The clinical manifestations of nervous system involvement in systemic lupus erythematosus (SLE) are highly diverse and their pathogenic mechanisms are incompletely understood. Neuropsychiatric SLE (NPSLE) poses difficulty in making the proper diagnosis, especially in circumstances where its initial symptoms are diffuse neuropsychiatric symptoms. We describe a 43-year old woman who exhibited a myoclonic jerk of the abdominal wall, followed shortly by acute confusion, which was attributed to SLE. Therapy with high dose corticosteroids completely reversed the symptoms. Myoclonus can be an anticipatory symptom of diffuse neurologic dysfunction in patients with NPSLE.