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

Fibromuscular dysplasia (FMD) is a rare, segmental, nonatheromatous, and noninflammatory arterial disease of unknown etiology. It predominantly involves renal artery (60–75%) followed by extracranial part of the internal carotid artery and vertebral arteries (25–30%). The disease typically affects middle-aged women and involves intermediate-sized arteries throughout the body. There are rare case reports of extracranial FMD compounding a trauma case. A patient was brought to trauma center emergency with a history of fall from height. There were one previous episode of seizure and two episodes of vomiting. His Glasgow Coma Scale on admission was E1V1M4. Noncontrast computed tomography of the head showed fracture on the right zygomatic, temporal, and parietal bone, with underlying thin subdural hemorrhage. The patient underwent left frontotemporoparietal decompressive craniectomy and lax duraplasty with bone flap in bone bank. On the 1st postoperative day, he succumbed to his injuries despite timely surgery and necessary interventions. It was only postmortem when FMD was diagnosed in carotid artery by histopathological examination. On microscopy, intimal changes were seen in the form of expansion of subendothelial loose matrix with mesenchymal cells, thickening and hyalinization of the internal elastic lamina, areas of duplication and disruption of internal elastic lamina. Medial wall changes included thickening of the wall, focal loss of the smooth muscle, and replacement with fibrosis (dysplastic change). This case emphasizes the importance of considering this disease in the differential diagnosis of children and young adults with stroke (which subsequently lead to his fall).

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“…FMD should additionally be considered in this differential. [ 6 ] In our case, all investigations were negative.…”

Section: Discussionmentioning

confidence: 55%

Internal Carotid Artery Fibromuscular Dysplasia in a Child: Incidental Postmortem Finding after Head Injury

et al. 2017

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“…Isolated intracranial FMD presenting as a stroke in a 19-year-old woman was reported by Birnbaum et al . [3], and a cerebral angiogram showed a string of beads abnormality of the proximal middle cerebral artery and anterior cerebral artery, consistent with FMD. A corkscrew appearance of the retinal vessels was reported in 12 of 32 patients (n=32) with neurofibromatosis type 1 [4].…”

Section: Discussionmentioning

confidence: 99%

Corkscrew angiopathy of intracranial vessels in a young stroke patient: a case report

Alurkar¹,

Karanam²,

Oak³

2012

J Med Case Reports

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IntroductionWe present a rare finding of a ‘corkscrew appearance’ of the distal cerebral vessels in a young Asian woman who presented with acute stroke.Case presentationA 32-year-old Asian woman presented with a 3-month history of recurrent right-sided transient ischemic attacks. Her clinical workup and brain imaging results were normal. A digital subtraction angiogram revealed an abnormal corkscrew appearance of all intracranial distal vessels. She was discharged on a single antiplatelet drug. She had no further transient ischemic attacks on clinical follow-up. A digital subtraction angiogram performed 1 year later revealed no changes in the appearance of these vessels.ConclusionTo the best of our knowledge no similar previous reports exist in the literature. The present report describes a unique case of an unusual corkscrew appearance of the distal intracranial vessels. However, the underlying etiology in the present case remains unknown.

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“…Another case describes a 38-year-old male with symptoms of brain stem ischemia due to FMD of the basilar artery [ 10 ]. Another case of a 19-year-old female with FMD of the proximal segments of the left MCA and ACA presented with left-sided headache, polyphagia, hypersomnia, truancy, visual hallucinations, right central facial weakness, and left-sided ptosis [ 11 ]. A 28-year-old male presented with headache, right-sided numbness and weakness, diminished right-sided vision, and aphasia with angiographic evidence of isolated left proximal posterior cerebral artery [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

A Case of Isolated Intracranial Fibromuscular Dysplasia

McDonald

2020

Cureus

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Fibromuscular dysplasia (FMD) is a non-inflammatory, non-atherosclerotic disease resulting in stenosis and arterial wall weakening of typically medium-sized arteries. Intracranial aneurysm is an uncommon presentation of FMD, with a significant proportion presenting in the posterior circulation. Presented is the rare case of an adult female with left-sided stroke with angiographically-confirmed left middle cerebral artery FMD, with pan-scanning unremarkable for other demonstrable evidence of FMD. The patient’s neurological deficits completely resolved. The patient was stable for discharge home on antiplatelet therapy. Appropriate imaging and screening should be performed to ensure FMD is localized versus systemic. Patients with intracranial FMD should be protected from cerebrovascular events with antiplatelet therapy.

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

Cited by 8 publications

References 7 publications

Internal Carotid Artery Fibromuscular Dysplasia in a Child: Incidental Postmortem Finding after Head Injury

Internal Carotid Artery Fibromuscular Dysplasia in a Child: Incidental Postmortem Finding after Head Injury

Corkscrew angiopathy of intracranial vessels in a young stroke patient: a case report

A Case of Isolated Intracranial Fibromuscular Dysplasia

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