What does the Report say on "Fibromuscular Dysplasia increases risk for Intracranial Hemorrhage" ?

If you are under the age of 40 years and have the common symptoms like high blood pressure, dizziness, migraine headaches or a kind of swooshing sound in your ears, screening for the FMD should be carried out. You may not have heard of this disease FMD or Fibromuscular Dysplasia. The symptoms this disease has is common in nature. Women generally suffer from this condition and is twice to have complications of Intracranial Aneurysms. 

What is this FMD ? 

Also known as Fibromuscular dysplasia. The condition rises in the arteries. There is abnormal development of the cell in the walls of artery. When there is abnormal development, the flow of blood doesn't occur as normal as usual. Artery walls starts to contain lesion which ultimately narrows the artery. Difficulty arises for the blood to flow freely. Due to this, there is beating, dissection or aneurysms in the arteries making it weak.

FMD occurs in any part of the body. But mainly, it occurs in the kidney arteries or renal arteries. Now, the researchers found the aneurysms in the brain where women are at high risk conditions. 

Brain aneurysms happen around 30,000 people in US every year in which 40% of people die within 24 hours. Aneurysm is simply an enlargement of the artery which is caused by the weakness of the artery walls. Due to the weakness, it causes a bulge or dissections. 

 In the cases of SCAD "Severe Coronary Artery Dissection", FMD has been the leading cause of it. Every 1 in 5 patient is suffering from the aneurysm and 1 in 4 patients as Dissection cases. The symptoms of this FMD arises particularly affecting certain points and parts. When there is high-blood pressure, there is symptoms of headache, dizziness and eve the swishing sound in the ear.

From various theories, hormones has been a part of role where women are suffering from Fibromuscular dysplasia more than the men. Nowadays, children are more affected than the adults but have different properties. When adults are affected, more than one artery in same site or different site is affected but in children only one is affected, not in crowded form. 

Fig: Bead appearance of the artery seen. "String-of-beads" feature seen in the Fibromuscular dysplasia (credit)

Blood vessels are detected and diagnosed through the imaging duplex ultrasound CTA or MRA. Presently, cather based angiography is recommended in diagnosing the FMD cases.  

Reuters health reported that the women have two times increased risk in developing the Intracranial aneurysms who has been diagnosed as Fibromuscular dysplasia. 

Wide range of population was estimated in prevalence of Intracranial aneurysms in FMD patients. In the study,  all the patients were taken who were suffering from the Fibromuscular dysplasia.

From the survey, it was found out that 21.7% of patient occurred with aneurysm, 25.7% occurred with dissection and 41.7% of people had aneurysm or dissection before the diagnosis of FMD. 

Around 11.% women presented with no subarachnoid hemorrhage. 11.9% women who was diagnosed as Renal FMD , 13.7% of women diagnosed as Cervial FMD, 13.2% presenting with both renal and cervical FMD presented with Intracranial hemorrhage. As a whole around one third of women (30.2%) presented with the cases of intracranial hemorrhage. 

For the Intracranial hemorrhage to occur, there were risk factors like smoking, increased age at smoking, tobacco pack year, history of smoking and even the subarchanoid hemorrhage. This study defined that the women are recommended for Intracranial imaging if they are suffering from the Fibromuscular dysplasia.

So, What happens during aneurysm ? 

Our heart pumps blood to various organ. The arteries from the neck pumps the blood to the brain. This again makes smaller arteries and supply to the brain. When these arteries come and join each other, they make a loop.

The artery walls are designed to adapt to the flowing pulsatile blood. There happens to form some weak spots in the arteries of Circle of willis, due to many arteries connecting with each other. Due to the flowing and pulsating nature of the blood, there is further stress to the weak spots. Due to further stress, a bulge occurs which is commonly called as Saccular or berry aneurysm. It becomes large and after all burst and rupture the artery walls. If it is thick enough, it bulge and disturbs the surrounding brain tissues and nerves. Chemicals contained in the spilled blood can narrow the other arteries and damage the brain tissue. Brain injury occurs due to swelling and hematoma formation in the brain tissues. 

Fig: Cerebral aneurysm (credit) 

Conclusively, when there is ruptured cerebral artery or aneurysm formation, it is considered as medical emergency. Just to keep in mind that Fibromuscular dysplasia is not a rare kind of disease. So, any kind of presentation of FMD like arterial dissection, aneurysm, subarachnoid hemorrhage or any vascular loops are important to recognize. Further diagnosis and treatment should be implicated.

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Reference: 

JAMA Neurol 2017 [Report]

Medscape , Reuters Health Information [ Increased Risk of Intracranial Aneurysm in Women With Fibromuscular Dysplasia - Medscape ]

NCBI, PMC ; US National Library of Medicine, National Institute of Health [ Fibromuscular dysplasia: what the radiologist should know: a pictorial review]

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