J Korean Ophthalmol Soc.  2016 Jan;57(1):161-166. 10.3341/jkos.2016.57.1.161.

Systemic Lupus Erythematosus Presenting as Monocular Elevation Deficiency

• Kim SW
• Kim YH
• Chung SA

• KMID: 2213864
• DOI: http://doi.org/10.3341/jkos.2016.57.1.161

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.
CONCLUSIONS
Eye movement abnormality can be an initial manifestation of SLE, which should be considered as a differential diagnosis especially in young female patients.

Keyword

Anti-phospholipid antibody; Monocular elevation deficiency; Systemic lupus erythematosus

MeSH Terms

Anemia
Biopsy
Diagnosis
Diagnosis, Differential
Diplopia
Eye Movements
Female
Fingers
Headache
Humans
Infarction
Lupus Erythematosus, Systemic*
Lymphopenia
Magnetic Resonance Imaging
Mesencephalon
Myositis
Serologic Tests
Skin
Vasculitis
Young Adult

Figure

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  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).

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  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.

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  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.

Reference

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