We proceeded with interventional anticoagulation by placing a 7 French guiding catheter in left common carotid artery (CCA), at which time a standard systemic heparinization was started. Microguidewire (Agility 14, Codman Neurovascular, Raynham, MA) was passed through the lesion and two self-expanding stents (6 × 40 mm, Precise Pro RX, Cordis, Miami Lakes, FL) were implanted and dilated by a 5 × 30 mm balloon (Aviator, Cordis, Miami Lakes, FL). The angiographic result in the ICA was excellent (Fig. 2B); however, the lumen of left CCA, at the tip of the guiding catheter, was irregularly narrowed with an intimal flap, typical of an acute dissection (Fig. 3C). A bolus of eptifibatide (an antiplatelet drug of the glycoprotein IIb/IIIa inhibitor class) followed by an IV infusion was immediately administered. Three more self-expanding stents of the same make and type were placed in left ICA and CCA continually bridging the lesion, with a good angiographic result (Fig. 3D, E).
An extensive laboratory workup was performed. Immunological tests (antistreptolysin titer, rheumatoid factor, anti-DNA antibody, antinuclear antibody, antineutrophil cytoplasmic antibody, antiphospholipid antibodies, angiotensin-converting enzyme) and thyroid function tests were normal. Serum Borrelia and syphilis serology were negative. Alpha-1-antitrypsin and homocysteine levels were within normal range. Methylenetetrahydrofolate reductase, factor V, factor II, and apolipoprotein E genotyping were normal. The patient was released from the hospital in stable condition placed on acetylsalicylic acid and clopidogrel.
Painful Horner syndrome is considered a red flag for carotid artery dissection and is usually presented to a clinician in the setting of an acute or subacute time span.3 Chronicity of Horner syndrome is usually associated with more benign states such as hemicrania continua.4 Our patient was first diagnosed with miosis of the right eye 8 years before being examined in our emergency department and presented to us as chronic Horner syndrome. Since she had never undergone imaging procedures, we treated her as an acute patient and an urgent CT angiogram followed by a catheter cerebral arteriogram was performed revealing dissections of both of internal carotid arteries as well as left vertebral and left CCA. In her history, we identified two major risk factors for CAD—smoking and hypertension.5 These risk factors are regarded as predisposing factors for endothelial injury at the site of which thrombus formation is more likely.6 Baumgartner et al. have found that Horner syndrome occurs significantly more often in CAD without ischemic events rather than with ischemic events.6 Since Horner syndrome in CAD is explained by mechanical injury of the oculosympathetic pathway caused by subadventitial dissection, it is proposed that this type of dissection carries a lower risk of stroke.7 Our patient did not use oral contraceptives, which is another common risk factor for CAD and likewise had neither diabetes nor dyslipidemia.8 Vessel wall inflammation has been proposed as a pathogenetic factor in CAD. A subset of patients with spontaneous CAD showed signs of a generalized transient inflammatory arteriopathy in contrast-enhanced high resolution magnetic resonance imaging and positron emission tomography-CT in one study.9 This subset of patients may be more prone to multiple dissections. This is supported by the findings that multiple CAD (a) occur preferentially in women, (b) cause clinical symptoms and signs mainly in one vascular territory (Horner syndrome very often), (c) patients usually do not have fibromuscular dysplasia or any other underlying arteriopathy apparent, (d) the majority of cases are preceded by a minor trauma or infections, (e) most patients have favorable clinical outcomes, and (f) most patients have benign long-term prognosis.10
The only major trauma for this patient was a car accident in 1993 when she fractured her leg but there was no reported head trauma. Minor trauma to the neck, such as sudden neck stretch occurring in car accidents, has been described as a possible cause of CAD,3 but because of considerable time gap between the accident and her symptoms (21 years before she presented to us and 13 years before the first sign of Horner syndrome), we did not consider the relationship. The history and lack of supportive laboratory data indicating underlying systemic disease pointed to a diagnosis of spontaneous CAD.
Given the anatomy of the oculosympathetic pathway, Horner syndrome can be a symptom of injury of both the central and peripheral nervous system ranging from the hypothalamus to the chest.11 In its classic acute presentation associated with headache or neck pain, it is a valuable sign of possible ICA dissection and demands urgent diagnostics and treatment. This case illustrates that in its chronic presence, it can be as equally alerting as its acute counterpart and is something to be considered with precaution.
University Department of Neurology
University Hospital Center Zagreb
Authors' contributions: Study concept and design: Adamec, Habek. Acquisition of data: Adamec, Matijević, Pavliša, Habek. Analysis and interpretation of data: Adamec, Matijević, Pavliša, Zadro, Habek. Drafting of the manuscript: Adamec, Habek. Critical revision of the manuscript for important intellectual content: Adamec, Zadro, Habek. Administrative, technical, and material support: Adamec, Matijević, Pavliša, Zadro, Habek. Informed consent was received for the use of the patient's image in Figure 1.
1. Habek M, Petravić D, Ozretić D, Brinar VV. Neurological picture. Horner syndrome due to jugular vein thrombosis (Lemierre syndrome). J Neurol Neurosurg Psychiatry 2008;79:293.
2. Almog Y, Gepstein R, Kesler A. Diagnostic value of imaging in horner syndrome in adults. J Neuroophthalmol 2010;30:7–11.
3. Debette S, Leys D. Cervical-artery dissections: predisposing factors, diagnosis, and outcome. Lancet Neurol 2009;8:668–78.
4. Sjaastad O, Vincent M. Indomethacin responsive headache syndromes: chronic paroxysmal hemicrania and Hemicrania continua. How they were discovered and what we have learned since. Funct Neurol 2010;25:49–55.
5. Demetriades AM, Miller NR, Garibaldi DC. Bilateral internal carotid artery dissection presenting as isolated unilateral Horner syndrome. Ophthal Plast Reconstr Surg 2009;25:485–6.
6. Baumgartner RW, Arnold M, Baumgartner I, Mosso M, Gonner F, Studer A, Schroth G, Schuknecht B, Sturzenegger M. Carotid dissection with and without ischemic events: local symptoms and cerebral artery findings. Neurology 2001;57:827–32.
7. Campos-Herrera CR, Scaff M, Yamamoto FI, Conforto AB. Spontaneous cervical artery dissection: an update on clinical and diagnostic aspects. Arq Neuropsiquiatr 2008;66:922–7.
8. Wu HC, Chen YC, Chen CJ, Chen ST, Lee TH. Spontaneous bilateral internal carotid artery dissection with acute stroke in young patients. Eur Neurol 2006;56:230–4.
9. Pfefferkorn T, Saam T, Rominger A, Habs M, Gerdes LA, Schmidt C, Cyran C, Straube A, Linn J, Nikolaou K, Bartenstein P, Reiser M, Hacker M, Dichgans M. Vessel wall inflammation in spontaneous cervical artery dissection: a prospective, observational positron emission tomography, computed tomography, and magnetic resonance imaging study. Stroke 2011;42:1563–8.
10. Arnold M, De Marchis GM, Stapf C, Baumgartner RW, Nedeltchev K, Buffon F, Galimanis A, Sarikaya H, Mattle HP, Bousser MG. Triple and quadruple spontaneous cervical artery dissection: presenting characteristics and long-term outcome. J Neurol Neurosurg Psychiatry 2009;80:171–4.
11. Mughal M, Longmuir R. Current pharmacologic testing for Horner syndrome. Curr Neurol Neurosci Rep 2009;9:384–9.
Keywords:© 2012 American Academy of Optometry
Horner syndrome; cervical artery dissection; chronic