57-Year-Old Man With Vertigo

Abstract

A 57-year-old man presented to the clinic with sudden onset of severe vertigo, bitemporal headache, nausea, and vomiting of 1 hour's duration. Before his symptoms appeared, he was bending over while working at a construction site. Approximately 3 weeks previously, he had a mechanical fall on ice, landing on his back and sustaining a whiplash injury. He reported no direct trauma to the head or loss of consciousness following the incident. Subsequently, he experienced intermittent bitemporal headaches that would recur every few days. This symptom was associated with left-sided neck pain. He reported no fever, blurred vision, or photophobia. He had been taking ibuprofen, 750 mg twice daily, for the preceding 2 weeks with minimal improvement in his symptoms. His medical history was notable for partial sensory seizures secondary to a benign intracranial dysembryoplastic neuroepithelial tumor. The seizures had been well controlled with levetiracetam for several years. He had not experienced any adverse effects at his current dose. He took no other medications. He had no history of headache syndromes, hypertension, hyperlipidemia, diabetes mellitus, or smoking. On presentation, he was hemodynamically stable. His blood pressure was 106/62 mm Hg, heart rate was 49 beats/min, oxygen saturation was 99% while the patient breathed room air, and respiratory rate was 19 breaths/min. The patient was alert and oriented to time, place, and person. He had no external injuries on examination. His pupils were equal in size and reactive to light and accommodation. Extraocular movements were intact. He did have right-sided horizontal nystagmus, without a change in direction of the fast phase. Cranial nerves II through XII were intact. He had no focal sensory or motor deficits. No abnormalities were detected on evaluation of cerebellar function and deep tendon reflexes. The rest of his physical examination findings were unremarkable. Laboratory tests (reference ranges provided parenthetically) included a complete blood cell count and basic metabolic panel: serum creatinine, sodium, potassium, and bicarbonate levels were within normal limits. A lipid profile revealed a total cholesterol level of 222 mg/dL (desirable, <200 mg/dL), high-density lipoprotein cholesterol concentration of 48 mg/dL (≥40 mg/dL), low-density lipoprotein cholesterol level of 127 mg/dL (desirable, <100 mg/dL), triglyceride value of 234 mg/dL (<150 mg/dL), and hemoglobin A1c level of 5.7% (4.0%-5.6%).1.Based on the patient's history, which one of the following additional physical examination procedures should be performed next?a.Dix-Hallpike maneuverb.Test for Kernig signc.Head impulse testd.Test for Lhermitte signe.Caloric reflex test The Dix-Hallpike test is a positional maneuver designed to reproduce vertigo and elicit nystagmus in patients with intermittent symptoms that are provoked by positional changes. It is a test for canaliths of the posterior semicircular canal and is intended for patients who are asymptomatic at rest. For a patient in the midst of an acute vertiginous episode, any positional change could cause worsening of symptoms and may be misinterpreted as a positive Dix-Hallpike maneuver result. In addition, one must exercise caution while performing the Dix-Hallpike maneuver in patients with suspected dissection. The Kernig sign is an indication of nuchal rigidity that is valuable in patients with suspected meningeal inflammation. It is a test of resistance to full extension of the knee when the patient is in a supine position with 90-degree hip flexion. The temporal profile of this patient's symptoms and the absence of signs like fever and photophobia make meningitis less likely. The head impulse test is one of the most important bedside tests to assess the vestibulo-ocular reflex and should be performed next in this patient. It is performed by grasping the patient's head and instructing the patient to focus on a distant target, about 10 degrees from a neutral position. The examiner then applies brief, high-acceleration, 10- to 15-degree head turns to both sides. In a normal response, the eyes remain on target. If “catch-up saccades” or rapid corrective eye movements occur in one direction, it indicates a peripheral vestibular lesion on that side.1Baloh R.W. Vestibular neuritis.N Engl J Med. 2003; 348: 1027-1032Crossref PubMed Scopus (260) Google Scholar A normal head impulse test result in a patient with acute vestibular syndrome is highly suspicious for a central vestibular lesion. The Lhermitte sign is a transient, sharp “electric shock”–like sensation that radiates down the spine and into the limbs, elicited by flexion of the neck. It is associated with multiple sclerosis and other lesions of the spinal cord like tumors or myelopathy. These diagnoses are less likely given our patient's history. Caloric reflex testing is a test for vestibular disorders but is often difficult to perform in an office setting. After ensuring that the tympanic membrane is intact, warm or cold water is infused into the ear, and the patient is monitored for a normal response of nystagmus, with fast component away from the cold water–infused ear and toward the warm water–infused ear. A unilateral lack of response is suggestive of a peripheral lesion on that side. It is important to note that this test can cause considerable distress and nausea in patients who are awake, especially while in the midst a symptomatic episode. Our patient underwent the head impulse test at the bedside, and no catch-up saccades were noted, ie, the test result was normal.2.In view of this patient's presentation and evaluation, which one of the following is the most likely etiology at this point?a.Benign paroxysmal positional vertigo (BPPV)b.Acute vestibular syndrome (AVS)c.Vestibular migrained.Medication overuse headachee.Medication adverse effect Benign paroxysmal positional vertigo is typically characterized by recurrent episodes of vertigo lasting a few minutes or less. It is brought on by head movements like lying down, sitting up, or turning over. The duration and severity of this patient's symptoms make this diagnosis unlikely. The patient's current presentation can be classified as an AVS, characterized by rapid onset of persistent vertigo, nausea/vomiting, head motion intolerance, nystagmus, and instability. The absence of focal neurologic deficits is not enough to rule out a central etiology. The HINTS examination (head impulse test, nystagmus, and test of skew) is a 3-step bedside oculomotor examination that helps identify a central cause and is reported to be more sensitive than early magnetic resonance imaging (MRI) in AVSs.2Kattah J.C. Talkad A.V. Wang D.Z. Hsieh Y.H. Newman-Toker D.E. HINTS to diagnose stroke in the acute vestibular syndrome: three-step bedside oculomotor examination more sensitive than early MRI diffusion-weighted imaging.Stroke. 2009; 40: 3504-3510Crossref PubMed Scopus (704) Google Scholar As described previously, a normal head impulse test result, suggestive of an intact vestibulo-ocular reflex, is highly suggestive of a central cause. Although nystagmus can be present in both central and peripheral causes of AVS, the presence of torsional, vertical, or bidirectional horizontal nystagmus (alternation of fast phase) is associated with central causes. When testing for skew deviation, an alternate cover test is performed with the patient looking directly ahead at a fixed point. If catch-up saccades are noted in the uncovered eye, it is suggestive of vertical misalignment and associated with a central cause. Vestibular migraine or migrainous vertigo can present with episodic symptoms similar to BPPV but often differs with respect to symptom duration. Patients usually have a history of typical migraine headaches with onset of symptoms at a young age. Similar to classic migraines, these episodes can be associated with photophobia, phonophobia, or visual aura. None of these findings were present in our patient. Medication overuse headache is unlikely, given the lack of a temporal association between the use of ibuprofen and onset of symptoms. In addition, this diagnosis is more likely with medications like opioids and combination analgesics (aspirin/acetaminophen/caffeine) used regularly for several months. Although headaches are a common adverse effect of levetiracetam, the patient has been on a stable dose for several years without experiencing any adverse effects. Before attributing his symptoms to a medication adverse effect, potentially lethal etiologies like intracranial vascular abnormalities must be ruled out. Once a central etiology is suspected, the next step is imaging. Our patient underwent noncontrast computed tomography (CT), which did not reveal any intracranial hemorrhage, mass effect, or acute infarct. The previously diagnosed benign intracranial neoplasm was unchanged. The patient was admitted to the hospital for management of nausea and vertigo with supportive measures. Over the following 24 hours, the patient's nausea and headache improved with oral ondansetron, intravenous droperidol, and fluids. However, his vertigo persisted and was exacerbated by ambulation.3.Which one of the following is the most appropriate next step in this patient's evaluation?a.Repeated noncontrast head CTb.Duplex ultrasonography of neck vesselsc.Magnetic resonance angiography (MRA)d.Digital subtraction arteriographye.Magnetic resonance venography On initial presentation, the patient's head CT revealed no abnormalities. Although repeated noncontrast head CT may show evidence of an evolving infarct that could have been missed by an earlier CT, repeated CT has low yield in patients with vertigo. Based on the patient's presentation, the main concern would be a cerebellar or medullary pathologic process, and MRI has an overall greater sensitivity for imaging the posterior fossa when compared to CT due to less bony artifact. Therefore, repeated CT is not recommended. Duplex ultrasonography of the neck can evaluate the carotid, subclavian, and extracranial section of the vertebral vessels, but it does not identify intracranial vascular pathology. The test of choice at this time would be MRA. Magnetic resonance imaging has a much higher sensitivity for detection of abnormalities within the posterior fossa such as medullary/cerebellar stroke or acoustic neuroma. Parenchymal changes may be visible within a few hours of onset of ischemia.3Chalela J.A. Kidwell C.S. Nentwich L.M. et al.Magnetic resonance imaging and computed tomography in emergency assessment of patients with suspected acute stroke: a prospective comparison.Lancet. 2007; 369: 293-298Abstract Full Text Full Text PDF PubMed Scopus (870) Google Scholar For detection of flow abnormalities within the vertebral or basilar arteries, MRI alone is not always reliable, and additional vascular imaging with MRA would be preferred. Both MRA and CT angiography (CTA) are noninvasive and have high sensitivity and specificity for diagnosing vascular abnormalities of the posterior circulation.4Becker K.J. Purcell L.L. Hacke W. Hanley D.F. Vertebrobasilar thrombosis: diagnosis, management, and the use of intra-arterial thrombolytics.Crit Care Med. 1996; 24: 1729-1742Crossref Scopus (32) Google Scholar When compared to the conventional criterion standard of digital subtraction angiography, CTA and MRA have largely replaced angiography given the high diagnostic rates and the noninvasive nature of these studies. However, digital subtraction angiography might be necessary in cases in which noninvasive imaging is equivocal but clinical suspicion remains high. Magnetic resonance venography can help assess for venous sinus thrombosis, the predominant symptom of which would be worsening headache. Given that the patient's headache resolved but he had persistent vertiginous symptoms, venous sinus thrombosis would be less likely. On hospital day 3, MRA of the head and neck was obtained, which yielded findings consistent with a right vertebral artery dissection (VAD) with segmental areas of marked stenosis and occlusion throughout the course of the artery. There was loss of flow-related MRA signal in the proximal right posterior inferior cerebellar artery. An area of acute infarction involving the inferior medial right cerebellar hemisphere was also identified.4.Which one of the following would be the best next step in the management of this patient?a.Thrombolytic therapy with tissue plasminogen activatorb.Mechanical thrombectomy with intra-arterial thrombolysisc.Antithrombotic therapyd.Vertebral angioplasty with stent placemente.Continue symptomatic management and initiate statin therapy for secondary prevention This patient would not be a candidate for thrombolytic therapy because he is outside the therapeutic window of 3 to 4.5 hours from symptom onset within which thrombolytics are recommended for an ischemic stroke. The use of intravenous tissue plasminogen activator is contraindicated in patients with aortic dissections. Although controversial, it is still considered in those with carotid and vertebral dissections. There remains some concern for exacerbation of the dissection or causing a subarachnoid hemorrhage if the dissection extends intracranially. In selected patients, mechanical thrombectomy is effective in the anterior circulation with CTA evidence of clot in the distal internal carotid artery or proximal middle cerebral artery within 6 hours. With use of CT perfusion, some patients may be eligible for thrombectomy up to 24 hours after symptom onset. Studies to date have largely included the anterior circulation. Initiation of antithrombotic therapy is the best management option in this patient. Both anticoagulation and antiplatelet therapy would be reasonable options. The Cervical Artery Dissection in Stroke Study randomized 250 patients with symptomatic carotid dissections and VADs to antiplatelet vs anticoagulant therapy.5CADISS Trial InvestigatorsAntiplatelet treatment compared with anticoagulation treatment for cervical artery dissection (CADISS): a randomised trial.Lancet Neurol. 2015; 14 ([published correction appears in Lancet Neurol. 2015;14(6):566]): 361-367Abstract Full Text Full Text PDF PubMed Scopus (310) Google Scholar The study found an overall low rate of stroke recurrence at 2.1%. At 3 months, 4 of 250 patients had ipsilateral stroke recurrence without a significant difference between the 2 arms. There were no bleeding events in the antiplatelet group, but 1 major (subarachnoid hemorrhage) and 2 minor (hematuria and hemoptysis) events in the anticoagulation arm. When antiplatelet therapy is considered, monotherapy with aspirin or clopidogrel is reasonable.6Johnston S.C. Easton J.D. Farrant M. et al.Clinical Research Collaboration, Neurological Emergencies Treatment Trials Network, and the POINT InvestigatorsClopidogrel and aspirin in acute ischemic stroke and high-risk TIA.N Engl J Med. 2018; 379: 215-225Crossref PubMed Scopus (597) Google Scholar A short course of dual antiplatelet therapy may also be considered, based on individual patient factors. Endovascular options like angioplasty, stent placement, and other surgical interventions are reserved for patients with recurrent ischemia despite appropriate antithrombotic therapy. This would not be recommended for our patient. Continuation of symptomatic measures alone is not a valid option because initiation of antithrombotic therapy is vital to preventing recurrence of stroke. The Stroke Prevention by Aggressive Reduction in Cholesterol Levels study7Amarenco P. Bogousslavsky J. Callahan III, A. et al.Stroke Prevention by Aggressive Reduction in Cholesterol Levels (SPARCL) InvestigatorsHigh-dose atorvastatin after stroke or transient ischemic attack.N Engl J Med. 2006; 355 ([published correction appears in N Engl J Med. 2018;378(25):2450]): 549-559Crossref PubMed Scopus (2319) Google Scholar found that 80 mg of atorvastatin per day reduced the overall incidence of strokes and cardiovascular events in patients with recent stroke or transient ischemic attack (5-year absolute risk reduction of 2.2% and 3.5%, respectively). However, this study did not include patients with vascular dissections. It is unclear if our patient would benefit from statin therapy in the setting of nonatherosclerotic, posttraumatic VAD. Based on his history and lipid values, the patient has an estimated atherosclerotic cardiovascular disease risk of 5.6%. His low-density lipoprotein cholesterol level is under 190 mg/dL. He does not have a history of hypertension or diabetes. Thus, he does not meet criteria for primary prevention with statin therapy either. A regimen of aspirin, 81 mg/d, was initiated. The patient's symptoms gradually improved with continued supportive measures.5.Which one of the following is the most common condition associated with VAD?a.Fibromuscular dysplasia (FMD)b.Amyloid angiopathyc.Polyarteritis nodosad.Homocystinuriae.Infective endocarditis Although most patients who present with cervical dissections do not have any predisposing vascular pathology, some associations have been noted in the literature. These associations include Ehlers-Danlos syndrome type IV, Marfan syndrome, FMD, cystic medial necrosis, and segmental mediolytic arteriopathy. Of these conditions, FMD has been most commonly associated with cervicocephalic vascular dissections. It is a noninflammatory arteriopathy with a predilection for females. Of the 921 patients enrolled in the US Registry for FMD,8Kadian-Dodov D. Gornik H.L. Gu X. et al.Dissection and aneurysm in patients with fibromuscular dysplasia: findings from the U.S. Registry for FMD.J Am Coll Cardiol. 2016; 68: 176-185Crossref PubMed Scopus (122) Google Scholar vascular dissections occurred in 25.7%; 41.7% had an aneurysm and/or dissection by the time of diagnosis. Dissections were most commonly identified in the extracranial carotid, vertebral, renal, and coronary arteries. Given the high morbidity associated with vascular events in this population, it is recommended that every patient with FMD have a one-time CTA or MRA from head to pelvis. Conversely, 15% to 20% of patients with spontaneous carotid or vertebral dissections have angiographic changes of FMD,9Schievink W.I. Spontaneous dissection of the carotid and vertebral arteries.N Engl J Med. 2001; 344: 898-906Crossref PubMed Scopus (1282) Google Scholar and therefore these patients should be screened for FMD. Amyloid angiopathy has been associated with intracranial hemorrhages but not with spontaneous cervical artery dissections. Polyarteritis nodosa is a systemic necrotizing vasculitis that commonly affects small to medium muscular arteries. There are isolated case reports of polyarteritis nodosa–associated vascular dissections involving the aorta or coronary arteries. Homocystinuria is a disorder of a methionine metabolism and is associated with mental retardation, ectopia lentis, and vascular events that are largely thromboembolic. Rarely, severe infective endocarditis can be associated with vascular dissections, generally involving the aorta. For our patient, CTA of the abdomen and pelvis revealed no evidence of FMD involving the visceral vessels. The etiology of his dissection remained unclear. Given the temporal association between whiplash injury and the onset of symptoms, trauma was considered the most likely cause. The patient remained hemodynamically stable and had complete resolution of symptoms by day 4. He was discharged from the hospital and had close outpatient neurologic follow-up for surveillance imaging. Repeated MRI obtained after 3 months and 6 months revealed expected temporal evolution of the right cerebellar hemisphere infarct with stable changes of right VAD. Aspirin, 81 mg/d was continued indefinitely. Vertigo is a type of dizziness that can be distinguished from other types (like presyncope, disequilibrium, or other nonspecific dizziness) by the description of a spinning sensation. It is an illusion of motion, either of self or the environment, resulting from an asymmetry of the vestibular system. The duration of symptoms can help distinguish vertigo from other causes of dizziness. Vertigo does not last continuously for more than a few weeks because the central nervous system adapts to the asymmetry. Vertigo is often accompanied by nausea, vomiting, and gait instability. The presence of nystagmus is also often associated with vertigo. Once it is established that the presenting symptom is in fact vertigo, assessing the underlying etiology can be challenging because the differential diagnosis ranges from benign etiologies like vestibular neuritis to life-threatening etiologies such as brain stem or cerebellar infarction. The pathology could be at the level of the vestibular apparatus in the inner ear, vestibular nerve, vestibular nucleus in the medulla, or at the level of the cerebellum. In the clinical setting, they are often classified as peripheral and central causes of vertigo. Common peripheral causes include BPPV, vestibular neuritis, Meniere disease, and otitis media. These disorders can often be differentiated on the basis of their duration, progression, and associated clinical features. Benign paroxysmal positional vertigo is associated with brief recurrent episodes lasting only few seconds to minutes and associated with postural changes. The Dix-Hallpike maneuver is diagnostic, and the Epley maneuver can be therapeutic. Meniere disease is also associated with recurrent symptoms but usually lasts longer than BPPV. It is often associated with otalgia, unilateral hearing loss, and tinnitus. Vestibular neuritis is usually associated with viral syndromes and has an acute onset. It may last several days and produces abnormal head impulse test results because the vestibulo-ocular reflex is impaired. Central causes include vestibular migraines, demyelinating disorders, or ischemia/hemorrhage involving the cerebellum and/or brain stem. A comprehensive neurologic examination can sometimes highlight focal deficits that are suggestive of a central etiology. However, as described in this case, life-threatening central etiologies can sometimes have subtle presentations and require a high index of suspicion to make the diagnosis. In addition to classic cerebellar or brain stem symptoms, a careful oculomotor examination provides additional clues to uncovering a central etiology. An abnormal head impulse test result, the presence of vertical, torsional, or direction-changing horizontal nystagmus, and abnormal results on skew testing are suggestive of a central cause. Spontaneous cervicocephalic dissections are rare. Of these, internal carotid dissections are detected twice as frequently as VADs. One population-based study determined the incidence of VAD at 0.97 per 100,000 population.10Lee V.H. Brown Jr., R.D. Mandrekar J.N. Mokri B. Incidence and outcome of cervical artery dissection: a population-based study.Neurology. 2006; 67: 1809-1812Crossref PubMed Scopus (391) Google Scholar In the absence of conditions that compromise vascular structural integrity, the most commonly identified etiology is a preceding mechanical trigger event, often mild in intensity.11Engelter S.T. Grond-Ginsbach C. Metso T.M. et al.Cervical Artery Dissection and Ischemic Stroke Patients Study GroupCervical artery dissection: trauma and other potential mechanical trigger events.Neurology. 2013; 80: 1950-1957Crossref PubMed Scopus (127) Google Scholar Screening for blunt cerebrovascular injury with CTA is recommended for patients with cervical vertebral injury at all levels, even when asymptomatic.12Kopelman T.R. Leeds S. Berardoni N.E. et al.Incidence of blunt cerebrovascular injury in low-risk cervical spine fractures.Am J Surg. 2011; 202: 684-688Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar Vertebral artery dissection has been associated with consequent ischemia more often than hemorrhage; 90% of infarcts due to dissection are thromboembolic rather than hemodynamic in origin9Schievink W.I. Spontaneous dissection of the carotid and vertebral arteries.N Engl J Med. 2001; 344: 898-906Crossref PubMed Scopus (1282) Google Scholar (as a result of emboli from thrombus formation at the site of dissection). Computed tomographic angiography or MRA has largely replaced conventional invasive modalities like digital subtraction angiography.3Chalela J.A. Kidwell C.S. Nentwich L.M. et al.Magnetic resonance imaging and computed tomography in emergency assessment of patients with suspected acute stroke: a prospective comparison.Lancet. 2007; 369: 293-298Abstract Full Text Full Text PDF PubMed Scopus (870) Google Scholar Ischemic strokes are treated with thrombolytics if within the therapeutic window (3-4.5 hours). Antiplatelet/anticoagulation therapy is recommended for at least 3- to 6 months5CADISS Trial InvestigatorsAntiplatelet treatment compared with anticoagulation treatment for cervical artery dissection (CADISS): a randomised trial.Lancet Neurol. 2015; 14 ([published correction appears in Lancet Neurol. 2015;14(6):566]): 361-367Abstract Full Text Full Text PDF PubMed Scopus (310) Google Scholar based on follow-up vessel imaging with CTA/MRA. Imaging is commonly repeated at 3 months, 6 months, and 12 months from the time of diagnosis to assess for recanalization of the dissection. There is a limited role for endovascular stenting, generally reserved for patients with recurrent cerebral ischemia despite appropriate antithrombotics. Most patients with VADs have favorable outcomes because the incidence of symptomatic redissection is minimal at 3 to 6 months, estimated at 0.3% per year.10Lee V.H. Brown Jr., R.D. Mandrekar J.N. Mokri B. Incidence and outcome of cervical artery dissection: a population-based study.Neurology. 2006; 67: 1809-1812Crossref PubMed Scopus (391) Google Scholar Younger age and low NationalInstitutes of Health Stroke Scale scores are predictive of favorable outcomes.13Arnold M. Bousser M.G. Fahrni G. et al.Vertebral artery dissection: presenting findings and predictors of outcome.Stroke. 2006; 37 ([published correction appears in Stroke. 2007;38(1):208]): 2499-2503Crossref PubMed Scopus (273) Google Scholar It is crucial to consider VADs in the differential for headache and vertigo in the younger population, especially in those with a history of head or neck trauma.