Intracranial Fibromuscular Dysplasia Research Articles

Intracranial Fibromuscular Dysplasia in a Male Child Presented by Middle Cerebral Artery Dissection and Stroke

Fibromuscular dysplasia (FMD) is a group of nonatherosclerotic, noninflammatory arterial diseases that most commonly involve the renal and carotid arteries it typically occurs in women aged between 30 and 50 years. FMD in children is rare and its most common site of involvement are renal arteries. Stroke in children is generally uncommon and due to FMD is even rarer, which is why it is often unrecognized. We present a case of a boy, with an inconspicuous previous anamnesis and orderly development, who woke up at the age of 9 with weakness of the left side of the body and speech disorders. MR showed an ischemic lesion in the irrigation area of the right middle cerebral artery (arteria cerebri media- ACM). In neurological status mild dysarthria with left central facial nerve lesion was noted as well as mild paresis of the left extremities. Neurosonological testing showed segmental extensions and narrowing of the right ACM with underlying dissection, which speaks in favour of fibromuscular dysplasia. Therapy with acetylsalicylic acid 50 mg was initiated, and with further follow-up of the patient, complete regression of symptoms and normal neurological status were recorded.

COGNITIVE FUNCTION AND DEPRESSION IN PATIENTS WITH FIBROMUSCULAR DYSPLASIA

Although patients with fibromuscular dysplasia (FMD) commonly report symptoms of memory loss, the association of FMD with cognitive function and depression is not known. At a single center, participants with a diagnosis of extracranial or intracranial FMD without a history of stroke were enrolled.

Progressive cerebral vasculopathy and recurrent strokes due to intracranial fibromuscular dysplasia

• Fibromuscular dysplasia (FMD) is a non-atheromatous, non-inflammatory vasculopathy of unknown etiology. • FMD is rare and involves the renal arteries and extracranial vertebral and carotid arteries most commonly. • The treatment of cerebral FMD and prevention of complications remains a difficult task.

FREQUENCY OF CERVICAL AND INTRACRANIAL ARTERIES LESIONS AND ASSOCIATED CLINICAL SYMPTOMS IN PATIENTS WITH CONFIRMED RENAL FIBROMUSCULAR DYSPLASIA - ARCADIA-POL STUDY

Objective: To assess the frequency of cervical and intracranial arteries involvement and associated clinical symptoms in consecutive patients with renal fibromuscular dysplasia (FMD) enrolled into ARCADIA-POL study. Design and method: From 183 patients with FMD enrolled into ARCADIA-POL study since 2015 (Polish-French collaboration) all consecutive 157 patients (132F, 25 M, mean age:45.1 ± 15.1 years) with renal FMD were analyzed. All patients underwent clinical evaluation: ABPM, biochemical evaluation, biobanking, duplex Doppler of cervical and abdominal arteries and whole body angio-CT including cervical and intracranial arteries. Results: Among 157 patients with renal FMD, 38 (24.2%) had coexisting FMD lesions in cervical and/or intracranial arteries and/or intracranial aneurysms: 17 (10.8%) patients in carotid, 7 (4.5%) patients in vertebral and 24 (15.3%) patients had lesions in intracranial arteries. Dissections of carotid arteries were present in 3 (1.9%) patients and vertebral artery dissections in 3 (1.9%) patients. In 10 (6.4%) pts internal carotid artery aneurysm and in 2 (1.3%) patients vertebral artery aneurysm were found. 18 (11.5%) patients had intracranial aneurysms: 15 (9.6%) patients had one aneurysm, 2 (1.3%) pts 2 aneurysms and 1 (0.6%) patient had 3 aneurysms. Patients with and without cervical and/or intracranial FMD lesions didn’t differ in terms of age, gender, clinical and ambulatory blood pressure levels, hypertension prevalence and number of antihypertensive medications. There were no significant differences in the prevalence of symptoms such as headaches, tinnitus, dizziness and cervical bruits between the patients with and without cervical and/or intracranial FMD. There was only a significant difference in the presence of Horner's syndrome between patients with and without cervical and/or intracranial FMD (16.7% vs.1.9%; p = 0.001, respectively). There was no difference in the prevalence of stroke, transient ischemic attack or intracranial bleeding between the groups. Conclusions: There were no specific clinical features suggesting the presence of FMD lesions and vascular complications in cervical and/or intracranial arteries in patients with confirmed renal FMD included into ARCADIA-POL STUDY. Our study showed that systematic evaluation of cervical and intracranial arteries in patients with renal FMD results in revealing relatively high prevalence of FMD lesions and vascular complications in cervical and/or intracranial arteries.

PP.19.10] THE ARCADIA-POL STUDY– INVOLVEMENT OF CERVICAL AND INTRACRANIAL ARTERIES IN RELATION TO VASCULAR COMPLICATIONS AND ASSOCIATED CLINICAL SYMPTOMS IN PATIENTS WITH RENAL FMD

Objective: To assess the involvement of cervical and intracranial arteries and associated clinical symptoms in patients with renal fibromuscular dysplasia (FMD) enrolled into ARCADIA-POL study. Design and method: From 144 patients with confirmed FMD enrolled into ARCADIA-POL study in 2015, 127 patients (104F, 23 M, mean age:44.8 ± 15.9 years) were analyzed. All patients underwent clinical evaluation: ABPM, biochemical evaluation, biobanking, duplex Doppler of cervical and abdominal arteries and whole body angio-CT including cervical and intracranial arteries. Results: Among 127 patients with renal FMD, 31 (24.4%) had coexisting FMD lesions in cervical and/or intracranial arteries and/or intracranial aneurysms: 15 (11.8%) pts in carotid, 4 (3.1%) pts in vertebral and 19 (15.0%) pts in intracranial arteries. Dissections of carotid arteries were found in 4 (3.2%) pts and vertebral artery dissections in 3 (2.4%) pts. In 3 (2.4%) pts internal carotid artery aneurysm was found. 12 (9.5%) pts had intracranial aneurysms: 9 (7.1%) pts had one aneurysm, 2 (1.6%) pts 2 aneurysms, 1 (0.8%) pts 3 aneurysms and 1 (0.8%) pt 4 intracranial aneurysms. Patients with and without cervical and/or intracranial FMD lesions didn’t differ in terms of age, gender, clinical and ambulatory blood pressure levels, hypertension prevalence and number of antihypertensive medications. There was also no difference in the incidence of cervical and/or intracranial FMD lesions in patients with multifocal and unifocal lesions in renal arteries. There were no significant differences in the prevalence of symptoms such as headaches, tinnitus, dizziness and cervical bruits between the patients. There was only a significant difference in the presence of Horner's syndrome between patients with and without cervical and/or intracranial FMD (12.9% vs.2.4%; p = 0.017, respectively). There was no difference in the prevalence of stroke, transient ischemic attack or intracranial bleeding between the groups. Conclusions: There were no specific clinical features suggesting the presence of FMD lesions and vascular complications in cervical and/or intracranial arteries in patients with confirmed renal FMD included into ARCADIA-POL STUDY. Our study showed that systematic evaluation of cervical and intracranial arteries in patients with renal FMD resulted in revealing relatively high prevalence of FMD lesions and vascular complications in cervical and/or intracranial arteries.

Isolated intracranial fibromuscular dysplasia of the posterior cerebral artery

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory arterial disease that most commonly affects the renal and internal carotid arteries, but intracranial FMD is very rare. We report a patient with FMD involving the posterior cerebral arteries (PCAs). A 24year old female was presented with a 4day history of right homonymous hemianopsia with throbbing headache in the left temporo-occipital area. The brain magnetic resonance imaging (MRI) revealed an acute ischemic stroke in the left PCA territory, while the Time of Flight (TOF) magnetic resonance angiogram (MRA) showed segmental luminal irregularities in the left proximal PCA. The conventional angiogram revealed the “string of beads” appearance, a characteristic that is pathognomonic for FMD. The patient’s inhospital clinical course was stable, while there was no recurrence of stroke. This is the second report of FMD of the PCA. Notwithstanding incredibly rare incidences of isolated intracranial FMD and nonspecific findings of MRA, such a pathophysiology should be considered as the cause for a stroke in young patients, especially those with no cardiovascular risk factor.

Abstract 18: Defining Clinical Presentation, Imaging Findings, and Management of Intracranial Fibromuscular Dysplasia: a Single Center Experience

Introduction: Intracranial (IC) artery involvement in fibromuscular dysplasia (FMD) may manifest as stenosis, dissection, or cerebral aneurysm. We sought to identify clinical, imaging, and treatment characteristics of FMD patients with IC manifestations. Methods: Analysis of prospectively collected data from a single center of the US FMD patient registry with additional medical record and imaging review. Patients were consented for enrollment if FMD was confirmed by imaging in at least one vascular territory. Results: From 2/2/2009-7/10/2015, 474 FMD patients were enrolled in the registry. Of the 421 patients with IC arterial imaging, 52 (12.4%) had IC FMD, with catheter angiography available in 29 (55.8%) patients. Median age of initial FMD symptoms was 47 years (IQR 38 - 55) and 51 patients were female. Concomitant extracranial (EC) carotid FMD was present in 92.3%, vertebral FMD in 48.9%, and renal FMD in 50.0%. Among 52 patients with IC FMD, 41 (78.8%) had IC aneurysm (9.7% of entire cohort with IC imaging), 7 (13.5%) had IC dissection, 6 (11.5%) had focal IC stenosis, and 2 (3.8%) had multifocal IC FMD. Fifteen patients with IC FMD (28.8%) were symptomatic (9 TIA/ischemic stroke, 2 hemorrhagic stroke, 4 isolated cranial neuropathy). Among patients with IC FMD, 22 (42.3%) required intervention. Treatment included EC-IC carotid artery bypass in 2 patients, aneurysm coiling in 13, aneurysm clipping in 9, and IC stenosis stenting in 1 patient. At the time of last follow-up, 47 patients (90.4%) were treated with an anti- platelet agent. Conclusion: In a single center cohort, 12.4% patients with FMD had IC involvement. Arterial aneurysm was the most common manifestation followed by dissection and stenosis. Aneurysm was present in 9.7% of patients who underwent IC imaging, highlighting the importance of comprehensive vascular assessment of patients with FMD.

Intracranial fibromuscular dysplasia presenting as various ocular manifestations

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that mainly affects renal, extracranial carotid, and vertebral arteries. Intracranial FMD is uncommon unlike extracranial or renal FMD, and the primary manifestation of intracranial FMD is intracranial aneurysm. We report an unusual case of intracranial FMD showing various ocular manifestations, including central retinal artery occlusion, transient monocular blindness, and oculomotor nerve palsy without renal involvement.

Diagnosis and management of fibromuscular dysplasia: an expert consensus

Diagnosis and management of fibromuscular dysplasia: an expert consensus

Ischemic Stroke Because of Intracranial Fibromuscular Dysplasia

Ischemic stroke in a 12-year-old African-American boy was caused by intracranial fibromuscular dysplasia. Imaging investigations included computed tomography and magnetic resonance angiography, before reaching a definitive diagnosis with a conventional cerebral angiogram. This pediatric case of intracranial fibromuscular dysplasia highlights the need to consider this rare disorder in the differential diagnosis of pediatric stroke and the role of imaging in establishing a diagnosis. The literature of fibromuscular dysplasia is reviewed, and other causes of pediatric stroke are discussed.

A rare cause of ischemic stroke: fibromuscular dysplasia

Childhood ischemic stroke is uncommon and may be associated with many causes and require extensive evaluation. Fibromuscular dysplasia is a rare cause of unknown etiology of childhood stroke which is mostly related with renovascular hypertension and in adults about 85% of cases renal artery has been involved, whereas the intracerebral circulation is the main area affected in children and the documented cause of stroke. We report a 4-year-old girl who presented with facial paralysis and diagnosed as intracranial fibromuscular dysplasia without renal artery involvement.

Fibromuscular Dysplasia of the Distal Internal Carotid and Middle Cerebral Artery

Fibromuscular dysplasiais an uncommon condition of idiopathic, non-inflammatory and non-atherosclerotic disease of the musculature of arterial walls. The disease is rare, but it commonly affects young and middle aged women. Isolated intracranial cerebral fibromuscular dysplasia is extremely rare because cerebral fibromuscular dysplasia usually affects extracranial vessels. A 20-year-old woman was admitted with light hemiplegia and global aphasia. Brain MRI and MRA demonstrated acute left middle cerebral artery territory infarction with a multifocal stenosis and dilatation of the left middle cerebra artery and left internal carotid. The characteristic conventional cerebral angiographic findings demonstrated a typical string-of-beads appearance in the left distal internal carotid artery and proxiaml portion of the left middle cerebral artery, which suggested a medial type fibromuscular dysplasia. We report a case of isolated intracranial fibromuscular dysplasia with left middle cerebral artery territory infarction. Fibromuscular dysplasia should he considered as a stroke risk factors in children and young adults, especially in patients with no known cardiovascular risk factors.

Fibromuscular dysplasia of the basilar artery: An unusual case with medico-legal implications

A 28-year-old male car driver was reported to suddenly loose the control over his vehicle, to hit the right and middle crash barrier, and to be unconscious as the car came to a halt in a position at right angles to the road. The car was hit in its left side by an automobile with high velocity, and the 28-year-old driver died. Examination of the brain revealed a massive isolated basal subarachnoid hemorrhage and a complete tearing of the basilar artery. A macroscopically detectable aneurysm was not found. However, histological examination of the large arteries at the base of the brain showed (i) fibromuscular dysplasia (FMD) mostly involving the basilar artery (ii) with a ruptured micro-aneurysm in its upper third part. The observations of the eye witnesses that the driver initially lost control over his car were judged in favour of the accused to be due to that rupture of the micro-aneurysm, while complete transverse tearing of the basilar artery occurred during the car crash due to hyperextension and rotation of his neck. Intracranial FMD is a rare cause in the differential diagnosis of isolated basal subarachnoid hemorrhage. The medico-legal implications of this entity are described in the presented case.

Isolated intracranial fibromuscular dysplasia presents as stroke in a 19-year-old female

Isolated intracranial fibromuscular dysplasia is rare and may present with cerebrovascular events. It should be considered as etiology of stroke in otherwise healthy young patients. Though diagnosis is often challenging, characteristic morphologies may be revealed on magnetic resonance and catheter angiography. Cephalocervical fibromuscular dysplasia typically involves the extracranial portion of the internal carotid artery (nearly 95%). This rare case demonstrates isolated intracranial fibromuscular dysplasia in a 19-year-old female with left caudate and genu of internal capsule stroke.

Intracranial Atypical Fibromuscular Dysplasia With Ruptured Aneurysm-Case Report-

A 53-year-old woman was admitted with severe subarachnoid hemorrhage due to rupture of an aneurysm associated with atypical intracranial fibromuscular dysplasia (FMD). Angiography demonstrated the aneurysm and very irregular form of the left internal carotid artery (ICA), the right ICA, and right proximal middle cerebral artery (MCA). Other arteries showed signs of atherosclerosis. The aneurysm was treated by embolization, but she subsequently died of shock of unknown cause. Detailed examination of serial angiograms detected enlargement of the aneurysm and progression of the irregular appearance of the ICA. FMD is a non-inflammatory and non-atheromatous arteriopathy that commonly affects the cervical ICA and sometimes the intracranial ICA. The association with saccular aneurysm is widely known and the prevalence of incidental aneurysms is higher than that in the general population. The common "string of beads" finding is easily distinguished from other vascular diseases, but non-specific findings such as "tubular stenosis" and "diverticular-like outpouching" are harder to differentiate. FMD is associated with various complications and appropriate periodic follow-up examination is required. Detailed analysis of serial angiograms may facilitate diagnosis of this condition.

De novo formation of an aneurysm in a case of unusual intracranial fibromuscular dysplasia

Intracranial fibromuscular dysplasia (FMD) is a vascular disease of unknown origin occurring predominantly in young women. The internal carotid artery is most often involved, but other cerebral arteries may also be affected. We report the case of a young woman presenting with an unusual angiographic appearance of intracranial FMD of the internal carotid artery (ICA) that could not be categorized into any type of the Osborn–Anderson classification. During follow up the patient presented with an intracerebral and subarachnoid hemorrhage. Repeated angiography revealed multiple aneurysms in the pathologic segment of the vessel. The patient underwent surgical treatment with clipping of the aneurysms, wrapping of the pathologic segment of the ICA and biopsy of the superficial temporal artery. Histopathological sections revealed FMD of the intimal type. α 1-antitrypsin blood levels were normal. Cases of intracranial FMD previously reported in the literature are reviewed and various aspects of this rare disease are discussed.

Intracranial fibromuscular dysplasia in a 5-year-old child

A 5-year-old girl presenting with acute middle cerebral artery stroke was diagnosed as having intracranial fibromuscular dysplasia by angiographic findings of focal changes in the proximal right middle cerebral artery and pathological dilatation of the right internal carotid artery at the base of the skull, as well as dissection of at least one of the middle cerebral artery branches and nonfilling of two or perhaps three remaining middle cerebral artery segmental branches. Her clinical condition improved after management of the increased intracranial pressure. She did not receive any medication after discharge and had virtually no residual sequelae. Our case documents an unusual location and age of onset of a patient with fibromuscular dysplasia.

Fibromuscular Dysplasia of the Cervical Arteries associated with a Distal Vertebral Trunk Aneurysm

A 64-year-old, hypertensive female suddenly experienced severe headache. On admission, the patient had almost clear consciousness but was slightly restless and complained of severe headache and nausea. Neurological examination revealed only neck stiffness. A computed tomographic scan revealed subarachnoid hemorrhage. Angiographically, bilateral internal carotid and vertebral arteries had the "string of beads sign" at their cervical portion, and the left internal carotid artery also had the same sign at its cavernous portion. The left vertebral artery had low-origin posterior inferior cerebellar artery and a berry-shaped aneurysm at its distal trunk. A diagnosis of cervical and intracranial fibromuscular dysplasia (FMD) with a ruptured berry-shaped aneurysm of the distal vertebral trunk was made. The berry-shaped aneurysm was successfully treated with proximal clipping. Angiographically, right renal and axillary arteries also had the "string of beads sign," and the patient's hypertension seemed to be renovascular in etiology. The co-existence of intracranial FMD and cerebral aneurysm of unusual location suggests a possible relationship between the FMD and the development of cerebral aneurysm.

Angiographic spectrum of cervical and intracranial fibromuscular dysplasia.

Cephalocervical or intracranial fibromuscular dysplasia (FMD) can be identified by its characteristic angiographic appearance. Most of these lesions occur adjacent to the C1-2 interspace, characteristically sparing the origins and proximal segments of the major extracranial vessels. Approximately 65% of our patients had bilateral involvement of the cervical internal carotid arteries. Thirty percent were associated with one or more intracranial aneurysms. The vertebral arteries were involved in 10% of the cases. Twenty-four of 25 cases were associated with symptoms of either subarachnoid hemorrhage or focal cerebral ischemia.