Journal of the Neurological Sciences 337 (2014) 232–234

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Intracranial fibromuscular dysplasia presenting as various ocular manifestations Jae-Hwan Choi a, Jaeho Jung b,⁎, Kyung-Pil Park a, Seung Kuk Baik c, Kyung Un Choi d, Kwang-Dong Choi e, Hee-Young Choi b, Jong-Hoon Shin b a Department of Neurology, Pusan National University School of Medicine, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Republic of Korea b Department of Ophthalmology, Pusan National University School of Medicine, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Republic of Korea c Department of Radiology, Pusan National University School of Medicine, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Republic of Korea d Department of Pathology, Pusan National University School of Medicine, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Republic of Korea e Department of Neurology, Pusan National University Hospital, Pusan National University School of Medicine and Biomedical Research Institute, Republic of Korea

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Article history: Received 15 August 2013 Received in revised form 1 December 2013 Accepted 4 December 2013 Available online 11 December 2013 Keywords: Brian stem infarct Central retinal artery occlusion Fibromuscular dysplasia Oculomotor never palsy String of bead Transient visual loss

a b s t r a c t 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. © 2013 Elsevier B.V. All rights reserved.

1. Introduction

2. Case report

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease characterized by fibrous thickening of the intima, media, or adventitia of the artery [1]. It commonly affects the renal, extracranial carotid, and vertebral arteries [2]. The clinical presentation may range from an asymptomatic condition to a multisystem lifethreatening disease, depending on the involved arteries, the degree of stenosis, or the type of FMD. Hypertension, stroke or transient ischemic attack are common clinical manifestations, whereas ocular manifestations are rare. We report an unusual case of isolated intracranial FMD presenting with various ocular manifestations, including central retinal artery occlusion, transient monocular blindness, and oculomotor nerve palsy.

A 32-year-old man presented with a sudden onset of dizziness, drooping eyelid, and impaired ocular movements in the right eye. He had lost sight in his left eye because of central retinal artery occlusion (CRAO) 2 years ago (Fig. 1). Neuro-ophthalmologic examination revealed a best corrected visual acuity of 20/20 in the right eye and no light perception in the left. There were ptosis and limitation of adduction and elevation in the right eye, but other ocular movements were intact, including the pupils (Fig. 2A). These findings were compatible with right partial oculomotor nerve palsy. Diffusion-weighted MRI of the brain showed an acute infarction in the right paramedian midbrain area (Fig. 2B). MR angiography disclosed fusiform dilatation in the distal internal carotid arteries (ICAs) of both sides, which were suspected as cerebral vasculitis (Fig. 2C and D). Results from complete blood counts, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), serum chemistry, autoimmune antibody screening, coagulation factors, and echocardiography were normal. With a suspicion of acute infarction due to cerebral vasculitis, he was treated with antiplatelet agent and

⁎ Corresponding author at: Department of Ophthalmology, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Beomeo-ri, Mulgeum-eup, Yangsan 626-770, Republic of Korea. Tel.: + 82 55 360 2591; fax: + 82 55 362 2141. E-mail address: [email protected] (J. Jung). 0022-510X/$ – see front matter © 2013 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.jns.2013.12.009

J.-H. Choi et al. / Journal of the Neurological Sciences 337 (2014) 232–234

Fig. 1. Fundus photograph of 32-year-old man with central retinal artery occlusion 2 years ago. The retinal arterioles were severely narrowed, and there was heavy retinal edema. A small patch of retina which appeared normal was seen near the optic disc due to an efficient cilioretinal arterial circulation, but the macular was not spared.

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blurring in the right eye which had lasted for 10–20 s, and occurred 5–15 times per day. Neuro-ophthalmologic examination showed ptosis and limitation of adduction in the left eye, and DWI disclosed an acute infarction in the left paramedian midbrain area (Fig. 2C). Cerebral angiography demonstrated a ‘string of beads’ appearance of the right petrocavernous internal carotid artery (ICA), which was interpreted as fibromuscular dysplasia (FMD) (Fig. 3A). Both ophthalmic arteries were also irregularly shaped, including a shallow segment and a dilatation over a long segment (Fig. 3B and C). There was no compatible appearance for FMD in the renal or iliac arteries. He underwent right temporal artery biopsy under local anesthesia. Pathologic examination revealed intimal fibrous proliferation combined with irregularly arranged subendothelial mesenchymal cells, loose fibrous connective tissue and luminal narrowing (Fig. 4). However, the internal and external elastic laminae, of the tunica media were intact. These findings were histologically compatible with intimal FMD. After conservative treatments including hydration and anticoagulation, his ocular movements improved gradually. Transient visual blurring of the right eye also disappeared within one month. 3. Discussion

oral prednisolone medication, and his ocular movements improved gradually over the following several weeks. Six months later, he developed a drooping eyelid and impaired ocular movements in the left eye. He also reported transient visual

Fig. 2. (A) Nine-gaze photograph shows limitation of adduction and elevation in the right eye. (B) A diffusion-weighted image (DWI) demonstrates an acute infarction in the right paramedian midbrain area, which may involve the fascicle of the oculomotor nerve. (C) A DWI, performed 6 months later when the partial oculomotor nerve palsy developed again in the left side, discloses an acute infarction in the left paramedian midbrain area. (D and E) MR angiography shows fusiform dilatation in the distal internal carotid arteries (ICAs) of both sides, but no vascular abnormalities in posterior circulation.

Fibromuscular dysplasia (FMD) is an idiopathic, segmental, nonatherosclerotic and noninflammatory vascular disease that may cause stenosis, aneurysm, dissection or obstruction of small and medium-sized arteries [1]. It most commonly affects the renal, extracranial carotid, and vertebral arteries [2,3]. Medial FMD is the most frequent pathologic subtype, and is usually associated with the ‘string of beads’ pattern on angiography. Intimal or adventitia FMD is less common than medial FMD, but the subtypes are not mutually exclusive [4]. The same patient may have various subtypes of FMD in different arterial bed. Our patient showed a typical ‘string of beads’ pattern consistent with medial FMD in the distal ICA, but an intimal type in the temporal artery on pathological study. Although cerebrovascular FMD may be often asymptomatic or associated with non-specific symptoms, including headache, tinnitus, lightheadedness, and syncope, the more specific neurological syndromes of transient ischemic attack and stroke may be the first presentation of FMD [1–3]. Cerebrovascular symptoms are related to critical stenosis or occlusion of major arteries, cerebral embolism originating from stenotic arteries, spontaneous arterial dissection, or rupture of an intracranial aneurysm. Ocular complications are uncommon in FMD, but our patient showed various ocular manifestations, including central retinal artery occlusion (CRAO), transient monocular blindness (TMB) and oculomotor nerve palsy without renal involvement. Transient monocular or binocular blindness has been reported as a common ocular complication of FMD [5–8]. It has been seen in severe stenosis of carotid circulation, which causes transient retinal or choroidal hypoperfusion. On the other hand, sudden visual loss from occlusion of the ophthalmic or retinal artery is a rare manifestation, and there have been only two reports published about CRAO or cilioretinal artery occlusion secondary to FMD [9,10]. To the best of our knowledge, this is the first case showing both TMB and CRAO associated with intracranial FMD. Cerebral angiography revealed a multifocal stenosis of both ophthalmic arteries as well as fusiform dilatation of the right distal ICA at the petrocavernous portion. These findings suggest that TMB and CRAO in our patient may be ascribed to focal ischemia due to either a hemodynamic compromise of the ophthalmic artery or a thromboembolic mechanism from the stenotic ophthalmic artery. Arterial dissection or vasospasm may be another possible explanation. The impairment of ocular motility has been rarely described in FMD. In previous reports, ocular motor nerve palsies usually resulted from complications of FMD, such as compression by a giant aneurysm arising from the ICA or the carotid-cavernous fistula [11,12]. On the other hand, our patient developed both partial oculomotor nerve palsies due to midbrain infarction involving the fascicle of the oculomotor nerve. Although

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Fig. 3. Cerebral angiography shows fusiform dilatation and ‘string of beads’ appearance of the right petrocavernous internal carotid artery (ICA, A). Both ophthalmic arteries exhibit focal dilatation at their origin from the ICA, and diffuse luminar irregularity in the distal portion (B and C).

Fig. 4. (A) The superficial temporal artery shows an intimal fibroproliferation with subintimal dissection (arrows) and luminal narrowing (asterisk). Hematoxylin–eosin staining, 40 ×. (B) Masson's trichrome stain (400 ×) demonstrates a marked fibrous thickening and dense collagen deposition of the tunica intima.

cerebral angiography did not show typical angiographic appearances of FMD at the basilar or vertebral artery, paramedian branches arising from the top of the basilar artery may be involved, like the ophthalmic artery. Intracranial FMD seems to be uncommon unlike extracranial or renal FMD, and the primary manifestation of intracranial FMD is intracranial aneurysm. Since intracranial FMD involving the distal ICA may present as unusual ocular manifestations like in our patient, FMD should be considered as a possible etiology of TMB or CRAO, especially in a young patient without definite causes.

Conflicts of interest All authors report no conflicts of interest.

References [1] Slovut DP, Olin JW. Fibromuscular dysplasia. N Engl J Med 2004;29(350):1862–71. [2] Olin JW, Froehlich J, Gu X, Bacharach JM, Eagle K, Gray BH, et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation 2012;125:3182–90.

[3] Touzé E, Oppenheim C, Trystram D, Nokam G, Pasquini M, Alamowitch S, et al. Fibromuscular dysplasia of cervical and intracranial arteries. Int J Stroke 2010;5:296–305. [4] Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie P, Gimenez-Roqueplo AP, Jeunemaitre X. Fibromuscular dysplasia. Orphanet J Rare Dis 2007;2:28. [5] Collins Jr GJ, Rich NM, Clagett GP, Spebar MJ, Salander JM. Fibromuscular dysplasia of the internal carotid arteries. Clinical experience and follow-up. Ann Surg 1981;194:89–96. [6] Krzanowski M. Fibromuscular dysplasia of the internal carotid artery as a cause of transient cerebral ischemia episodes. Pol Arch Med Wewn 1997;98:546–50. [7] Van Damme H, Sakalihasan N, Limet R. Fibromuscular dysplasia of the internal carotid artery. Personal experience with 13 cases and literature review. Acta Chir Belg 1999;99:163–8. [8] Finsterer J, Strassegger J, Haymerle A, Hagmüller G. Bilateral stenting of symptomatic and asymptomatic internal carotid artery stenosis due to fibromuscular dysplasia. J Neurol Neurosurg Psychiatry 2000;69:683–6. [9] Sawada T, Harino S, Ikeda T. Central retinal artery occlusion in a patient with fibromuscular dysplasia. Retina 2004;24:461–4. [10] Warrasak S, Tapaneya-Olarn W, Euswas A, Sriphojanart S, Sirikulchayanonta V, Leelachaikul P. Fibromuscular dysplasia: a rare cause of cilioretinal artery occlusion in childhood. Ophthalmology 2000;107:737–41. [11] Bhuriya R, Arora R, Khosla S. Fibromuscular dysplasia of the internal carotid circulation: an unusual presentation. Vasc Med 2008;13:41–3. [12] Itoyama Y, Fujioka S, Takaki S, Morioka M, Hide T, Ushio Y. Occlusion of internal carotid artery and formation of anterior communicating artery aneurysm in cervicocephalic fibromuscular dysplasia—follow-up case report. Neurol Med Chir (Tokyo) 1994;34:547–50.

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...
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