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Year : 1999  |  Volume : 47  |  Issue : 1  |  Page : 77--8

Fibromuscular dysplasia of internal carotid artery.

S Srivastava, P Sharma, S Bhaumik, M Behari 
 Department of Neurology and Neuroradiology, All India Institute of Medical Sciences, New Delhi, India., India

Correspondence Address:
S Srivastava
Department of Neurology and Neuroradiology, All India Institute of Medical Sciences, New Delhi, India.

How to cite this article:
Srivastava S, Sharma P, Bhaumik S, Behari M. Fibromuscular dysplasia of internal carotid artery. Neurol India 1999;47:77-8

How to cite this URL:
Srivastava S, Sharma P, Bhaumik S, Behari M. Fibromuscular dysplasia of internal carotid artery. Neurol India [serial online] 1999 [cited 2023 Mar 29 ];47:77-8
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A 36 year old, right handed farmer was admitted with sudden onset of right hemiparesis and global aphasia. Patient had moderately severe throbbing bifrontal headache without vomiting or seizures. There were no cranial nerve symptoms except for facial asymmetry. Apart from smoking, there were no known risk factors for stroke. He had no neurologic symptoms prior to the current illness. Physical examination showed normal blood pressure on both arms and normal peripheral pulses. No carotid bruit was heard. Cardiovascular, respiratory and abdominal examination were normal. Neurologically, patient was conscious but had global aphasia. There was supranuclear right facial paresis and right upper and lower limb weakness (Grade III/V) with hyperreflexia and extensor plantar response. His haemogram, biochemical parameters, lipid profile, coagulation parameters and antinuclear factor were normal. ECG, X-ray, chest, echocardiogram and carotid doppler study were also normal. Non-contrast CT scan of head on the day of admission showed multiple old small infarcts in left middle cerebral artery territory with evidence of recent infarct in the same territory [Figure. 1]. Four vessel digital substraction angiography showed fusiform dilatation of both internal carotid arteries (ICA) in their upper cervical and intracranial segments. Right ICA showed typical beaded appearance in its intracranial portion [Figure. 2]. Left middle cerebral artery (MCA) was occluded. Abdominal vessels were normal.

FMD is a heterogenous vasculopathy reported from all over the world including India.[9] The most common site is the renal artery, followed by internal carotid, iliac, subclavian and vertebral artery. The aetiology of FMD is unclear and humoral factors, mechanical trauma, genetic predisposition, ischaemia of arterial wall and immunologic factors have all been implicated.[10]

Four histological types of FMD have been described-intimal fibroplasia, medial hyperplasia, perimedial dysplasia and medial fibroplasia. The last mentioned accounts for 90% of the cases. Histologically, this lesion most often results in multifocal areas of arterial stenosis, secondary to hyperplasia of fibrous and muscular components of the arterial wall alternating with areas of marked arterial thinning and aneurysmal dilatation.[11] Fibromuscular dysplasia is associated with spontaneous dissection, aneurysm formation, carotido cavernous fistulas, thromboembolism and haemodynamic compromise of the distal circulation and therefore some patients with this arterial disorder will have cerebral ischaemic symptoms. In Chiras' report, it represented, the second most common cause of ischaemic strokes accounting for about 10% of cases.[12]

Usually, the clinical diagnosis is made on the basis of angiography. Angiographically, three characteristic patterns are seen.[9] The most common is the string of beads appearence seen in approximately 80% of cases. A second angiographic appearance is that of a relatively smooth, unifocal or multifocal tubular stenosis. A third pattern is termed atypical fibromuscular dysplasia. One wall of the affected segment is involved and demonstrates a diverticulum like smooth out pouching that may progressively enlarge into true saccular aneurysm. Clinical symptoms accountable to single accessible lesion can be treated by surgical treatment.[13] Proper treatment of multiple inaccessible lesions, such as the one found in our case, remains completely an enigma.


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