Background and aims Central nervous system (CNS) involvement is one of the serious complication of systemic lupus erythematosus (SLE). We will show a representative case of SLE to suggest that the delay in the initial treatment will bring aftereffects.

Methods We described a 31-year-old Japanese female patient who was diagnosed with SLE at age 16 y/o. SLE has been treated with prednisone for 14 years. The patient with dysarthria, double vision and vertigo visited a nearby hospital. The first brain MRI scan revealed the absence of infarction. She received administration of intravenous immunoglobulin with a primary diagnosis of Fisher syndrome. One day later, she developed facial nerve palsy, truncal ataxia and lower-extremity weakness. The second brain MRI scan revealed the presence of pontine infarction and she was transferred to our hospital. Anti-Sm antibody and anti-ribosomal-P antibody in sera were positive. On the other hand, lupus anticoagulant and B2GPI cardiolipin antibody were negative. Anti-neuronal antibody in CSF was positive. For such occasions, the chief pathogenesis of this case is considered thrombosis of cerebral artery than CNS angiitis. Treatment was started with pulses of methylprednisolone and pulses of cyclophosphamide, followed by oral prednisone.

Results After intensive medical therapy, dysarthria and facial nerve palsy were improved. However, double vision and truncal ataxia has remained.

Conclusions If effective treatment has not been performed early like this case, it is likely to leave physical impairment. Although oculomotor disturbance is rare complication of SLE, SLE patients must be examined and treated considering that this complication will develop as a part of neuropsychiatric-SLE.