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Name: FMD = Fibromuscular Dysplasia

Description: most commonly affects the renal (kidney) and internal carotid arteries (to brain)

This casebook is published and has been read 973 times.

The author of this casebook has identified the following medical topics as being highly relevant to this casebook.

  • Aneurysm -- Pathological outpouching or sac-like dilatation in the wall of any blood vessel (ARTERIES or VEINS) or the heart (HEART ANEURYSM). It indicates a thin and weakened area in the wall which may later rupture. Aneurysms are classified by location, etiology, or other characteristics.
  • Hypertensive disease -- Persistently high systemic arterial BLOOD PRESSURE. Based on multiple readings (BLOOD PRESSURE DETERMINATION), hypertension is currently defined as when SYSTOLIC PRESSURE is consistently greater than 140 mm Hg or when DIASTOLIC PRESSURE is consistently 90 mm Hg or more.
  • angiogram -- Radiography of blood vessels after injection of a contrast medium.
  • Vascular Diseases -- Pathological processes involving any of the BLOOD VESSELS in the cardiac or peripheral circulation. They include diseases of ARTERIES; VEINS; and rest of the vasculature system in the body.
  • Dysplasia -- (dis-PLAY-zha) Cells that look abnormal under a microscope but are not cancer.
Notes

What is Fibromuscular Dysplasia?

Fibromuscular dysplasia (FMD) is the abnormal development or growth of cells in the walls of arteries that can cause the vessels to narrow or bulge. The carotid arteries, which pass through the neck and supply blood to the brain, are commonly affected. Arteries within the brain and kidneys can also be affected. A characteristic “string of beads” pattern caused by the alternating narrowing and enlarging of the artery can block or reduce blood flow to the brain, causing a stroke or mini-stroke. Some patients experience no symptoms of the disease while others may have high blood pressure, dizziness or vertigo, chronic headache, intracranial aneurysm, ringing in the ears, weakness or numbness in the face, neck pain, or changes in vision. FMD is most often seen in persons age 25 to 50 years and affects women more often than men. More than one family member may be affected by the disease. The cause of FMD is unknown. An angiogram can detect the degree of narrowing or obstruction of the artery and identify changes such as a tear (dissection) or weak area (aneurysm) in the vessel wall. FMD can also be diagnosed using computed tomography, magnetic resonance imaging, or ultrasound.

Is there any treatment?

There is no standard protocol to treat FMD. Any treatment to improve blood flow is based on the arteries affected and the progression and severity of the disease. The carotid arteries should be tested if FMD is found elsewhere in the body since carotid involvement is linked to an increased risk of stroke. Patients with minimal narrowing may take a daily antiplatelet such as an aspirin or an anticoagulant to thin the blood and reduce the chances that a clot might form. Medications such as aspirin can also be taken for headache and neck pain, symptoms that can come from FMD. Patients with arterial disease who smoke should be encouraged to quit as smoking worsens the disease. Further treatment may include angioplasty, in which a small balloon is inserted through a catheter and inflated to open the artery. Small tubes called stents may be inserted to keep arteries open. Surgery may be needed to treat aneurysms that have the potential to rupture and cause bleeding within the brain.

What is the prognosis?

Currently there is no cure for FMD. Medicines and angioplasty can reduce the risk of initial or recurrent stroke. In rare cases, FMD-related aneurysms can burst and bleed into the brain, causing stroke, permanent nerve damage, or death.

What research is being done?


The National Institute of Neurological Disorders and Stroke (NINDS), a component of the National Institutes of Health (NIH) within the U.S. Department of Health and Human Services, is the nation’s primary funding source for research on the brain and nervous system. The NINDS conducts research on stroke and vascular lesions of the nervous system and supports studies through grants to medical institutions across the country.

Bookmarks The following information, which has been distilled by the casebook author from this and other websites is particularly relevant to this casebook.
Bookmarks - Web
Web Page Notes Concepts
 The 'Rare' Disease That Isn't - WSJ.com Wall Street Journal article, names various doctors who have experience with this under-diagnosed disease (Dysplasia)
 
 Fibromuscular dysplasia - Wikipedia, the free encyclopedia Pretty technical (Dysplasia)
 
 The Fibromuscular Dysplasia Association of America FMD Society of America. Information for doctors and for and about patients (Dysplasia)
 
 Fibromuscular Dysplasia Information Page: National Institute of Neurological Disorders and Stroke (NINDS) U.S. National Institute of Neurological Disorders and Stroke (Dysplasia)
 
 Contemporary management of fibromuscular dysplasia. [Curr Opin Cardiol. 2008] - PubMed Result Up-to-date article from a medical journal but useful for patients too. Many links to other articles (Dysplasia)
 
 NEJM -- Fibromuscular Dysplasia From the New England Journal of Medicine (Dysplasia)
 

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