Clinical Review & Education JAMA Ophthalmology Clinical Challenge A Special Case of Horner Syndrome Kinda Najem, MD; Edward A. Margolin, MD A man aged 51 years was admitted to the internal medicine service with a recent history of left-sided hemibody paresthesia, dysphagia, and ataxia. He also reported a single episode of binocular diplopia that lasted for 1 day and resolved spontaneously. Otherwise, his medical history was unremarkable. On physical examination, there was mild right-sided ptosis and miosis. Pain and temperature sensation were not evaluated. After instilling 10% topical cocaine eyedrops, the right pupil did not change its size whereas the left pupil dilated by 2 mm, supporting a diagnosis of right-sided Horner syndrome. With the exception of delayed initiation of saccades, extraocular motility was full, and there was no nystagmus. WHAT WOULD YOU DO NEXT? A. Perform a lumbar puncture and measure opening pressure and cerebrospinal fluid composition B. Order magnetic resonance imaging and magnetic resonance angiography of the brain and cervical spine C. Perform an apraclonidine test to confirm diagnosis of Horner syndrome D. Order tests for erythrocyte sedimentation rate, C-reactive protein level, and syphilis serologies jamaophthalmology.com (Reprinted) JAMA Ophthalmology Published online August 18, 2016 Copyright 2016 American Medical Association. All rights reserved. Downloaded From: http://archopht.jamanetwork.com/ by a Cornell University User on 08/19/2016 E1 Clinical Review & Education JAMA Ophthalmology Clinical Challenge Diagnosis A Apparent diffusion coefficient map Lateral medullary syndrome B Diffusion-weighted magnetic resonance image What to Do Next? B. Order magnetic resonance imaging and magnetic resonance angiography of the brain and cervical spine Discussion The patient’s multiple neurologic symptoms prompted urgent imaging of the brain and were likely localized to the brainstem. In all patients with Horner syndrome, angiography imaging of the neck (computed tomography angiography or magnetic resonance angiography) should also be performed to fully evaluate the sympathetic chain and to specifically rule out a carotid artery dissection. Apraclonidine testing is not used in the setting of acute Horner syndrome, as the adrenergic denervation hypersensitivity can take a few weeks to develop. Lumbar puncture and serologic testing could be indicated in subsequent workup, but they should be performed after neuroimaging. Brain magnetic resonance imaging demonstrated restricted diffusion within the right medulla consistent with the recent (10 days or less) infarct (Figure). There was a flow void within the posterior inferior cerebellar artery, which likely represented a thrombus or an embolus within this vessel. Wallenberg syndrome, the eponym for lateral medullary syndrome, arises from the infarction of the lateral medulla and is usually secondary to atherothrombosis, embolism originating from the heart, or arterial dissection.1 Atherosclerosis is more likely to be found in older patients with vascular risk factors, arterial dissection is more common in younger patients and can either be spontaneous or associated with trauma, and embolism should be contemplated in patients with valvular disease or arrhythmias.2,3 Wallenberg syndrome is usually caused by the compromise of the ipsilateral vertebral artery and, occasionally, the posterior inferior cerebellar artery.3 The syndrome is characterized by impaired pain and temperature sensation of the ipsilateral face and contralateral trunk and limbs, ipsilateral central Horner syndrome, and ipsilateral ataxia, dysarthria, and dysphagia.2,3 Crossed hemisensory disturbances are reported in 90% of cases in large case series, making this finding highly sensitive.1,4,5 Patients often complain of vertigo and sensations of environmental tilt; they will report that the Figure. A, Apparent diffusion coefficient map demonstrates abnormal diffusion restriction within the right medulla (arrowhead), consistent with an acute infarct. B, Diffusion-weighted imaging sequence on magnetic resonance imaging shows high signal within the right medulla (arrowheads). room is tilted upside down or to the side.3 Another common symptom is lateropulsion of the body and of the oculomotor system; patients will describe a sensation of being pulled toward the side of the lesion, and when asked to fixate straight ahead, eyes will move toward the side of the lesion.2,3 Nystagmus can be part of the syndrome and can be horizontal, torsional, or both. Ocular tilt reaction, skew deviation, abnormal smooth pursuits, and saccades are also often present.2,3 While most patients with Wallenberg syndrome will present to an emergency department rather than the ophthalmologist’s office, ocular symptoms can be dominant, and thus, ophthalmologists should be familiar with this condition. Prompt recognition of the lateral medullary infarction is of utmost importance, as treatment options are time sensitive. This syndrome is usually associated with a good prognosis, with most patients recovering with minimal sequelae at 6 months.1,6 Patient Outcome The patient was referred to a stroke prevention clinic and received anticoagulation. His left-sided hemibody paresthesia, dysphagia, and ataxia progressively improved during his admission. No further episodes of diplopia developed. ARTICLE INFORMATION REFERENCES Author Affiliations: Department of Ophthalmology, University of Toronto, Toronto, Ontario, Canada. 1. Fukuoka T, Takeda H, Dembo T, et al. Clinical review of 37 patients with medullary infarction. J Stroke Cerebrovasc Dis. 2012;21(7):594-599. Corresponding Author: Kinda Najem, MD, 801 Eglinton Ave West, Ste 301, Toronto, ON M5N 1E3, Canada (kinda.najem@gmail.com). 2. Day GS, Swartz RH, Chenkin J, Shamji AI, Frost DW. Lateral medullary syndrome. CJEM. 2014;16(2): 164-170. Published Online: August 18, 2016. doi:10.1001/jamaophthalmol.2016.1654. 3. Zee DS, Newman-Toker D. Supranuclear and internuclear ocular motility disorders. In: Miller NR, Newman NJ, Biousse V, Kerrison JB, eds. Walsh and Hoyt’s Clinical Neuro-Ophthalmology. London, England: Lippincott Williams & Wilkins; 2005: 907-967. Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. 4. Kameda W, Kawanami T, Kurita K, et al; Study Group of the Association of Cerebrovascular Disease in Tohoku. Lateral and medial medullary infarction. Stroke. 2004;35(3):694-699. 5. Kim JS. Pure lateral medullary infarction. Brain. 2003;126(pt 8):1864-1872. 6. Nelles G, Contois KA, Valente SL, et al. Recovery following lateral medullary infarction. Neurology. 1998;50(5):1418-1422. Additional Contributions: We thank the patient for granting permission to publish this information. E2 JAMA Ophthalmology Published online August 18, 2016 (Reprinted) Copyright 2016 American Medical Association. All rights reserved. Downloaded From: http://archopht.jamanetwork.com/ by a Cornell University User on 08/19/2016 jamaophthalmology.com