To describe an uncommon initial presentation of SLE, with successful treatment outcome.

The prevalence of neuropsychiatric systemic lupus erythematosis (NSPLE) ranges from 12-95% and varies in its manifestation. Psychosis is uncommon (2.5-3.5%) and cranial nerve palsies are rare (0.5-1%).

22-year-old woman presented with 2 days of vomiting, confusion and agitation. Her acute onset disorientation and combativeness resulted in intubation. Past medical history suggested aloplecia, 6 month history of Raynaud’s phenomenon, and bilateral joint swelling.  With improved agitation she was extubated on day 2. Subsequent clinical examination showed mildly impaired comprehension, periorbital edema, scleral edema, complete ptosis of left eye, dysconjugate gaze, involvement of cranial nerve III, IV and VI in the left eye and III and VI in the right eye, mild right upper extremity weakness, and brisk deep tendon reflexes and positive Babinski sign on the left.

MRI brain and orbit showed multiple patchy areas of restricted diffusion and gyral edema with abnormal leptomeningeal and cranial nerve enhancement, most suspicious for meningoencephalitis. MRA and MRV brain were unremarkable. Echocardiogram, and CT chest, abdomen and pelvis were unremarkable. CSF analysis showed elevated lymphocytes and proteins.  Several viral and bacterial titers, including cultures, were negative. Laboratory studies showed elevated ESR, positive ANA, elevated rheumatoid factor, Smith and Smith/RNP  antibodies and positive lupus anticoagulant. A diagnosis of lupus was suspected. Parenteral methylprednisolone was administered for 5 days in addition to one dose of IV cyclophosphamide. There was significant improvement in ophthalmoplegia and comprehension. She was discharged on day 8 on prednisone taper, hydroxychloroquine and atovaquone. Repeat brain MRI at 5 weeks showed only mild residual gliosis in the left lateral temporal lobes. At 9 week follow up, symptoms, including ophthalmoplegia, had completely resolved.

This case highlights the importance of early recognition of rare presentations of lupus meningoencephalitis for optimal management.