Journal of Neuro-Ophthalmology, September 2012, Volume 32, Issue 3

Lyme-Associated Orbital Inflammation Presenting as Painless Subacute Unilateral Ptosis

A 90-year-old woman presented with subacute painless left ptosis. Examination of the left eye revealed ptosis with loss of the superior eyelid sulcus, 2 mm of proptosis, mild tenderness with retropulsion, and optic disc edema. Levator function and extraocular movements were normal, and there was no relative afferent pupillary defect. MRI demonstrated thickening of the extraocular muscles in the left orbit with lacrimal gland enlargement and mild enhancement of the optic nerve sheath. Serology revealed a positive enzyme-linked immunosorbent assay for Lyme antibodies and a positive Western blot of Lyme IgG titer. The patient recalled a tick bite 6 months earlier, at which time Lyme serologies were negative. After 3 weeks of intravenous ceftriaxone, she had a significant improvement and a full recovery by 3 months. Lyme disease should be included in the differential diagnosis of orbital inflammation, especially in Lyme-endemic areas.

Lyme-Associated Orbital Inflammation Presenting as Painless Subacute Unilateral Ptosis Luna Xu, BS, Bryan J. Winn, MD, Jeffrey G. Odel, MD Abstract: A 90-year-old woman presented with subacute painless left ptosis. Examination of the left eye revealed ptosis with loss of the superior eyelid sulcus, 2 mm of proptosis, mild tenderness with retropulsion, and optic disc edema. Levator function and extraocular movements were normal, and there was no relative afferent pupillary defect. MRI demonstrated thickening of the extraocular muscles in the left orbit with lacrimal gland enlargement and mild enhancement of the optic nerve sheath. Serology revealed a positive enzyme-linked immunosorbent assay for Lyme antibodies and a positive Western blot of Lyme IgG titer. The patient recalled a tick bite 6 months earlier, at which time Lyme serologies were negative. After 3 weeks of intrave-nous ceftriaxone, she had a significant improvement and a full recovery by 3 months. Lyme disease should be included in the differential diagnosis of orbital inflammation, especially in Lyme-endemic areas. Journal of Neuro-Ophthalmology 2012;32:246-248 doi: 10.1097/WNO.0b013e318234dafc © 2012 by North American Neuro-Ophthalmology Society CASE REPORT A90-year-old woman fromConnecticut presented with sub-acute painless left eyelid ptosis and increased tearing for 1 month accompanied by mild fatigue and unintentional weight loss. She recalled a tick bite to her right ear 6 months earlier without associated erythema migrans; although Lyme titers were negative, she was treated presumptively with a 3-week course of 100 mg of oral doxycycline twice daily. Ophthalmologic examination revealed visual acuity of 20/25, right eye, and 20/50, left eye. Pupils were 2 mm and reactive on the right eye and postsurgically irregularly dilated on the left eye. There was no relative afferent pupillary defect. Color vision was normal in the right eye and reduced in the left eye. On slit-lamp examination, posterior lenses were present and there was no evidence of uveitis. Extraocular movements were full. There was no resistance to retropulsion of the globes, although there was tenderness on the left eye with retropulsion and 2 mm of left proptosis. There was loss of the left superior lid sulcus, and left ptosis was present with a margin reflex distance-1 (MRD-1) of 5 mm on the right and 2 mm on the left (Fig. 1). Levator function was 15 mm bilaterally. Cranial nerves V and VII were intact bilaterally. Funduscopic examination on the left eye demonstrated mild optic disc edema with peripapillary hemorrhages (Fig. 2), while the right fundus was normal. Automated visual field testing was unreliable while confrontation fields were full. MRI dem-onstrated increased thickness of the extraocular muscles in the left orbit with mild enlargement of the lacrimal gland and contrast enhancement of the optic nerve sheath (Fig. 3). Laboratory results revealed a positive enzyme-linked immunosorbent assay for Lyme antibodies (Lyme index 5.08; normal range, ,1.00), with a positive Western blot for B. burgdorferi IgG (8 bands) and negative IgM. Com-plete blood count, angiotensin-converting enzyme, rapid FIG. 1. At presentation, the patient had 3 mm of left ptosis with the loss of the superior eyelid sulcus. College of Physicians and Surgeons (LX), Columbia University, New York, New York; and Department of Ophthalmology (BJW, JGO), Columbia University, New York, New York. The authors report no conflicts of interest. L. Xu was funded by the Doris Duke Clinical Research Fellowship. Address correspondence to Bryan J. Winn, MD, Harkness Eye Institute, Columbia University Medical Center, 635 W. 165th Street, Box EI 106, New York, NY 10032; E-mail: [e-mail] 246 Xu et al: J Neuro-Ophthalmol 2012; 32: 246-248 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. plasma reagin, C-reactive protein, erythrocyte sedimenta-tion rate, antineutrophil cytoplasmic antibody, myasthenia gravis panel, and thyroid panel (T4, thyroid-stimulating hormone, anti-thyroid peroxidase, antithyroglobulin) were unremarkable. The patient was treated with a 3-week course of 1,500 mg of intravenous ceftriaxone daily. At a 3-week follow-up visit, her vision on the left eye had improved to 20/25 with normal color vision. MRD-1 on the left increased to 3.5 mm. At 3 months, the patient's ptosis (Fig. 4) and optic disc edema had completely resolved. DISCUSSION Seidenberg and Leib (1) first described Lyme-associated or-bital myositis in a 5-year-old girl in 1990. Our literature search revealed 4 cases of serologically confirmed Lyme-associated orbital inflammation, all of which had a compo-nent of orbital myositis (2-5). Lyme antibodies are usually detected 6 weeks after the initial infection. This may explain the negative Lyme titer 6 months prior to our patient's pre-sentation and her later seroconversion. The most common presentation of orbital myositis is acute and unilateral, with symptoms of orbital and periorbital pain worsening with ocular movement (85%-100% of cases), diplopia (50%- 100% of cases), mild proptosis of 1-2 mm (30%-56% of cases), swollen eyelids, and conjunctival hyperemia (6). Our patient's presentation was unusual, as her primary complaint was unilateral painless ptosis without diplopia. The initial differential diagnosis also included neoplasm, thyroid orbit-opathy, myasthenia gravis, and levator aponeurosis dehis-cence. The diffuse thickening of extraocular muscles seen on MRI was previously reported in other orbital Lyme myo-sitis cases (4,7). Unilateral optic disc edema and optic nerve sheath enhancement suggested a diagnosis of optic perineur-itis. Optic perineuritis can spare central visual field and acuity and thus may account for our not seeing a relative afferent pupil defect or a central scotoma on visual field testing. The reported cases of Lyme orbital myositis can be categorized as manifestations of early disseminated disease or late disease. Although 2 g of intravenous ceftriaxone daily or 20-24 million units of penicillin G daily are typically recommended as first-line agents for ophthalmic or neurologic disease, there are no clear recommendations for the treatment of orbital disease without ocular or neurologic involvement (8). Successful treatment of Lyme orbital inflammation with either 3-4 weeks of oral doxycycline (3,4,7) or 2-3 weeks of intravenous ceftriaxone (2,5) has been reported. In our case, the history of prior doxycycline treatment at the time of the initial tick bite prompted intravenous ceftriaxone treatment. REFERENCES 1. Seidenberg KB, Leib ML. Orbital myositis with Lyme disease. Am J Ophthalmol. 1990;109:13-16. FIG. 2. The left optic nerve is edematous with flame hem-orrhages surrounding the disc. FIG. 3. Contrast-enhanced T1 axial (A) and coronal (B) fat-suppressed orbital MRI shows enlargement of the extra-ocular muscles and lacrimal gland (arrow) on the left and enhancement of the left optic nerve sheath (arrowhead). FIG. 4. Three months after intravenous ceftriaxone treat-ment, the superior eyelid sulcus returned and the ptosis of the left eye resolved. Xu et al: J Neuro-Ophthalmol 2012; 32: 246-248 247 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. 2. Carvounis PE, Mehta AP, Geist CE. Orbital myositis associated with Borrelia burgdorferi (Lyme disease) infection. Ophthalmology. 2004;111:1023-1028. 3. Nieto JC, Kim N, Lucarelli MJ. Dacryoadenitis and orbital myositis associated with Lyme disease. Arch Ophthalmol. 2008;126:1165-1166. 4. Pendse S, Bilyk JR, Lee MS. The ticking time bomb. Surv Ophthalmol. 2006;51:274-279. 5. Holak H, Holak N, Huzarska M, Holak S. Tick inoculation in an eyelid region: report on five cases with one complication of the orbital myositis associated with Lyme borreliosis. Klin Oczna. 2006;108:220-224. 6. Costa RM, Dumitrascu OM, Gordon LK. Orbital myositis: diagnosis and management. Curr Allergy Asthma Rep. 2009;9:316-323. 7. Fatterpekar GM, Gottesman RI, Sacher M, Som PM. Orbital Lyme disease: MR imaging before and after treatment: case report. AJNR Am J Neuroradiol. 2002;23:657-659. 8. Balcer LJ, Winterkorn JM, Galetta SL. Neuro-ophthalmic manifestations of Lyme disease. J Neuroophthalmol. 1997;17:108-121. 248 Xu et al: J Neuro-Ophthalmol 2012; 32: 246-248 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. [CLorbitalinfections]