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Abstract
Purpose
To report a case of monocular elevation deficiency as the presenting manifestation of systemic lupus erythematosus (SLE).
Case summary
A 23-year-old, otherwise healthy female presented with a 3-day history of vertical diplopia and headache. She had a left hypotropia, which worsened in adduction and supra-duction and a profound inferior oblique underaction (-3). Magnetic resonance imaging showed an enhancement around the left superior oblique muscle and multiple infarctions in the left midbrain. On repetitive serological tests, anemia, lymphopenia, and anti-phospholipid antibody were positive. A presumptive diagnosis was a myositis of left superior oblique muscle and hyper-coagulation related with anti-phospholipid antibody. Two months after high-dose steroid treatment, the vertical diplopia was resolved. Five months later, the left hypotropia recurred as a more severe form with the inability to elevate the left eye in all directions. In addition, the infarction associated with vasculitis recurred in the left midbrain. As the treatment with high-dose steroid failed to relieve her ocular symptoms, recession of the left inferior rectus was performed 8 months later. One month after the surgery, she developed multiple lesions of erythematous nodosa with tenderness. Skin biopsy of the lesion in the fingers showed the histological findings consistent with lupus.
References
1. Arevalo JF, Lowder CY, Muci-Mendoza R. Ocular manifestations of systemic lupus erythematosus. Curr Opin Ophthalmol. 2002; 13:404–10.
2. Teoh SC, Yap EY, Au Eong KG. Neuro-ophthalmological manifes-tations of systemic lupus erythematosus in Asian patients. Clin Experiment Ophthalmol. 2001; 29:213–6.
3. Keane JR. Eye movement abnormalities in systemic lupus erythematosus. Arch Neurol. 1995; 52:1145–9.
4. Yoon CK, Park JH, Yu HG. Retinopathy associated with systemic lupus erythematosus. J Korean Ophthalmol Soc. 2009; 50:1215–20.
5. Im CY, Kim SS, Kim HK. Bilateral optic neuritis as first manifes-tation of systemic lupus erythematosus. Korean J Ophthalmol. 2002; 16:52–8.
6. Hwang HS, Kim DH. Transient myopia with severe chemosis asso-ciated with systemic lupus erythematosus. J Korean Ophthalmol Soc. 2007; 48:1445–8.
7. Ahn SM, Kim SS. A case of coexisting neuromyelitis optica in sys-temic lupus erythematosus. J Korean Ophthalmol Soc. 2013; 54:1469–74.
8. Kim IT, Na SC, Lee KJ. Vascular occlusions associated with anti-phospholipid antibodies in systemic lupus erythematosus. J Korean Ophthalmol Soc. 2000; 41:427–32.
9. Kim IT, Chang SD. Papilledema and cerebral venous thrombosis in a patient with systemic lupus ery thematosus. J Korean Ophthalmol Soc. 1999; 40:2015–9.
10. Shin SY, Lee JM. A case of multiple cranial nerve palsies as the ini-tial ophthalmic presentation of antiphospholipid syndrome. Korean J Ophthamol. 2006; 20:76–8.
11. Saleh Z, Menassa J, Abbas O. . Cranial nerve VI palsy as a rare initial presentation of systemic lupus erythematosus: case report and review of the literature. Lupus. 2010; 19:201–5.
12. Koch M, Langmann A. Diplopia as the presenting sign of systemic lupus erythematosus: the chameleon diagnosis. J AAPOS. 2006; 10:184–5.
13. Appenzeller S, Veilleux M, Clarke A. Third cranial nerve palsy or pseudo 3rd nerve palsy of myasthenia gravis? A challenging diag-nosis in systemic lupus erythematosus. Lupus. 2009; 18:836–40.
14. Fonseca P, Manno RL, Miller NR. Bilateral sequential trochleitis as the presenting feature of systemic lupus erythematosus. J Neuroophthalmol. 2013; 33:74–6.
15. Montehermoso A, Cervera R, Font J. . Association of anti-phospholipid antibodies with retinal vascular disease in systemic lupus erythematosus. Semin Arthritis Rheum. 1999; 28:326–32.
Figure 1.
The clinical manifestation and imaging at the initial presentation. (A) Gaze photographs showing a profound inability to elevate the left eye in adduction. (B) Axial T2-weighted, and diffusion brain magnetic resonance imaging (MRI) views showing fo-cal high-signal intensity with diffusion restriction in the left paramedian midbrain suggesting vasculitis and infarction (red arrows) and orbit MRI view with mild haziness around the left superior oblique muscle (yellow arrow).
Figure 2.
The clinical manifestation and imaging five months after the initial presentation. (A) Gaze photographs showing five months after the initial presentation showing a profound inability to elevate the left eye in all directions. (B) Axial T2-weighted, and diffusion brain magnetic resonance imaging (MRI) views showing a new focal high-signal intensity with diffusion restriction in the left paramedian midbrain, more upper segment than previous lesion (red arrows) and orbit MRI view with no remarkable finding in-cluding extraocular muscles.
Figure 3.
Gross appreance and histological finding of the skin lesions. (A) Photographs showing erythematous discoid rash with ten-derness and pruritus at 2nd and 3rd fingers (black arrows). (B) Histologic finding of skin lesion showing perivascular lymphocytic infiltration and a few necrotic keratinocytes suggesting lupus erythematosus (HE stain, ×100). HE = Hematoxylin-Eosin.
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