Downloaded from http://pn.bmj.com/ on September 30, 2017 - Published by group.bmj.com TEST YOURSELF Gesundheit! Sneezing, paraesthesiae and ataxia in a 35-year-old man V Stokes,1 W Küker,2 U G Schulz1 1 Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, UK 2 Department of Neuroradiology, John Radcliffe Hospital, Oxford, UK Correspondence to Dr Ursula Schulz, NIHR Clinician Scientist and Honorary Consultant Neurologist, Nuffield Department of Clinical Neurosciences (Neurology), Level 6, West Wing, John Radcliffe Hospital, Headley Way, Oxford OX3 9DU, UK; ursula.schulz@clneuro.ox.ac.uk A 35-year-old right-handed male chef reported three similar neurological episodes in the preceding 6 months. Each episode began with a paroxysm of sneezing. In the first, sneezing was followed by sudden onset dizziness, and then over a few minutes by double vision, left-sided paraesthesiae, left-sided weakness and then a severe left-sided headache, all resolving after a few hours. The second and third episodes, approximately 2 months apart, were less severe. During the second event, he also noticed his right pupil was bigger than the left. In the third episode, 6 weeks before his clinic visit, he developed several hours of ataxia and double vision, but no sensory or motor symptoms; this was followed by left-sided headache. The patient underwent MRI brain and magnetic resonance angiography (MRA) of the cervical vessels (figure 1). Question 1 What does the imaging show? Comment The MRA shows a dissection of the proximal right vertebral artery, causing a stenosis and a pseudoaneurysm distal to the stenosis, with thrombus in the pseudo-aneurysm. The MRI brain also shows an arteriovenous malformation (AVM) in the right temporoparietal lobe. Question 2 What is the diagnosis? What would you do next? 362 Practical Neurology 2011;11:362–365. doi:10.1136/practneurol-2011-000044 Comment The most likely diagnosis is recurrent ischaemic events due to a vertebral artery dissection, with subsequent embolisation from either the dissection or from thrombus in the pseudoaneurysm. Pseudoaneurysms develop when haemorrhage into the blood vessel wall separates the media from the adventitia during a dissection. A false lumen occurs if blood re-enters the true vessel lumen. The dissection lumen may thrombose and cause stenosis of the true lumen or there may be embolisation from the thrombus. Pseudoaneurysms develop after 10–46% of vertebral artery dissections, although they resolve spontaneously in 80%; even if they persist, they only very rarely cause a stroke.1 Apart from antiplatelet therapy, intervention is therefore generally not recommended. Our patient had been asymptomatic for 2 months when we discussed the investigation results with him. We therefore opted for conservative management with low-dose aspirin, and further clinical and imaging follow-up. The patient initially did not return as planned, but then returned to clinic several months later following a more prolonged episode of leftsided sensory symptoms. It emerged that there had been at least 10 further episodes over the preceding 6 months. Although not entirely stereotyped, they were all similar: the patient sneezed and would then notice diplopia, unsteadiness and left-sided paraesthesiae, although not all three symptoms on every occasion. Symptom severity and duration varied between some minutes Downloaded from http://pn.bmj.com/ on September 30, 2017 - Published by group.bmj.com TEST YOURSELF and several hours and appeared to be related to the force of sneezing. The patient admitted that he was becoming increasingly afraid of sneezing, apprehensive that this might set off further symptoms. Further imaging showed a persisting pseudoaneurysm, which had slightly decreased in size. Brain imaging showed no ischaemic lesions. Nevertheless, we still felt that the most likely diagnosis was recurrent embolisation from the aneurysm, triggered by sneezing. It transpired that the patient had not taken his aspirin, due to previous peptic ulcer disease. He was now started on clopidogrel, but continued to have symptoms. He returned to hospital some weeks later after a particularly severe attack, with persisting diplopia, ataxia and left-sided sensory loss. The MRI brain now demonstrated several acute cerebellar infarcts (figure 2), strongly supporting the diagnosis of recurrent ischaemic events. As these had continued with the patient on antiplatelet treatment, we now had to consider more invasive management, as the patient was at risk of further, potentially more severe events. Figure 1 MRI brain and magnetic resonance angiogram at the initial presentation. The MRA (A, B) shows the dissected right vertebral artery with a proximal stenosis and a pseudoaneurysm distal to this. MRI brain (C) shows the right parietal arteriovenous malformation. Question 3 Which other treatment options would you consider? Comment Further management options to reduce recurrent embolism from the aneurysm included anticoagulation and endovascular treatment. As the benefit of anticoagulation over antiplatelet therapy in this situation is uncertain, and because the patient strongly preferred intervention to longterm medication, we proceeded with endovascular treatment. We considered two options: to coil the aneurysm, preserving the vessel lumen with a stent, or to occlude the vertebral artery with coils. Inserting a stent would have been technically difficult and risky in this case because of kinking and stenosis of the vessel. Furthermore, a stent would remain a potential source of further thromboembolism and require long-term antiplatelet therapy. Coil occlusion of the parent vessel provides a definitive treatment of a pseudoaneurysm, with no further risk of embolisation and no need for long-term medication. In patients with intracranial dissecting aneurysms it is the preferred treatment if sufficient collaterals exist,2 and it also appeared to be the safest and most durable treatment in our patient. He tolerated a test occlusion of his diseased vertebral artery well and underwent coil occlusion without complication (figure 3). At 6 months after the procedure, he reported no further events of Question 4 Is the AVM is related to the patient’s presentation? How would you manage it? Figure 2 Acute cerebellar infarcts on T2-weighted (A) and diffusion-weighted (B) MRI brain, and coronal fluid attenuated inversion recovery images (C). Diffusion-weighted imaging shows that the right-sided cerebellar infarct (black), is older than the left-sided infarcts. Practical Neurology 2011;11:362–365. doi:10.1136/practneurol-2011-000044 363 Downloaded from http://pn.bmj.com/ on September 30, 2017 - Published by group.bmj.com TEST YOURSELF Question 5 What do you make of the association of this patient’s symptoms with sneezing? Comment Figure 3 Intra-arterial angiogram. (A) Shows the pseudoaneurysm and demonstrates the stenosis and vascular kink proximal to the aneurysm, which would have made stenting difficult. (B) The occluded vessel after coil embolisation. neurological disturbance. More importantly, he has also sneezed on multiple occasions, without provoking symptoms. Comment Flow-related aneurysms close to AVMs have been described. However, in our patient the AVM and the dissection were too far apart for flow-related phenomena to have an effect. It is possible that the patient has an underlying vascular disorder that predisposed him both to the development of AVM and of arterial dissections, but this is speculative. Nevertheless, the AVM is a further management issue. AVMs are usually diagnosed when they cause symptoms, mostly seizures or haemorrhage. Our patient’s AVM is asymptomatic, and the risk of future complications is uncertain. The annual risk of haemorrhage from an asymptomatic AVM is estimated to be 1–2%.3 As our patient is still young, he has a considerable lifetime risk of haemorrhage, which must be weighed against the procedural risk of any intervention, and the uncertainty that such an intervention would provide a definitive cure. The ARUBA trial (A Randomised Trial of Unruptured Brain AVMs, www.arubastudy.org) compares the outcome of conservative management with intervention in patients with AVM that have not bled. We have discussed the uncertainties of AVM management with the patient. He is keen on intervention, and after several discussions with neuroradiologists and neurosurgeons, stereotactic radiosurgery was felt to be the most straightforward and lowest risk treatment to offer him, which he is currently awaiting. 364 Practical Neurology 2011;11:362–365. doi:10.1136/practneurol-2011-000044 The intriguing part of this patient’s presentation was the association of his symptoms with sneezing. Sneezing has been reported as a possible cause for vertebral artery dissection. The mechanism is unclear, but pressure changes or neck movements may lead to an intimal tear and dissection of the vessel wall. Similarly, they may also cause thrombus to dislodge. Given the presence of ischaemic lesions on the patient’s third magnetic resonance scan, and the cessation of attacks after occluding the vertebral artery, it now appears certain that he had recurrent embolic events triggered by sneezing. However, it is surprising that sneezing was the only mechanism to set off the events: coughing or Valsalva manoeuvres never preceded the patient’s neurological symptoms. We therefore considered that sneezing may have been a symptom of, rather than the trigger for, the patient’s attacks. This is not inconceivable, as the ‘sneezing centre’ is in the lateral medulla, a frequent infarct location in vertebral artery dissection. However, although sneezing may rarely be a symptom of ischaemia, such infarcts more commonly present with an inability to sneeze. It seems unlikely that sneezing, if a symptom, would have occurred with all attacks, and only at their onset. Finally, given the high number of similar attacks, we considered that the patient might have had focal seizures originating from his AVM. Sneezing can be a seizure symptom, but it is usually associated with temporal lobe epilepsy. The AVM was in the wrong location for such seizures, and overall the attacks did not appear sufficiently stereotyped to be epileptic. Learning points ■ ■ ■ Pseudoaneurysms develop after 10–46% extracranial vertebral artery dissections. These are usually asymptomatic and usually resolve spontaneously. Embolisation from a vertebral artery pseudoaneurysm is rare and intervention beyond treatment with antiplatelet agents is generally not required. Further treatment options include occlusion of the parent vessel and coiling and/or stenting of the aneurysm. The best way forward will depend on the individual vascular anatomy and on local facilities and expertise. Downloaded from http://pn.bmj.com/ on September 30, 2017 - Published by group.bmj.com TEST YOURSELF Competing interests None. Provenance and peer review Not commissioned; externally peer reviewed. References 1. Touzé E, Randoux B, Méary E, et al. Aneurysmal forms of cervical artery dissection: associated factors and outcome. Stroke 2001;32:418–23. 2. Yamaura I, Tani E, Yokota M, et al. Endovascular treatment of ruptured dissecting aneurysms aimed at occlusion of the dissected site by using Guglielmi detachable coils. J Neurosurg 1999;90:853–6. 3. Al-Shahi R, Warlow C. A systematic review of the frequency and prognosis of arteriovenous malformations of the brain in adults. Brain 2001;124:1900–26. Practical Neurology December 2011 Vol 11 No 6 365 Downloaded from http://pn.bmj.com/ on September 30, 2017 - Published by group.bmj.com Gesundheit! Sneezing, paraesthesiae and ataxia in a 35-year-old man V Stokes, W Küker and U G Schulz Pract Neurol 2011 11: 362-365 doi: 10.1136/practneurol-2011-000044 Updated information and services can be found at: http://pn.bmj.com/content/11/6/362 These include: References Email alerting service This article cites 3 articles, 1 of which you can access for free at: http://pn.bmj.com/content/11/6/362#BIBL Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article. Notes To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/