Pôster:

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease of unknown cause that can affect any organ system, most frequently the skin, joints, kidneys, and the nervous, hematologic, and cardiovascular systems. Neurological and psychiatric symptoms are reported in 20 to 95% of SLE patients in the pediatric age group. The most common neurological manifestations of SLE are cognitive dysfunction, stroke, seizures, headaches, and peripheral neuropathy. Movement disorders, cranial neuropathies, ocular involvement, transverse myelitis, and meningitis are less reported.

Case report

Female patient, 10 years old, admitted with arthritis in the elbow and left wrist. Associated with arthritis, she presented more pronounced choreiform movements in the left upper limb, dyslalia, abnormal gait and emotional lability. After 15 days of the initial condition, diffuse petechiae appeared throughout the body and ecchymosis in the left periorbital region. It was diagnosed as Rheumatic Fever, which was treated with Penicillin G Benzathine, Prednisone and Haloperidol. MRI was performed demonstrating aneurysmal dilatation in the M1 segment of the right middle cerebral artery and hypersignal foci in Flair located in semioval centers that could represent gliosis. Then, there was remission of arthritis and chorea. Seven months after the initial treatment, she returned to the service because of right knee arthritis and arthralgia in elbows. On discontinuation of Haloperidol there was a return of choreatic symptoms. During this period, petechiae also appeared on lower limbs. Autoantibody search revealed anti-nuclear antibody reagent with nuclear fine speckled pattern (Title 1/640); Anti-SSA antibody 8.9 U / ml and anti-SSB less than 7 U / ml; anti-beta-2-glycoprotein 1 antibody IgG 24.1 U / ml and IgM 11.8 U / ml. With these results a diagnosis of Systemic Lupus Erythematosus and Antiphospholipid Antibody Syndrome was established. Treatment with hydroxychloroquine, ASA, Prednisone and Amitriptyline was initiated, with symptom remission.

Conclusion

Chorea is the most important movement disorder of SLE, occurring, in most cases, at the onset of the disease. However, the prevalence of this manifestation is 0 - 7% in patients with SLE of the pediatric age group. Because of its rarity, it is necessary to exclude other more frequent causes such as Syndenham's chorea, in rheumatic fever, mainly in Latin American, African and Asian countries. In addition, the investigation of antiphospholipid antibodies in cases of unexplained chorea in the pediatric population should be performed whenever is possible, due to the strong association between Antiphospholipid Antibody Syndrome and chorea.