Embouchure Claudication A Trumpeters Unique Presentation of Giant Cell Arteritis

Clinical Correspondence Embouchure Claudication: A Trumpeter’s Unique Presentation of Giant Cell Arteritis Ryan E. Chenevey, MD, Elahhe R. Afkhamnejad, MS, Timothy J. Martin, MD A n 84-year-old white man was seen in the emergency department because of rapidly progressing vision loss in the right eye over a week. He complained of pain in his cheeks and lips beginning after 20–30 minutes of playing trumpet, which progressed in the week before his vision loss. He had lost 9 pounds in the 30 days before presentation. He denied fever, headache, or pain while combing his hair. He was not aware of pain with chewing. On examination, visual acuities were hand motions in his right eye and 20/30 in his left eye. He had an obvious right afferent pupillary defect. Intraocular pressures were 15 mm Hg in the right eye and 13 mm Hg in the left eye. He had no motility deficits or ptosis. The slit-lamp examination was notable only for age-appropriate nuclear sclerotic cataract. The fundus examination revealed 2+ edema of the right optic nerve with a flame hemorrhage inferiorly. The left optic nerve was normal. The remainder of the fundus examination was normal. The patient had profound visual field loss to confrontation in the right eye; visual fields were normal in the left eye. Optical coherence tomography showed retinal nerve fiber layer thickening in the right eye consistent with optic disc edema. Intravenous fluorescein angiography showed patchy choroidal filling with intense staining of the optic disc in the right eye; this was normal in the left eye. Erythrocyte sedimentation rate was elevated at 54 mm/h, and C-reactive protein was elevated at 48.3 mg/L. An MRI/MRA of the brain showed no evidence of acute ischemia, high-grade stenosis, vessel occlusion, or aneurysm. The patient was admitted for intravenous corticosteroids with concern for arteritic anterior ischemic optic neuropathy secondary to giant cell arteritis (GCA). A temporal artery biopsy was positive for GCA, with intimal hyperplasia, focal disruption of the internal elastic lamina, and giant cells infiltrating the muscular wall. The patient received 1 g of intravenous methylprednisolone daily for 3 days while hospitalized, and after discharge received a long-term course of oral prednisone with the addition of tocilizumab (Actemra) in consultation with rheumatology. Vision improved from hand motions at presentation to counting fingers. Almost a year later, the steroid and tocilizumab treatments have improved the patient’s overall health and likely prevented contralateral visual loss. The patient has a dense age-related cataract in his left eye, which is scheduled for removal. He no longer experiences jaw claudication and is beginning to play the trumpet again. GCA is a vasculitis of large and medium vessels that can cause blindness if prompt treatment is not initiated. It is frequently accompanied by symptoms of weight loss, fatigue, fever, and headaches (1). Jaw claudication, manifesting as pain in the muscles of mastication with chewing, was present in 54% of patients with biopsy-proven GCA in a study of 525 patients (2). Another study found that the likelihood of a positive temporal artery biopsy was 9 times greater with complaint of jaw claudication, casting this pain as the strongest clinical indicator of GCA (3). We propose that this patient’s pain in his cheeks and lips when playing the trumpet is from the same mechanism as jaw claudication—ischemia to facial muscles with extended use. Playing a wind instrument such as the trumpet requires extended use of a group of perioral muscles known as the embouchure. These facial muscles, which include the orbicularis oris, buccinator, and depressor anguli oris, are supplied by the second portion of the maxillary artery, a branch off the external carotid artery. This is the same artery that supplies the muscles of mastication (4). This unique case is of interest as this patient’s particular activity, playing the trumpet, uncovered consequences of ischemia from GCA in the distribution of the external carotid arteries, producing the unconventional symptom of embouchure pain. We propose that this symptom is analogous to jaw claudication and may be just as suggestive of GCA in the appropriate clinical setting. Department of Ophthalmology (REC, TJM), Wake Forest School of Medicine, Winston-Salem, North Carolina; and Wake Forest School of Medicine (ERA), Winston-Salem, North Carolina. STATEMENT OF AUTHORSHIP Category 1: a. conception and design: T. J. Martin, R. E. Chenevey, and E. R. Afkhamnejad; b. acquisition of data: T. J. Martin, R. E. Chenevey, and E. R. Afkhamnejad; c. analysis and interpretation of data: T. J. Martin, R. E. Chenevey, and E. R. Afkhamnejad. Category 2: a. drafting the manuscript: T. J. Martin, R. E. Chenevey, and E. R. Afkhamnejad; b. revising it for intellectual content: T. J. Martin, R. E. The authors report no conflicts of interest. Address correspondence to Elahhe R. Afkhamnejad, MS20099 Canyon View Dr., Santa Clarita, CA 91351; E-mail: [e-mail] 498 Chenevey et al: J Neuro-Ophthalmol 2019; 39: 498-499 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence Chenevey, and E. R. Afkhamnejad. Category 3: a. final approval of the completed manuscript: T. J. Martin, R. E. Chenevey, and E. R. Afkhamnejad. REFERENCES 1. Kawasaki A, Purvin V. Giant cell arteritis: an updated review. Acta Ophthalmol. 2009;87:13–32. Chenevey et al: J Neuro-Ophthalmol 2019; 39: 498-499 2. Gabriel SE, O’Fallon WM, Achkar AA, Lie JT, Hunder GG. The use of clinical characteristics to predict the results of temporal artery biopsy among patients with suspected giant cell arteritis. J Rheumatol. 1995;22:93. 3. Hayreh SS, Podhajsky PA, Raman R, Zimmerman B. Giant cell arteritis: validity and reliability of various diagnostic criteria. J Ophthalmol. 1997;123:285–296. 4. Netter F. Arteries of oral and pharyngeal regions. In: Netter F. Atlas of Human Anatomy. 6th edition. Philadelphia, PA: Lippincott Williams & Wilkins, 2014:Plate 72. 499 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.