Dados do Trabalho

Title

A 60 YEAR-OLD FEMALE PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND ANTIPHOSPHOLIPID SYNDROME PRESENTING WEAKNESS IN LEGS AND ARMS

Background

Neurologic and psychiatric manifestations of systemic Lupus erythematosus (SLE) are serious but incompletely understood. The ACR case definitions for neuropsychiatric SLE(NPSLE) constitutes of 19 syndromes, all related with significant negative impact on patient quality of life. The correct diagnosis, the attribution of the syndrome to SLE or other causes, and the proper treatment are still challenging.

Case report

A female 60 years-old Caucasian patient presented to the emergency department with right-sided arm and leg weakness and numbness, urinary retention and dizziness. Upon physical examination she had strength 2/5 in both arms, 3/5 in right leg and 4/5 in left leg. All reflexes and sensibility were unremarkable. She has a history of SLE and Antiphospholipid syndrome (APS), but with no previous thrombotic events. Analysis of cerebrospinal fluid showed 16 cells, 95% mononuclear and 5% polymorphonuclear, Glucoses 52mg/dL, total protein 69 mg/dL. Herpes-virus simplex, cytomegalovirus and Epstein-Barr virus serologies were negative, as well acid-fast bacilli smear for Tuberculosis and India ink smear for Cryptococcosis. Cranial Magnetic Resonance Imaging (MRI) showed areas of gliosis in right frontal and occipital lobes and left temporal lobe. Spinal MRI showed in T2 showed high-signal intensity in posterior area of the spinal cord from C3 to C7 and from to D8-D9. Patient was treated with metilprednisolone 500mg pulse for 5 days, Cyclophosphamide 0,75g/m², Rituximab 1g in day 0 and day 15, and anticoagulation was started. Later, serum antibory against aquaporin-4 (AQP-4) was positive. Patient evolved with strength 3/5 in upper levels, better control of sphincter, bur kept low limbs weakness. A diagnosis of neuromyelitis optica was given.

Conclusion

Myelopathy is a described syndrome in NPSLE, but the attribution of this case to SLE or primary cause is a challenge. Anyway treatment was giving focusing in inflammatory and ischemic causes, obtaining partial response. It is necessary advances in neuroimaging and biomarkers to better understanding of NPSLE.