Department of Neurology, Refferal Center for Demyelinating Diseases of the Central Nervous System, University Hospital Center Zagreb, Zagreb, Croatia (IA, VM, IZ, MH), Department of Radiology, University Hospital Center Zagreb, Zagreb, Croatia (GP), and Department of Neurology, School of Medicine, University of Zagreb, Zagreb, Croatia (MH).
Received January 26, 2012; accepted March 27, 2012.
Ivan Adamec University Department of Neurology University Hospital Center Zagreb Kišpatićeva 12 HR-10000 Zagreb Croatia e-mail: firstname.lastname@example.org
Purpose. Chronic Horner syndrome is a rare clinical condition, the etiology of which often remains undiscovered. A patient is presented with an 8-year history of Horner syndrome who was diagnosed with multiple cervical artery dissections.
Case report. A 42-year-old woman presented to our emergency department with a severe occipital headache that woke her up from sleep 3 days earlier. She had a history of headaches and recalled one in particular dating back to 2003. At that time, she sought medical attention at general practitioner's office because of the terrible headache and a noticeable disparity of her pupils. She was told that she had miosis of the right pupil. The examination conducted in 2011 revealed Horner syndrome with right miosis and ptosis. A four-vessel cerebral angiography revealed an occlusion of the right internal carotid artery. The morphology of stenosis and pseudoaneurysm of C1 segment of left internal carotid artery, as well as a pseudoaneurysm of V3/V4 junction of left vertebral artery indicated a probable dissective etiology.
Conclusions. This case illustrates that Horner syndrome with a chronic presentation can be as potentially dangerous as its acute counterpart and should be judiciously investigated.
The long and complicated course of the oculosympathetic pathway predisposes it to a wide variety of pathologic processes, ranging from harmless vascular headaches to life-threatening conditions, such as carotid artery dissection, jugular vein thrombosis, or malignancy.1 In a series of 53 patients reported with Horner syndrome, nine of them had chronic Horner syndrome (lasting >1 year).2 None of these patients had cervical artery dissections (CADs), and in only one patient, the etiology was ultimately discovered (thyroid carcinoma).
We present a patient with an 8-year history of Horner syndrome who was diagnosed with multiple CADs.
A 42-year-old woman presented to our emergency department in May 2011 with a severe occipital headache that had awakened her 3 days earlier. She also complained of stiffness and pain in the back of her neck.
The patient had a history of headaches and recalled one in particular dating back to 2003. At that point, she sought medical attention at general practitioner's office because of the terrible headache and disparity of her pupils. She was told by the general practitioner that she had miosis of the right pupil. At the time, magnetic resonance imaging of the brain was contraindicated because of a metal implant in her left tibia (osteosynthesis in 1990 after fracturing her leg as a passenger in a car accident). As the headache passed spontaneously, she didn't seek any further medical attention. The patient also reported that she was not really bothered with the difference in size of her pupils that persisted.
During our examination, further medical history disclosed that she had been on antihypertensive therapy for a year. Also, she reported smoking 20 cigarettes a day for the past 25 years.
The clinical examination revealed Horner syndrome with right miosis, ptosis, and enophthalmus (2 mm) (Fig. 1). A cocaine test was performed, and 45 min after installation of 4% cocaine drops in both of her eyes, anisocoria increased by 2 mm. Although computerized tomography (CT) of the brain was normal, carotid artery dissection was suspected and CT angiogram was performed immediately. The angiogram revealed occlusion of the right internal carotid artery (ICA) and irregularity and narrowing of the left ICA and the left vertebral artery. This diagnostic picture was consistent with bilateral ICA and left vertebral artery dissection. A four-vessel cerebral angiography preceding the possibility of an interventional procedure revealed an occlusion of right ICA (Fig. 1A). The morphology of stenosis and pseudoaneurysm of C1 segment of left ICA, as well as a pseudoaneurysm of V3/V4 junction of left vertebral artery, indicated probable dissective etiology (Fig. 2B, C). The right vertebral artery was hypoplastic with extremely slow flow that was not contributing significantly to posterior circulation (not shown).
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.
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Horner syndrome; cervical artery dissection; chronic