Fibromuscular dysplasia as a rare cause of severe ischemic stroke in a young man

Displasia fibromuscular como causa rara de acidente vascular isquêmico em jovem

LETTERS

Fibromuscular dysplasia as a rare cause of severe ischemic stroke in a young man

Displasia fibromuscular como causa rara de acidente vascular isquêmico em jovem

Giuseppe Gervasi^I; Placido Bramanti^I; Rocco S. Calabrò^II

^IMD. Neurorehabilitation Unit, IRCCS Centro Neurolesi "Bonino-Pulejo", Messina, Italy

^IIPhD. Neurorehabilitation Unit, IRCCS Centro Neurolesi "Bonino-Pulejo", Messina, Italy

^{Correspondence}

Fibromuscular dysplasia (FD) is defined as an idiopathic, segmental, non inflammatory, non atherosclerotic disease of the arterial walls, leading to stenosis of small and medium-sized arteries. FD is an uncommon vascular disorder that occurs in young to middle-aged individuals affecting more often women than men¹.

CASE

A 43-years-old man was admitted to our Neuro-rehabilitation Unit with a severe aphasia and a moderate to severe right hemiparesis mainly involving the upper limb. CT scan showed a deep lesion in the left fronto-temporal region.

Complete hematological examination and cardiac ultrasounds were negative. A carotid Doppler scan showed an occlusion of left internal carotid artery (ICA), immediately after its origin. Cerebral catheter angiography showed an "irregular profile of ICA in the extracranial tract with fibroplastic phenomena and severe stenosis of its intracisternal tract and of the proximal tract of left MCA" (Figure).

Diagnosis of "major stroke due to mean cerebral artery dissection in patient with fibromuscular dysplasia" was supposed and the patient continued oral anticoagulation treatment for six months. At one year-follow-up the patient did not present any ischemic recurrences with a slow but significant improvement of his neurological condition.

DISCUSSION

FD is a rare vascular disease often overlooked or misdiagnosed. Cervical FD is asymptomatic in the 70% of cases; thus diagnosis can be posed with angiography examination or autopsy after death for unspecified stroke or complicated hypertension². The pathogenesis of FD is unclear. It is likely that FD may start as minor lesion of congenital origin, which predisposes to an abnormal fibro-proliferative response to mechanical or circulatory stimuli (i.e. hypertension, smoke and frequent microtrauma)³.

Spontaneous cervical artery dissection is a common cause of stroke in young adults and is associated with FD in 15% of cases. A typical patient with carotid artery dissection presents the classic triad with pain on one side of the head, face or neck accompanied by a partial Horner's syndrome and followed hours or days later by cerebral or retinal ischemia. The presence of any two elements of this triad should strongly suggest the diagnosis^4,5.

In our patient diagnosis was difficult since personal history and symptomatology were not typical. Firstly, the patient was a male, and FD is more common in female; secondly, the clinical presentation of the artery dissection was not so usual since the patient did not refer any kind of neck or head pain and did not present Horner's syndrome signs.

Nevertheless, vasculitides and collagenopathies can lead to a misdiagnosis². Since our patient did not present any clinical or laboratory sign of the main collagen or autoimmunity diseases, FD diagnosis may have been hypothesized.

The poor knowledge of the natural history and the lack of randomized trials that compared the different treatment options do not allow any satisfactory judgment to be made regarding the need for or the efficacy of any treatment, including surgery and oral anticoagulation.

With the present case we want to underline the importance of a specific and accurate diagnostic screening in young patients affected by cryptogenic stroke. Indeed, rare causes of stroke, such as FD, have to be suspected in young people even when clinical and/or laboratory tests are not significant.

Received 10 December 2010.

Received in final form 15 December 2010.

Accepted 22 December 2010

• 1. Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie P, Gimenez-Roqueplo AP, Jeunemaitre X. Fibromuscular dysplasia. Orphanet J Rare Dis 2007;2:28.
• 2. Slovut DP, Olin JW. Fibromuscular dysplasia. N Engl J Med 2004;350: 1862-1871.
• 3. Dayes LA, Gardiner N. The neurological implications of fibromuscular dysplasia. Mt Sinai J Med 2005;72:418-420.
• 4. Stahlfeld KR, Means JR, Didomenico P. Carotid artery fibromuscular dysplasia. Am J Surg 2007;193:71-72.
• 5. Touzé E, Oppenheim C, Trystram D, et al. Fibromuscular dysplasia of cervical and intracranial arteries. Int J Stroke 2010;5:296-305.

Publication Dates

• Publication in this collection
  20 May 2011
• Date of issue
  2011