A 43-year-old Japanese man with past histories of
oculomotor paralysis, auditory disorder, ischemic
enteritis, and recurrent oral ulceration was referred to our hospital because of blurred vision in his right eye. Ophthalmic examination revealed decreased best corrected visual acuity and
central scotoma in his right eye. Orbit magnetic resonance imaging (MRI) revealed an enlarged SOV in the right eye, with
Gadolinium (Gd) enhancement in the wall of the vein but not inside the vein, indicating
thrombosis. Multiple Gd-enhanced hyperintense lesions were also observed in the juxtacortical area of the brain. We diagnosed the patient with
vasculitis in the right SOV that was adversely affecting the optic nerve. We ruled out systemic
thrombophilia,
infections, and
malignancy by systemic examinations. The
human leukocyte antigen (HLA) typing was Cw1-, B54-, B61-, A2-, A24-, and DR4-positive and B51-negative. We treated the patient with systemic
steroid and
anticoagulant therapy. After three courses of
steroid pulse
therapy, his symptoms and the MRI findings of the right SOV and brain improved; therefore, we decided to discontinue the
anticoagulant therapy. One month after
anticoagulant cessation, MRI revealed recurrence of the
thrombus and enlargement of the right SOV despite the lack of vision worsening. We restarted the
anticoagulant therapy while continuing the oral
prednisolone treatment. At the final visit, 14 months after the onset of the disease, the patient was still receiving oral anticoagulation with
warfarin potassium and
prednisolone (5 mg/day). His symptoms and the right eye's visual function remained normal with a mildly enlarged SOV; there was less Gd enhancement and no brain lesions on MRI.
CONCLUSIONS AND IMPORTANCE: