Perineural Optic Nerve Enhancement in Giant Cell Arteritis: A Case Report and Review of the Literature

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Perineural Optic Nerve Enhancement in Giant Cell Arteritis: A Case Report and Review of the Literature Kimberly K. Nguyen, BS, Bayan A. Al Othman, MD, Ashwini T. Kini, MD, Andrew G. Lee, MD Downloaded from http://journals.lww.com/jneuro-ophthalmology by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC1y0abggQZXdtwnfKZBYtws= on 05/04/2022 G iant cell arteritis (GCA) is a medium–to-large vessel vasculitis of the elderly. Visual symptoms occur in 25%–50% of GCA cases secondary to inflammatory occlusion of the ophthalmic artery or short posterior ciliary arteries, resulting in ischemia to the optic nerve or optic nerve head (1). Perineural optic nerve enhancement uncommonly presents in the setting of GCA (2). We present a case of biopsy-proven GCA associated with concurrent bilateral optic nerve sheath enhancement on MRI that initially presented as diplopia and resulted in rapidly progressive vision loss. We review the literature on this unusual combination of clinical and radiographic findings. This is an unusual case of GCA-related perineural optic nerve enhancement presenting with efferent and subsequent afferent visual pathway–related symptoms. CASE REPORT An 82-year-old woman presented to an outside ophthalmologist for transient binocular diplopia. The eye examination was normal, and the diplopia was attributed to Parkinson disease. The medical history was significant for Parkinson disease, Crohn disease, hypothyroidism, coronary artery disease, arteriosclerotic cardiovascular disease, and cardiac arrhythmia. Her medications included levothyroxine, balsalazide, spironolactone, aspirin, carbidopalevodopa, amantadine, metoprolol succinate XL, pravastatin, and topical tacrolimus. Social, family, and allergic histories were otherwise noncontributory. One month after the transient diplopia episode, she developed subacute, painless, unilateral loss of vision of the left eye. New left-sided temple pain, jaw claudication, and University of Texas Health Science Center at Houston (KKN), Houston, Texas; Department of Ophthalmology (BAAO, ATK, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medicine, New York, New York; Department of Ophthalmology (AGL), University of Texas Medical Branch, Galveston, Texas; University of Texas MD Anderson Cancer Center (AGL), Houston, Texas; Texas A and M College of Medicine (AGL), Bryan, Texas; and Department of Ophthalmology (AGL), The University of Iowa Hospitals and Clinics, Iowa City, Iowa. The authors report no conflicts of interest. Address correspondence to Andrew G. Lee, MD, Blanton Eye Institute, Houston Methodist Hospital, 6560 Fannin Street, Suite 450 Houston, TX 77030; E-mail: [e-mail] e42 a 10-pound weight loss were present. Examination showed visual acuity of 20/30 in the right eye and 20/100 left eye with a left relative afferent pupillary defect. Automated perimetry (Humphrey visual field [HVF] 24-2) showed a dense inferior nasal step and superior arcuate defect with a mean deviation of 27.57 dB in the right eye and a dense central scotoma with a superior and inferior arcuate defect in the left eye (Fig. 1). External, anterior segment, motility, and intraocular pressure examinations were normal in both eyes. Dilated fundus examination showed a normal optic nerve in the right eye and pallid edema in the left eye. The erythrocyte sedimentation rate (ESR) was elevated at 80 mm/hours, and C-reactive protein (CRP) was elevated at 6.65 mg/L (normal less than 2 mg/L). She was admitted to the hospital and initiated on high-dose intravenous methylprednisolone. Her vision continued to decline to hand motion level in the left eye despite steroid treatment. MRI of the brain showed bilateral optic nerve sheath enhancement greater on the left than the right (Fig. 2). Temporal artery biopsy showed a full-thickness lymphocytic infiltrate, myxoid degeneration, and epitheliod histiocytes associated with coagulative necrosis consistent with GCA. She was discharged on 60 mg oral prednisone with outpatient follow-up. At 1 month follow-up, the patient’s visual acuity remained stable in the right eye and improved to counting fingers in the left eye. Ocular coherence tomography showed sector pallor in the right eye and diffuse optic atrophy in the left eye. Repeat HVF was stable for both eyes, and ESR and CRP were normal. She was referred to the rheumatology clinic to consider steroid sparing agents initiation with tapering of steroids. DISCUSSION GCA is a systemic medium–to-large vessel vasculitis that presents with variable symptoms such as headache, scalp tenderness, jaw claudication, and vision loss. ESR and CRP are typically elevated, and diagnosis is made with temporal artery biopsy. Significant optic nerve sheath enhancement on MRI is a rare finding in patients with biopsyproven GCA, and optic perineuritis (OPN) may be considered in the differential diagnosis (3). Nguyen et al: J Neuro-Ophthalmol 2021; 41: e42-e45 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 1. Humphrey visual field (HVF) 24-2 demonstrates a dense inferior nasal step and superior arcuate defect on the right and a dense central scotoma with a superior and inferior arcuate defect on the left. OPN is an uncommon inflammatory disorder that occurs most commonly in women in their fourth decade of life. It commonly presents as painful acute or subacute monocular vision loss due to optic nerve sheath inflammation compressing the optic nerve. Inflammation of the surrounding structures may result in extraocular symptoms, such as diplopia, ptosis, and ocular motor nerve palsies that can assist in the diagnosis of OPN although they are less common (1,2). MRI in OPN reveals enhancement of the optic nerve sheath and may involve other structures in the orbit (2). Perineural enhancement of the optic nerve has also been described in association with underlying conditions such as sarcoidosis, granulomatosis with polyangiitis, syphilis, herpes zoster, malignancy, and tuberculosis (3,4). Treatment is aimed at the underlying etiology; however, in idiopathic OPN, the treatment of choice is IV methylprednisolone. Visual prognosis is favorable with early initiation of medical therapy in patients with OPN. Relapses are common and typically respond to retreatment with corticosteroids (1,2). To the best of our knowledge, 6 previous studies have described optic nerve sheath enhancement in the setting of biopsy-proven GCA (Table 1). Our case is unique in that the patient experienced transient diplopia 1 month before diagnosis of GCA. We believe that the combination of transient efferent (diplopia) and subsequent afferent (ischemic optic neuropathy) signs and symptoms correlates with the radiographic findings of perineural optic nerve sheath enhancement due to GCA. Clinicians should be aware that FIG. 2. A. Coronal T1 post contrast MRI of the orbits shows enhancement of bilateral optic nerve sheaths, left more than the right. B. Axial T1 post contrast MRI of the orbits shows enhancement of bilateral optic nerve sheaths, left more than the right. Nguyen et al: J Neuro-Ophthalmol 2021; 41: e42-e45 e43 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence TABLE 1. Previously reported cases of giant cell arteritis with optic nerve sheath enhancement on MRI Author Symptoms at Presentation Age (yrs) Gender Pappolla et al (1) 76 Female Inferior visual field defect in the left eye, jaw claudication, frontal headache. Morotti et al (2) 74 Male Liu et al (3) 67 Male 1st presentation: fluctuating diplopia. 2nd presentation: subacute blurred vision in the right eye, central scotoma in the left eye, headache in the left periorbital region. 3rd presentation: left temporal headache, jaw claudication, mild bilateral skin necrosis of temporal region. Superior and central visual field loss, flashing lights, bilateral jaw claudication. Liu et al (4) 83 Female e44 Headache, hand motion vision in the right eye, and asymptomatic left eye. Lab Studies Treatment Outcome ESR 48 mm/hr, CRP 8.1 mg/L. Normal or negative RPR/VDRL, ANA, ANCA, anti-dsDNA, antiRo/La, RF, complement levels, LDH, TSH, B12, HIV, HbA1C. Normal CSF analysis. Temporal artery ultrasound showed hypoechogenic halo and narrowing of the arterial lumen bilaterally. MRI showed bilateral OPN. ESR 46 mm/hr, CRP 42.5 mg/L. Normal or negative serology for syphilis, HSV, VZV, CMV, toxoplasma gondii, HIV, HBV, HCV, borrelia burgdorferi, TB, ACE, ANA, ANCA, complement levels, Coombs test, B12, and folate. Normal CSF analysis. Temporal artery biopsy was negative. MRI showed bilateral OPN. ESR 27 mm/hr, CRP 0.7 mg/dL. Normal or negative ANA, ANCA, RF, dRVVT, anti-dsDNA, antiRo/La, complement levels, LDH, peripheral blood smear, NMO-IgG, TSH, B12, B1, copper, zine, RPR, HIV, lyme disease, HbA1C. CT chest, abdomen, and pelvis showed no malignancy. CSF analysis showed elevated protein concentration (91 mg/dL). Temporal artery biopsy confirmed GCA. MRI showed bilateral optic nerve sheath enhancement. ESR 36 mm/hr, platelets 494,000/mm3. Normal or negative findings for additional autoimmune, inflammatory, and neoplastic etiologies. Normal CSF analysis. Temporal artery biopsy consistent with GCA. MRI showed bilateral perineural optic nerve enhancement. IV methylprednisolone followed by oral prednisone taper over 1 year. Residual peripheral visual field defect in the left eye with preserved central vision. Resolved OPN on MRI. IV methylprednisolone followed by highdose oral prednisone with gradual taper. Complete blindness bilaterally without improvement. IV methylprednisolone followed by slow taper of oral prednisone. Visual acuity remained stable in the right eye and declined to 20/400 in the left eye. IV methylprednisolone followed by oral prednisone taper over several months. NLP vision bilaterally. Nguyen et al: J Neuro-Ophthalmol 2021; 41: e42-e45 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence (Continued ) Author Age (yrs) Gender Liu et al (4) 68 Female Morgenstern et al (5) 83 Our Patient 82 Symptoms at Presentation Lab Studies Treatment Outcome Acute blurred vision left eye. ESR 10 mm/hr, CRP 1.1 ng/dL, normal platelet count. IV methylprednisolone followed by oral prednisone taper. Male Acute bilateral vision loss, fever (101.5°F), neck pain. IV methylprednisolone followed by oral prednisone taper over 18 months. Female 1st presentation: transient diplopia 2nd presentation; subacute, painless, unilateral loss of vision of the left eye, leftsided temple pain, jaw claudication, 10-pound weight loss ESR 60 mm/hr. Normal CSF analysis. Negative serology for syphilis. Temporal artery biopsy confirmed GCA. MRI showed bilateral enhancement of the optic nerve sheath. Biopsy of optic nerve sheath demonstrated giant cells. ESR 80 mm/hr, CRP 6.65 mg/L. Temporal artery biopsy confirmed GCA. MRI showed bilateral enhancement of the optic nerve sheath, left greater than right. Visual acuity was 20/20 right eye and NLP left eye remained stable. Visual acuity was NLP right eye and 20/40 left eye and remained stable. IV methylprednisolone followed by oral prednisone taper. Visual acuity was 20/30 right eye and CF left eye and remained stable. ACE, angiotensin-converting Enzyme; ANA, antinuclear antibody; ANCA, antineutrophil cytoplasmic antibodies; CF, counting fingers; CMV, cytomegalovirus; CRP, C-reactive protein; dRVVT, dilute Russell viper venom test; GCA, giant cell arteritis; HBV, hepatitis B virus; HCV, hepatitis C virus; HSV, herpes simplex virus; LDH, lactate dehydrogenase; NMO, neuromyelitis optica; OPN, optic perineuritis; RF, rheumatoid factor; RPR/VDRL, Rapid Plasma Reagin/Venereal Disease Research Laboratory; TB, tuberculosis; TSH, thyroid-stimulating hormone; VZV, varicella zoster virus. although optic nerve sheath enhancement on MRI may suggest OPN, GCA can mimic both afferent and efferent presentations of orbital inflammatory disease. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: K. K. Nguyen, B. A. Al Othman, A. T. Kini, and A. G. Lee; b. Acquisition of data: K. K. Nguyen, B. A. Al Othman, A. T. Kini, and A. G. Lee; c. Analysis and interpretation of data: K. K. Nguyen, B. A. Al Othman, A. T. Kini, and A. G. Lee. Category 2: a. Drafting the manuscript: K. Nguyen, B. A. Al Othman, A. T. Kini, and A. G. Lee; b. Revising it for intellectual content: K. K. Nguyen, B. A. Al Othman, A. T. Kini, and A. G. Lee. Category 3: a. Final approval of the completed manuscript: K. K. Nguyen, B. A. Al Othman, A. T. Kini, and A. G. Lee. Nguyen et al: J Neuro-Ophthalmol 2021; 41: e42-e45 REFERENCES 1. Pappolla A, Silveira F, Norscini J, Miquelini L, Patrucco L. Bilateral optic perineuritis as initial presentation of giant cell arteritis. Neurologist. 2019;24:26–28. 2. Morotti A, Liberini P, Padovani A. Bilateral optic perineuritis as the presenting feature of giant cell arteritis. BMJ Case Rep. 2013;2013:bcr2011007959. 3. Liu TY, Miller NR. Giant cell arteritis presenting as unilateral anterior ischemic optic neuropathy associated with bilateral optic nerve sheath enhancement on magnetic resonance imaging. J Neuroophthalmol. 2015;35:360– 363. 4. Liu K, Chesnutt D. 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