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      Complete ophthalmoplegia, complete ptosis and dilated pupil due to internal carotid artery dissection: as the first manifestation of Takayasu arteritis

      case-report

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          Abstract

          Background

          Takayasu arteritis is a rare, chronic large vessel vasculitis involving the aorta and its primary branches. As the disease progresses, the active inflammation of large vessels leads to dilation, narrowing and occlusion of the arteries. Arterial dissection is due to separation of the layers of the arterial wall resulting in a false lumen, where blood seeps into the vessel wall. Neurological sequelae of intracranial arterial dissection results from cerebral ischemia due to thromboembolism and hypo perfusion. Internal carotid artery dissection in Takayasu arteritis is very rare and complete ophthalmoplegia due to internal carotid artery dissection is also rare. This is the first case report of Takayasu arteritis presenting as complete ophthalmoplegia due to internal carotid artery dissection.

          Case presentation

          A 38-year-old Sri Lankan female presented with sudden onset severe headache, fixed dilated pupil, complete ptosis and ophthalmoplegia on the right side. On imaging, dissection and dilatation was evident in the right internal carotid artery from the origin up to the cavernous segment. She also had stenosis and aneurysmal dilatation of right subclavian artery. Takayasu arteritis was diagnosed subsequently. She was started on aspirin and high dose steroids.

          Conclusions

          Internal carotid artery dissection within the cavernous sinus can lead to third, fourth and sixth nerve palsy due to compression, stretching and ischemia from occlusion of the nutritional arteries. This case report illustrates that internal carotid artery dissection should be a differential diagnosis in palsies of the third, fourth, or sixth cranial nerves, especially when associated with headache. In cases of internal carotid artery dissection, vasculitis such as Takayasu arteritis should also be considered.

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          Most cited references15

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          The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis.

          Criteria for the classification of Takayasu arteritis were developed by comparing 63 patients who had this disease with 744 control patients with other forms of vasculitis. Six criteria were selected for the traditional format classification: onset at age less than or equal to 40 years, claudication of an extremity, decreased brachial artery pulse, greater than 10 mm Hg difference in systolic blood pressure between arms, a bruit over the subclavian arteries or the aorta, and arteriographic evidence of narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities. The presence of 3 or more of these 6 criteria demonstrated a sensitivity of 90.5% and a specificity of 97.8%. A classification tree also was constructed with 5 of these 6 criteria, omitting claudication of an extremity. The classification tree demonstrated a sensitivity of 92.1% and a specificity of 97.0%.
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            Segments of the internal carotid artery: a new classification.

            This study proposes an anatomically based nomenclature for the internal carotid artery (ICA) that can be applied by all disciplines. In 1938, Fischer published a seminal paper describing five segments of the ICA that were designated C1 through C5. These segments were based on the angiographic course of the intracranial ICA rather than its arterial branches or anatomic compartments. Subsequent attempts to apply modern nomenclature to these numerical segments failed to recognize Fischer's original intent of describing patterns of arterial displacement by tumors and, therefore, resulted in a nomenclature that was anatomically inaccurate. Fischer's system was further limited, because segments were numbered opposite the direction of blood flow and the extracranial ICA was excluded. The authors propose a new classification, which includes the entire ICA, uses a numerical scale in the direction of blood flow, and describes the segments of the ICA according to a detailed understanding of the anatomy surrounding the ICA and the compartments through which it travels. Twenty cadaveric specimens with intravascular injection of silicone rubber were used for microscopic dissection and 20 dry skulls were inspected. Histological sections in critical areas were examined. The authors' classification has the following seven segments: C1, cervical; C2, petrous; C3, lacerum; C4 cavernous; C5, clinoid; C6, ophthalmic; and C7, communicating. This classification is practical, accounts for new anatomic information and clinical interests, and clarifies all segments of the ICA.
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              Takayasu's arteritis. Clinical study of 107 cases.

              The clinical experience derived from the retrospective study of 107 cases of TA over a 19 year period is presented. The disease predominated in females (8.5:1), with age of onset usually less than 20 years. In half of the cases an acute inflammatory phase was observed, characterized mainly by systemic and cardiovascular symptoms. Subsequently the natural course of TA was toward chronicity with gradual deterioration. The most frequent variety of TA (65 per cent of the patients) was Type III, in which the supra-aortic trunks and the abdominal aorta were involved. The predominant clinical features were reduction of amplitude of peripheral arterial pulses (96 per cent), vascular bruits (94 per cent), and raised blood pressure (72 per cent), mainly resulting from renal arterial involvement (62 per cent). Heart failure (28 per cent) is rarely the result of direct coronary arteritis. TA is most often confused with aortic coarctation, but usually the aortogram distinguishes these. The etiology of TA is discussed. The high incidence of previous and present active tuberculous (48 per cent) in the present series and previous experimental work suggest that tuberculosis may play an important role in the etiology of TA. Treatment for antihypertension and heart failure should be employed when indicated. Treatment with corticosteroids requires further evaluation. Treatment for tuberculosis is not justified in all cases until the exact role of tuberculosis is well established.
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                Author and article information

                Contributors
                tharukaherath11@gmail.com
                Sithu2@yahoo.com
                dhanushkavw@yahoo.com
                Sunethra_aes@yahoo.com
                Journal
                BMC Cardiovasc Disord
                BMC Cardiovasc Disord
                BMC Cardiovascular Disorders
                BioMed Central (London )
                1471-2261
                25 July 2017
                25 July 2017
                2017
                : 17
                : 201
                Affiliations
                ISNI 0000 0004 0556 2133, GRID grid.415398.2, , National Hospital, ; Colombo, Sri Lanka
                Article
                638
                10.1186/s12872-017-0638-7
                5526230
                28743241
                14dbc13b-e877-4031-a3c2-390a4fc0e8e4
                © The Author(s). 2017

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 1 May 2017
                : 20 July 2017
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2017

                Cardiovascular Medicine
                complete ophthalmoplegia,internal carotid artery dissection,takayasu arteritis

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