Nonarteritic Jaw Claudication

A 60-year-old woman with decreased visual acuity in her right eye and right-sided jaw claudication was found to have ocular ischemic syndrome secondary to complete occlusion of the brachiocephalic artery. Although jaw claudication is often considered to be pathognomonic for giant cell arteritis, it has a broad differential diagnosis including both vascular and nonvascular conditions.

Clinical Observation Nonarteritic Jaw Claudication Christine A. Petersen, MD, Courtney E. Francis, MD Abstract: A 60-year-old woman with decreased visual acuity in her right eye and right-sided jaw claudication was found to have ocular ischemic syndrome secondary to complete occlusion of the brachiocephalic artery. Although jaw claudication is often considered to be pathognomonic for giant cell arteritis, it has a broad differential diagnosis including both vascular and nonvascular conditions. Journal of Neuro-Ophthalmology 2017;37:281-284 doi: 10.1097/WNO.0000000000000497 © 2017 by North American Neuro-Ophthalmology Society J aw claudication is a symptom that often is discussed in the setting of a giant cell arteritis (GCA), for which it is a highly specific symptom (1). We present the case of a patient with jaw claudication and ocular ischemic syndrome. She had normal erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels, a negative temporal artery biopsy, and Doppler ultrasonography demonstrated complete occlusion of her brachiocephalic artery. This case highlights the fact that any condition which leads to decreased blood flow through the facial branches of the external carotid artery may lead to jaw claudication. CASE REPORT A 60-year-old woman with a medical history including diabetes mellitus type 2 peripheral vascular disease, chronic obstructive pulmonary disease, hypertension, hyperlipidemia, and obstructive sleep apnea was evaluated for a second opinion regarding vision loss in her right eye. Six months before our evaluation, she was seen Department of Ophthalmology, University of Washington, Seattle, Washington. Supported by unrestricted departmental grant from Research to Prevent Blindness. The authors report no conflicts of interest. Address correspondence to Courtney E. Francis, MD, HMC, # 359608, 325 9th Avenue, Seattle, WA 98104-2499; E-mail: francis3@ uw.edu Petersen and Francis: J Neuro-Ophthalmol 2017; 37: 281-284 by an optometrist for transient visual loss in her right eye. She also reported pain in the right eye and right temple region, scalp tenderness to palpation, weight loss, hip and shoulder pain, and pain in the jaw and temporal region with prolonged chewing. Visual acuity was 20/20, right eye, and 20/25, the left eye. Dot and blot hemorrhages were noted in both eyes. Her ESR was 22 mm/hr. An ultrasound study revealed a completely occluded brachiocephalic artery with retrograde flow in the right vertebral artery, right ophthalmic artery collateralization, and stenosis of the left carotid and subclavian system. Brain computed tomography without contrast showed dense vascular calcifications along the circle of Willis. She was advised to follow-up with her primary care physician. Four months later, the patient underwent left common femoral endarterectomy and patch angioplasty by left external iliac artery angiography, during which she had 300 mL of blood loss. She experienced postoperative hypotension requiring intensive care unit admission with 3 liters intravenous fluid resuscitation and vasopressors to maintain a main arterial pressure in the low 60s. After the procedure, the patient noted decreased vision in the right eye with a visual acuity of 20/100. She reported increasing headaches, scalp tenderness, and right-sided jaw claudication. Fluorescein angiography revealed diffuse choroidal ischemia of the right eye and delayed vascular filling of the left eye. ESR was 60 mm/hr and CRP 0.1 mg/L (normal , 10.0 mg/L). She was prescribed prednisone 60 mg/day for presumed GCA. The following day, a right temporal artery biopsy of 2.3 cm was obtained and revealed no evidence of GCA. Despite continuing oral corticosteroids, visual acuity in the right eye declined to counting fingers. She self-discontinued the prednisone after 1 month because of steroid side effects and self-referred to the neuroophthalmology clinic. On examination, visual acuity was count fingers at 4 feet, right eye, and 20/20, left eye. Her right pupil was middilated and minimally reactive with a relative afferent pupillary defect. Intraocular pressure was 26 mm Hg in the right eye and 24 mm Hg in the left eye. Slit-lamp 281 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation FIG. 1. Fundus appearance shows dilated veins and intraretinal hemorrhages (inset) in the right eye. examination of the right eye revealed neovascularization of the iris including the angle. Ophthalmoscopy of the right fundus showed dot and blot hemorrhages and dilated veins (Fig. 1). Her right central retinal artery was noted to pulsate without applying any external pressure on the globe. Fluorescein angiography of the right eye demonstrated choroidal nonperfusion with delayed arterial filling time and late vascular staining (Fig. 2). The patient's ESR and CRP were 16 mm/hr and 0.4 mg/L, respectively. She was diagnosed with ocular ischemic syndrome in the right eye, started on brimonidine drops, and referred to the retina clinic for panretinal photocoagulation. FIG. 2. Fluorescein angiogram of the right eye at 30.37 seconds (A), 43.65 seconds (B), 1:19.95 minutes (C), and 6:45.45 minutes (D). There is delayed choroidal filling with patchy perfusion, delayed arteriovenous transit time, and prominent vascular staining. 282 Petersen and Francis: J Neuro-Ophthalmol 2017; 37: 281-284 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation TABLE 1. Odds ratio for a positive temporal artery biopsy in patients with jaw claudication from suspected GCA Article Gonzalez-Lopez et al (3) Chmelewski et al (4) Rieck et al (5) Rodriguez-Pla et al (6) Moutray et al (7) Roth et al (8) Vilaseca (9) Hayreh et al (1) Mari et al (10) No. of TABs No. of Positive TABs (%) 335 98 82 125 110 51 103 363 278 81 30 22 46 21 7 45 106 79 No. of Patients With Jaw Claudication (%) No. of Patients With Jaw Claudication With Positive TAB (%) 52 (16) 27 (28) 31 (60) 15 (56) 23 (28) 17 (74) 5 (10) 26 (25) 3 (60) 19 (73) 57 (21) 30 (53) (24) (31) (27) (37) (19)* (14) (44) (29) (28) OR P Value 4.5 4.67 3.71 7.13 6.7 15.75 5.32 9.0 2.9† 0.007 0.0015 0.016 ,0.0001 0.0088 0.0009 ,0.0001 0.005 *5% of TABs evaluated were reported to be inadequate. † Reported as relative risk, not OR. GCA, giant cell arteritis; OR, odds ratio; TAB, temporal artery biopsy. DISCUSSION Jaw claudication denotes pain in the muscles of mastication due to ischemia from decreased flow through the facial branches of the external carotid artery (2). This symptom is strongly associated with GCA, and many studies have documented that the presence of jaw claudication greatly increases the chances of a positive temporal artery biopsy (Table 1). The diagnosis of GCA must be strongly considered in any patient presenting with jaw claudication, especially in the setting of other signs and symptoms of GCA including temporal headache, scalp tenderness, fever, fatigue, weight loss, anorexia, proximal muscle pain, or laboratory findings including elevated ESR or CRP, thrombocytosis, or anemia. Failure to consider the diagnosis can be dangerous given the high risk of vision loss or even loss of life due to myocardial infarction or cerebrovascular accident. However, there are many other conditions that can cause jaw claudication, which may or may not be on a vascular basis (Table 2) (11,12). TABLE 2. Causes of jaw claudication Vascular Giant cell arteritis Carotid atherosclerosis Thoracic aortic dissection Amyloidosis Polyarteritis nodosa Granulomatosis with polyangiitis Cryoglobulin-induced vasculitis Takayasu arteritis Nonvascular Temporal mandibular joint (TMJ) dysfunction Rheumatoid arthritis involving the TMJ Myasthenia gravis Parotid gland tumors McArdle disease Subacute thyroiditis Petersen and Francis: J Neuro-Ophthalmol 2017; 37: 281-284 Amyloidosis and other vasculitides including polyarteritis nodosa, granulomatosis with polyangiitis, cryoglobulininduced vasculitis and Takayasu arteritis can cause jaw claudication (13,14). In a study of 237 patients with primary systemic amyloidosis, Gertz et al (2) found that 9% of cases had jaw claudication. Takayasu arteritis can appear clinically very similar to GCA including loss of vision. However, distinguishing features include the age of onset, symptoms, and ethnicity (14). Vascular disease of the brachiocephalic, common carotid, or external carotid arteries may cause jaw claudication. Examples include dissection of the thoracic aorta including the arch (15) and carotid atherosclerosis. Atherosclerosis involving the external carotid artery is often less symptomatic than that involving the internal carotid artery. Interestingly, carotid stenting as an alternative treatment to carotid endarterectomy for carotid artery stenosis can worsen occlusive disease of the external carotid artery, if the stent is placed over the origin of the external carotid. This may cause or worsen jaw claudication, but the effect is generally transient and resolves without intervention (16,17). The co-occurrence of jaw claudication and ocular ischemic syndrome in our patient was unusual and initially there was concern for GCA. However, there was complete occlusion of the brachiocephalic artery. This led to jaw claudication due to decreased blood flow through the external carotid and ocular ischemic syndrome due to decreased flow through the internal carotid artery with retrograde flow through the right ophthalmic artery. While jaw claudication is a symptom highly suggestive of GCA, the clinician should maintain a broad differential diagnosis for this patient complaint. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: C. E. Francis; b. Acquisition of data: C. E. Francis and C. Petersen; c. Analysis and interpretation of data: C. Petersen and C. E. Francis. Category 2: a. Drafting the 283 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation manuscript: C. Petersen; b. Revising it for intellectual content: C. E. Francis. Category 3: a. Final approval of the completed manuscript: C. E. Francis. REFERENCES 1. Hayreh SS, Podhaisky PA, Raman R, Zimmerman B. Giant cell arteritis: validity and reliability of various diagnostic criteria. Am J Ophthalmol. 1997;123:285-296. 2. Gertz MA, Kyle RA, Griffing WL, Hunder GG. Jaw claudication in primary systemic amyloidosis. Medicine (Baltimore). 1986;65:173-179. 3. González-López JJ, González-Moraleja J, Burdaspal-Moratilla A, Rebolleda G, Núñez-Gómez-Álvarez MT, Muñoz-Negrete FJ. Factors associated to temporal artery biopsy result in suspects of giant cell arteritis: a retrospective, multicenter, case-control study. Acta Ophthalmol. 2013;91:763-768. 4. Chmelewski WL, McKnight KM, Agudelo CA, Wise CM. Presenting features and outcomes in patients undergoing temporal artery biopsy. A review of 98 patients. Arch Intern Med. 1992;152:1690-1695. 5. Rieck KL, Kermani TA, Thomsen KM, Harmsen WS, Karban MJ, Warrington KJ. 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