Dados do Trabalho

Title

Guillain-Barré syndrome preceding the diagnosis of Sjögren's syndrome: a case report

Background

Guillain-Barré Syndrome (GBS) is an acute, immuno-mediated and ascending polyneuropathy rarely associated with other autoimmune diseases. This case report aims to describe the occurrence of GBS, with later manifestation of Sjögren's Syndrome (SS).

Case report

Female patient, 72 years old, Caucasian, widow, farmer, hypertensive. Admitted with a history of 3 days of generalized weakness and intense myalgia, associated with fluid, mucous and bloodless diarrhea, vomiting, food intolerance, arthralgia, hyporexia, general malaise, fever not thermometrically with chills, diffuse erythematous macules and pruritus beginning at the extremities, later spreading through the body. During hospitalization, after 12 days, the patient developed marked thrombocytopenia (approximately 50.000/ mm³), severe hypokalemia (1.3mmol / L), lower limb motor deficit (LMW), low back pain, lip rhythm deviation, speech pain in the mandibular region. On physical examination, muscle strength decreased (grade III in upper limbs (MMSS) and II in MMII by the Medical Research Council scale) and abolished reflexes with maintained sensitivity. After neurological evaluation, the hypothesis of Guillain-Barré Syndrome was proposed, with a motor deficit grade IV in the Hughes Clinical Scale. Serologies for dengue, HIV I and II, HBSAG, ANTI-HCV and VDRL, all with non-reactive results. Luck showed proteinorinity of 67mg / dl (VR: 10-45), with no other alterations. Electroneuromyography of the MMSS and MMII revealed diffuse axonal loss, with preservation of the sensory fibers, a result compatible with the diagnostic hypothesis of acute motor axonal neuropathy (Guillian Barré variant form). Magnetic resonance imaging of normal brain. FAN positive (1: 160), with dense fine dotted nuclear pattern. Requested C3, C4, rheumatoid factor, ANTI-RO, ANTI-LA, ANTI-SM, VHS and PCR. After the 30th day of hospitalization, human immunoglobulin (IgH) was started 400mg / kg. It evolved well clinically after administration of 5 consecutive days of IgH, with significant improvement of motor deficit (grade II on the Hughes clinical scale) and lip rhythm deviation. After a 6-month follow-up, he complained of dry mouth, blurred vision and dry eye sensation, with palpebral ptosis on the right and xerosis on physical examination, with anti-RO positivity: 50 (VR <7). diagnosis of Sjögren's Syndrome. Initiated treatment with hydroxychloroquine 400mg / day and linseed oil.

Conclusion

In conclusion, this report describes a rare case of GBS preceding the diagnosis of SS and its laboratory investigation, presenting a favorable outcome after treatment, without symptomatic recurrence.

Área

Sjögren’s Syndrome