Co-Cccurrence of Guillain-Barre Syndrome and Primary Sjögren Syndrome in an Elderly Woman

Abstract

Purpose: Co-occurrence of Guillain-Barré syndrome (GBS) and other autoimmune diseases is rare. We present the case of a patient with co-occurrence of GBS and primary Sjögren syndrome (pSS).

Case report: An 82-year-old woman presented with acute ascending flaccid paralysis and acute respiratory failure. Nerve conduction studies and cerebrospinal fluid analysis confirmed the diagnosis of GBS of acute inflammatory demyelinating polyradiculoneuropathy subtype. The initial unresponsiveness to plasma exchange therapy raised the suspicion of other potential diseases. She was later proved to have underlying pSS. Her neurological deficits and respiratory failure improved dramatically with combination therapy of intravenous immunoglobulin (IVIg) and immunosuppressive agent.

Conclusion: pSS should be considered as a possible cause of refractory GBS, particularly in elderly women. Combination therapy with IVIg and immunosuppressive agent may be beneficial.