nurse, I challenged the physician’s of stenosis and a “classic string of made the difference for me. dissected my left vertebral artery. After knowledge and he re-evaluated my beads” appearance, which is character- (Unfortunately, I learned an FMD being stabilized, I was sent back to the case. Had I been sent home, I would istic for fibromuscular dysplasia patient who was stented the same day first hospital I had been to because have had a massive stroke and died. (FMD). My physician also looked at and who received two stents in her left that is where my primary physician Even after that I was misdiagnosed my renal arteries. My right renal artery carotid artery had an episode last July. was on staff. By this time I had the again. If it weren’t for my nursing was also affected, which explains the Both her carotid arteries collapsed and “worst headache of my life.” training, I most likely would not have sudden onset of hypertension. she had a severe stroke.) I go in for survived this ordeal. From the dissections, I formed yearly checkups on my carotid and I ended up dissecting my left verte- carotid aneurysms and needed to have renal arteries. My carotid arteries look bral and bilateral carotid arteries. stents placed in my carotid arteries. In great and it appears my right renal Months later, I continued to be sympto- 2001, a Multi-Link Tetra stent was artery has more stenosis now than a matic and went to see a vascular doctor placed in my right carotid and a year year ago. For now, I will continue tak- at The Cleveland Clinic. My physician later, I received a JOSTENT in my left ing blood pressure medication and Beading on carotid ultrasound Courtesy of Dr. Jeffrey Olin Medial Fibroplasia Courtesy of Dr. Jeffrey Olin Medial Courtesy of Dr. Jeffrey Olin 44 PERSONAL ESSAY SEPTEMBER 2007 diuretics. At some point, I will need to have a angio- I survived with no permanent disabilities — I am has also gotten involved with the FMDSA organ- plasty on the renal artery. one of the lucky ones! I ization and is working out the details of a patient As a result of my experience, I have changed my registry for fibromuscular dysplasia. focus and am doing everything I can to raise aware- Pam Mace is a registered nurse with 14 years Pam can be contacted at pam.mace@ ness of FMD. FMD is more common in women and of experience. She currently lives in Milton, MA fmdsa.org. More information is available at The is a cause of stroke. It was only last year that the and works in the emergency room at Milton Fibromuscular Dysplasia Society of America National Stroke Association listed FMD as a cause Hospital. Pam has also worked as the Clinical (online at www. fmdsa.org). of stroke. FMD can appear in any arterial bed, but Director of Emergency Services at Lincoln Park is more common in the renal and carotid arteries. I Hospital in Chicago. She has worked in the car- know of several patients with mesenteric and subcla- diac cath lab, critical care transport, ICU, and vian FMD. Most of my physicians were not familiar CCU. She is currently the President of FMDSA Letter to the Editor with FMD. Everywhere I go, I need to educate my and is dedicated to raising awareness of fibro- physicians, but this is changing.
Load more

Recommended publications
  • Arterial Manifestations in Young People
    Arterial Manifestations in Young People Ann Marie Kupinski, PhD RVT RDMS FSVU North Country Vascular Diagnostics, Inc, & Albany Medical College, Albany, NY Objectives Describe arterial pathology encountered in young people Discuss criteria used to diagnose nonatherosclerotic disease entities Present cases which illustrate ultrasound findings of arterial disease in young people Arterial Disease in Young People • Approx. 90% of PAD and extracranial arterial disease is due to atherosclerosis • Nonatherosclerotic diseases can include: • Inflammatory diseases • Non-inflammatory diseases (FMD) • Congenital abnormalities • Acquired diseases • Injuries Testing Options • Ultrasound – Useful with large vessel disease – Giant Cell, Takayasu’s, Radiation arteritis – Injury/Trauma • Physiologic testing (PVR, PPG, pressures) – Useful with small vessel disease Buerger’s Disease (Thromboangiitis obliterans) Vasospastic Disease Fibromuscular dysplasia FMD Noninflammatory Nonatherosclerotic Young individuals (mean onset 48 yrs) Women (3:1) Affects small to medium- sized arteries Intima, media or adventitia FMD Distribution Renal 60-75% Cerebrovascular 25-30% Visceral 9% Extremity Arteries 5% Has also been observed in coronary arteries, pulmonary arteries and the aorta 28% of patients have at least two vascular beds involved Intimal fibroplasia Smooth focal stenosis with a concentric band Long smooth tubular stenosis Poloskey, et al. Circulation 2012;125 Medial fibroplasia Alternating areas of thinned media and thickened fibromuscular
    [Show full text]
  • Renal Artery Fibromuscular Dysplasia Update
    COVER STORY Renal Artery Fibromuscular Dysplasia Update Diagnosis and management of this serious but underdiagnosed disease. BY JOE F. LAU, MD, PHD; ROBERT A. LOOKSTEIN, MD; AND JEFFREY W. OLIN, DO ibromuscular dysplasia (FMD) is a noninflam- prevalence than previously thought. Cragg and associ- matory, nonatherosclerotic arterial disease that ates identified FMD in the renal arteries in 3.8% of 1,862 predominantly affects the renal and carotid arter- potential renal donors who underwent renal angiography.4 ies but has also been identified in almost every Neymark and colleagues found on renal angiography Farterial bed.1,2 FMD may cause arterial stenosis, occlu- that 6.6% (47/716) of potential renal donors had FMD.5 sion, aneurysm, and/or dissection, but many patients are If these studies are accurate and applied to the general likely asymptomatic and may remain undiagnosed. FMD population, and we were to assume an estimated preva- occurs most commonly in women who are between the lence of at least 5% within the United States population ages of 20 and 60, but it can be present at any age and of women over the age of 18, then approximately 5 to 8 occurs in approximately 10% of men.2,3 The prevalence million women may have FMD. of FMD in the general population is unknown; however, Renal artery FMD occurs in about 70% of patients with several lines of evidence suggest that FMD has a higher FMD and is often bilateral. The most common mani- A B Figure 1. A 26-year-old woman with a 6-week history of severe hypertension.
    [Show full text]
  • Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions a Scientific Statement from the American Heart Association
    AHA Scientific Statement Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions A Scientific Statement From the American Heart Association Jeffrey W. Olin, DO, FAHA, Co-Chair; Heather L. Gornik, MD, MHS, FAHA, Co-Chair; J. Michael Bacharach, MD, MPH; Jose Biller, MD, FAHA; Lawrence J. Fine, MD, PhD, FAHA; Bruce H. Gray, DO; William A. Gray, MD; Rishi Gupta, MD; Naomi M. Hamburg, MD, FAHA; Barry T. Katzen, MD, FAHA; Robert A. Lookstein, MD; Alan B. Lumsden, MD; Jane W. Newburger, MD, MPH, FAHA; Tatjana Rundek, MD, PhD; C. John Sperati, MD, MHS; James C. Stanley, MD; on behalf of the American Heart Association Council on Peripheral Vascular Disease, Council on Clinical Cardiology, Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Radiology and Intervention, Council on Epidemiology and Prevention, Council on Functional Genomics and Translational Biology, Council for High Blood Pressure Research, Council on the Kidney in Cardiovascular Disease, and Stroke Council ibromuscular dysplasia (FMD) is nonatherosclerotic, pathway. A delay in diagnosis can lead to impaired quality of Fnoninflammatory vascular disease that may result in arte- life and poor outcomes such as poorly controlled hypertension rial stenosis, occlusion, aneurysm, or dissection.1–3 The cause and its sequelae, TIA, stroke, dissection, or aneurysm rupture. of FMD and its prevalence in the general population are not It should also be noted that FMD may be discovered inciden- known.4 FMD has been reported in virtually every arterial bed tally while imaging is performed for other reasons or when but most commonly affects the renal and extracranial carotid a bruit is heard in the neck or abdomen in an asymptomatic and vertebral arteries (in ≈65% of cases).5 The clinical mani- patient without the classic risk factors for atherosclerosis.
    [Show full text]
  • Uncommon Vascular Disorders
    Unusual Vascular Disorders John R. Bartholomew, MD, FACC, MSVM Professor of Medicine – Cleveland Clinic Lerner College of Medicine Section Head – Vascular Medicine Department of Cardiovascular Medicine - Cleveland Clinic Disclosure Statement: I have no financial disclosures related to this lecture Unusual Vascular Disorders These disorders may mimic other more commonly seen diseases. They are important for formulating a differential diagnosis and managing your cardiovascular patient. Non-Atherosclerotic Arterial Vascular Disorders • Fibromuscular Dysplasia • Popliteal Artery Entrapment Syndrome • Cystic Adventitial Disease • External Iliac Artery Endofibrosis • Thromboangiitis obliterans • Segmental Arterial Mediolysis • Uncommon Arteriopathies When should you think of these disorders? - Younger patients - Patients with no traditional risk factors for atherosclerosis Case Report • A 44 year old female presents to your office complaining of a whooshing noise in her ears. She has a history of hypertension but otherwise is in good health. You suspect she might have……. a). Fibromuscular dysplasia b). Meniere's disease c). An acoustic neuroma d). A glomus tumor e). A stroke Fibromuscular Dysplasia or FMD • Affects small to medium sized vessels • Nonatherosclerotic, noninflammatory vascular disease • Affects young to middle aged women • Results in arterial stenosis, occlusion, aneurysm formation or dissection Renal Cerebrovascular Visceral Extremities Hypertension Asymptomatic or Abdominal pain, Intermittent headache, pulsatile weight loss,
    [Show full text]
  • Prevalence of Intracranial Aneurysm in Women with Fibromuscular Dysplasia a Report from the US Registry for Fibromuscular Dysplasia
    Research JAMA Neurology | Original Investigation Prevalence of Intracranial Aneurysm in Women With Fibromuscular Dysplasia A Report From the US Registry for Fibromuscular Dysplasia Henry D. Lather, BS; Heather L. Gornik, MD, MHS; Jeffrey W. Olin, DO; Xiaokui Gu, MA; Steven T. Heidt, BA; Esther S. H. Kim, MD, MPH; Daniella Kadian-Dodov, MD; Aditya Sharma, MBBS; Bruce Gray, DO; Michael R. Jaff, DO; Yung-Wei Chi, DO; Pamela Mace, RN; Eva Kline-Rogers, MS, RN, NP; James B. Froehlich, MD, MPH IMPORTANCE The prevalence of intracranial aneurysm in patients with fibromuscular dysplasia (FMD) is uncertain. OBJECTIVE To examine the prevalence of intracranial aneurysm in women diagnosed with FMD. DESIGN, SETTING, AND PARTICIPANTS This cross-sectional study included 669 women with intracranial imaging registered in the US Registry for Fibromuscular Dysplasia, an observational disease-based registry of patients with FMD confirmed by vascular imaging and currently enrolling at 14 participating US academic centers. Registry enrollment began in 2008, and data were abstracted in September 2015. Patients younger than 18 years at the time of FMD diagnosis were excluded. Imaging reports of all patients with reported internal carotid, vertebral, or suspected intracranial artery aneurysms were reviewed. Only saccular or broad-based aneurysms 2 mm or larger in greatest dimension were included. Extradural aneurysms in the internal carotid artery were included; fusiform aneurysms, infundibulae, and vascular segments with uncertainty were excluded. MAIN OUTCOMES AND MEASURES Percentage of women with FMD with intracranial imaging who had an intracranial aneurysm. RESULTS Of 1112 female patients in the registry, 669 (60.2%) had undergone intracranial imaging at the time of enrollment (mean [SD] age at enrollment, 55.6 [10.9] years).
    [Show full text]
  • Endovascular Treatment of Ruptured Vertebral Artery Dissecting Aneurysm in Fibromuscular Dysplasia
    Published online: 2019-05-23 THIEME Case Report | Relato de Caso 149 Endovascular Treatment of Ruptured Vertebral Artery Dissecting Aneurysm in Fibromuscular Dysplasia Tratamento endovascular de aneurisma dissecante roto de artéria vertebral na displasia fibromuscular Luana Antunes Maranha Gatto1 DiegodoMonteRodriguesSeabra1 Jennyfer Paulla Galdino Chaves1 Gelson Luis Koppe1 Zeferino Demartini Jr1 1 Department of Neurosurgery and Interventional Neuroradiology, Address for correspondence Luana Antunes Maranha Gatto, MD, Hospital Universitário Cajuru, Curitiba, PA, Brazil Departamento de Neurocirurgia e de Neurorradiologia Intervencionista, Hospital Universitário Cajuru, Rua São José, 300, Curitiba, PA, 80050350, Arq Bras Neurocir 2019;38:149–152. Brazil (e-mail: [email protected]). Abstract Background Fibromuscular dysplasia (FMD) affects predominantly the cervical and Keywords renal arteries and may cause the classical angiographic pattern of string-of-beads. The diagnosis is increasing with the advances of imaging techniques. ► fibromuscular Case Report A 37-year-old man presenting with subarachnoid hemorrhage due to a dysplasia dissecting aneurysm of the vertebral artery was treated by angioplasty with stent, with ► dissecting aneurysm good outcome. All of the cervical and renal arteries were diseased and showed ► endovascular dysplasia and/or ectasias. procedure Conclusions There are no guidelines or protocols to treat patients with FMD. ► carotid stenosis ► angioplasty ► postoperative complications Resumo Introdução A displasia fibromuscular (DFM) afeta predominantemente as artérias Palavras-chave cervicais e renais e pode causar o padrão angiográfico clássico de cordão de contas. O diagnóstico tem aumentado com os avanços das técnicas de imagem. ► displasia Relato de Caso Homem de 37 anos, apresentando hemorragia subaracnoidea por fi bromuscular aneurisma dissecante da artéria vertebral, foi tratado por angioplastia com stent, com ► aneurisma dissecante bom resultado.
    [Show full text]
  • First International Consensus on the Diagnosis and Management Of
    VMJ0010.1177/1358863X18821816Vascular MedicineGornik, Persu et al. 821816research-article2019 Consensus Document Vascular Medicine 1 –26 First International Consensus on the diagnosis © The Author(s) 2019 Article reuse guidelines: and management of fibromuscular dysplasia sagepub.com/journals-permissions https://doi.org/10.1177/1358863X18821816DOI: 10.1177/1358863X18821816 journals.sagepub.com/home/vmj Heather L Gornik (Co-Chair)1*, Alexandre Persu (Co-Chair)2*, David Adlam3,4, Lucas S Aparicio5, Michel Azizi6,7,8, Marion Boulanger9, Rosa Maria Bruno10, Peter de Leeuw11, Natalia Fendrikova-Mahlay1, James Froehlich12, Santhi K Ganesh12, Bruce H Gray13, Cathlin Jamison14, Andrzej Januszewicz15, Xavier Jeunemaitre16,17, Daniella Kadian-Dodov18, Esther SH Kim19, Jason C Kovacic18, Pamela Mace20, Alberto Morganti21, Aditya Sharma22, Andrew M Southerland23, Emmanuel Touzé9, Patricia van der Niepen24, Jiguang Wang25, Ido Weinberg26, Scott Wilson27,28, Jeffrey W Olin18**, and Pierre-Francois Plouin6,7,8**, on behalf of the Working Group ‘Hypertension and the Kidney’ of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM) 1 Division of Cardiovascular Medicine, Department of Cardiovascular 19Division of Cardiovascular Medicine, Vanderbilt University Medical Medicine, University Hospitals Cleveland Medical Center and UH Center, Nashville, TN, USA Harrington Heart and Vascular Institute, Cleveland, OH, USA 20Fibromuscular Dysplasia Society of America (FMDSA), North Olmsted, 2Division of Cardiology, Department of
    [Show full text]
  • Three Phases of the Fibromuscular Dysplasia Experience
    Central JSM Atherosclerosis Bringing Excellence in Open Access Case Report *Corresponding author Sherry M. Bumpus, School of Nursing, Eastern Michigan University, 340 Everett L. Marshall Building, Ypsilanti, MI, Stigma, Fear, and Acceptance: USA, Tel: 734-487-2279; Email: Submitted: 27 September 2016 Three Phases of the Accepted: 15 January 2017 Published: 16 January 2017 Fibromuscular Dysplasia Copyright © 2017 Bumpus et al. Experience OPEN ACCESS Sherry M. Bumpus1,2*, Rachel Krallman2, Steven Heidt2, and Eva Keywords Kline-Rogers2 • Fibromuscular dysplasia • Mental health 1 School of Nursing, Eastern Michigan University, USA • Quality of life 2 Department of Internal Medicine-Cardiology, University of Michigan Health System, • Vascular diseases USA Abstract Fibromuscular Dysplasia (FMD) is an arteriopathy that can affect any vascular territory, though most often affects the renal and carotid arterial beds. Symptoms are consistent with the vascular bed affected and are often attributed to other conditions, leading to delays in diagnosis. We present a case of a middle-aged woman who presented to the emergency department (ED) with altered mental status and confusion, with an ED discharge diagnosis of altered mental state secondary to depression and alcohol abuse. During follow-up testing, she was diagnosed with hypertensive encephalopathy and, subsequently, FMD. The true prevalence of FMD is unknown, although current estimates vary from 3-4%. Like other “uncommon” disorders, appropriate diagnoses are often delayed or missed altogether. This delay to diagnosis, in combination with non-specific symptoms and providers’ unfamiliarity with FMD, leads to frustration for many patients. A recent FMD qualitative publication revealed patients’ concerns about physical symptoms along with greater frequency of anxiety and depression.
    [Show full text]
  • 10 Tips Doctors Should Know About Fibromuscular Dysplasia (FMD)
    O M U S B R C U I L F A R • D A Y C S I P R L E A M S A I A F S O O Y C T I E FMDSA • FIBROMUSCULAR DYSPLASIA SOCIETY OF AMERICA • 10 Tips Doctors Should Know About Fibromuscular Dysplasia (FMD) 1. FMD is nonatherosclerotic and noninflammatory disease which can affect arteries in every vascular bed, but it most commonly involves renal arteries and internal carotid and/or vertebral arteries. FMD can present as stenosis, aneurysm, dissection and/or tortuosity. 2. FMD is a diagnosis made by imaging. Good quality CT angiography, MR angiography, duplex ultrasound, or their combination is an important part of the evaluation. Catheter-based angiography remains the gold standard in diagnosing FMD, but it is usually reserved for selected cases. One time, head to pelvis imaging (with CT or MR Angiography) is recommended to determine which arteries are affected and to check for aneurysms and dissections of arteries. 3. Hypertension and headache are two of the most prevalent manifestations in FMD patients. FMD patients who do not have carotid, vertebral or intracranial involvement can still have headaches. FMD patients can have essential (primary) hypertension not caused by renal FMD (in other words, non-renovascular hypertension). Pulsatile tinnitus, a “swooshing” noise in the ears timed to the heart beat, is a common symptom among patients with carotid and vertebral artery FMD. Another common sign of FMD is a bruit heard with a stethoscope over an artery on physical examination. 4. Most patients with FMD are managed conservatively with medical therapy and surveillance.
    [Show full text]
  • Progressive Cerebral Vasculopathy and Recurrent Strokes Due to Intracranial fibromuscular Dysplasia T ⁎ Nicole B
    Interdisciplinary Neurosurgery 15 (2019) 19–21 Contents lists available at ScienceDirect Interdisciplinary Neurosurgery journal homepage: www.elsevier.com/locate/inat Neuroanatomical Studies Progressive cerebral vasculopathy and recurrent strokes due to intracranial fibromuscular dysplasia T ⁎ Nicole B. Sur, MDa, , Sakir H. Gultekin, MDb, Amer M. Malik, MDa, Sebastian Koch, MDa a University of Miami, Department of Neurology, 1120 NW 14th St, 13th Floor, Miami, FL 33136, United States of America b University of Miami, Department of Pathology, Holtz Children's Hospital, 1611 NW 12th Ave, R#2044, Miami, FL 33136, United States of America ARTICLE INFO Keywords: Fibromuscular dysplasia Ischemic stroke Cerebrovascular disease Rare causes of stroke Cerebral vasculopathy 1. Introduction difficulty and mild right hemiparesis. Lab workup revealed normal complete blood count, chemistry, lipid Fibromuscular dysplasia (FMD) is a non-atheromatous, non-in- panel and glycosylated hemoglobin (A1C). Thyroid stimulating hor- flammatory vasculopathy of unknown etiology, affecting primarily mone (TSH) was low and free T4 level was normal. C reactive protein small and medium sized arteries [1]. FMD is typically seen in women (CRP) was elevated at 7(?units?) and erythrocyte sedimentation rate and the renal, extracranial carotid and vertebral arteries are most (ESR) and antinuclear antibody (ANA) were normal. EKG revealed commonly affected. Very rarely, FMD has been reported in the in- normal sinus rhythm. Brain MRA at our facility showed MCA stenosis tracranial carotid arteries and it is here that we report on a case of along the distal M1 and proximal M2 branches. Subsequent digital recurrent ischemic stroke in a middle-aged woman with autopsy-proven subtraction angiogram (DSA) showed mild bilateral segmental beading intracranial FMD.
    [Show full text]
  • Fibromuscular Dysplasia (FMD) Fact Sheet
    Fibromuscular Dysplasia (FMD) Fact Sheet What is Fibromuscular Dysplasia? Fibromuscular dysplasia (also known as FMD) is a medical condition characterized by abnormal cell growth within the arterial wall. FMD is different from other blood vessel disorders that affect the arteries, such as atherosclerosis (blockage of arteries secondary to cholesterol plaque), vasculitis (inflammation of the arteries), and thrombosis (formation of blood clots). FMD can lead to different abnormalities of arteries, such as narrowing, a beaded appearance, or even serious complications such as aneurysms (bulging of the arteries) or dissections (tears of the arteries). FMD is not known to involve the veins or the lymphatic system. What causes Fibromuscular Dysplasia? The cause of FMD is not yet clearly known. Likely genetic and environmental factors play a role. FMD is much more common in women (Approximately 90% in the US Registry for FMD). What population does it affect? Predominately middle-aged women, but it has presented across the lifespan of men, women and children What are common signs and symptoms? High blood pressure in patients under age 40, or uncontrolled high blood pressure at any age Unrelenting headaches (migraine type), neck pain, dizziness, or asymmetry of the pupils A pulsatile whooshing or swooshing sound in the ears (pulsatile tinnitus) A bruit (abnormal sound when listening with a stethoscope) in the abdomen (abdominal bruit) or neck (cervical bruit); findings suggest there is turbulence of blood flow in the artery; Dissection or aneurysm of an artery Transient ischemic attack (TIA) or stroke Asymptomatic (no symptoms present) FMD can be silent and be detected by testing during a routine physical.
    [Show full text]
  • Variable Clinical Spectrum of Fibromuscular Dysplasia of the Brachial Arteries
    Variable Clinical Spectrum of Fibromuscular Dysplasia of the Brachial Cleveland, Ohio Arteries. Ana Casanegra, MD1, Vikram Kashyap, MD2, Sandra Yesenko, BA, RVT1, Carmela Tan, MD3, Heather L. Gornik, MD, MHS1 1: Vascular Medicine Section, Department of Cardiovascular Medicine, 2: Department of Vascular Surgery, 3: Department of Anatomic Pathology. Cleveland Clinic, Cleveland, Ohio. Abstract Case 1 Case 2 Findings 62 year-old female 63 year-old female Figure 4: Brachial Background: Fibromuscular dysplasia (FMD) is an uncommon artery surgical vascular disorder most frequently manifest in the renal and carotid • Referred to FMD clinic for a second opinion. • Developed acute pain and paleness in her left arm from the elbow to pathology. arteries. Involvement of the upper extremity arteries has been • FMD was diagnosed 15 years before with a carotid ultrasound and the hand. Hematoxylin & Eosin reportedly rarely in the medical literature and is usually unilateral. We (Panel A) and Movat’s subsequent angiogram as workup for pulsatile tinnitus. • Patient was anticoagulated and transferred to our institution. identified two patients in a single center with bilateral brachial FMD. stain (panel B) with elastic fibers in black. Case 1: 62 year-old woman with pulsatile tinnitus due to FMD of • She had known FMD involvement of internal carotid and renal • Cardioembolic sources were ruled out, as well as hypercoagulable arteries bilaterally. Arrowheads mark the bilateral internal carotid arteries. She also had renal artery FMD with states. external elastic well-controlled hypertension on two agents. She was found to have a • HTN controlled with two antihypertensive medications. • Upper extremity angiogram demonstrated bilateral beaded lamina.
    [Show full text]