P485: Event-related potentials in patients with primary Sjögren’s syndrome

Abstract

s of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S179 Abstract P484 – Figure 2P484 – Figure 2 neuropathy with a high variable clinical course and outcome. Despite immunotherapy up to 20% of patients remain severely disabled and mortality in the first year is approximately 4%. Aim of this study is to perform an epidemiological analysis of clinical and electrophysiological features of patients admitted in our ward for GBS in last ten years; to test the incidence of different GBS subtypes and their clinical and electrophysiological evolution; to test on our sample the prognostic value of variables known in literature and we suggested integration with new prognostic markers. We collected data prospectively from 89 patients regarding: demographic features, previous infections, clinical manifestations, kind of therapy received, motor deficit and disability (evaluated respectively with MRC sumscore and GBS disability score (GBSDS)). We measured dosage of protein in the cerebrospinal fluid (CSF). Serial nerve conduction studies (NCS) were performed. Differences between groups were compared by Fisher’s exact test. Potential prognostic factors were tested in uni and multivariable logistic analysis and was estimated by regression analysis. 19% of GBS were acute motor axonal neuropathy (AMAN) and showed more severe prognosis. High age was predictive of poor prognosis in acute inflammatory demyelinating neuropathy (AIDP) but not in AMAN. High GBSDS at nadir and cranial nerves involvement were predictive of being enable to run at 1 year. Conduction block did not have a significant distribution in GBS subtypes and showed no correlation with clinical outcome. In NCS performed between 30 and 90 days from the clinical onset, a pathological distal cMAP duration (DcMAPD) represented a distinctive marker of primary demyelinating pathophysiological mechanism, in our sample of GBS. The endurance of pathological DcMAPD showed a predictive value on long term disability in AIDP. This study confirms the key-role of serial NCS for a proper diagnosis of GBS subtypes. Further clinical-electrophysiological studies are necessary to define more inclusive work-up in GBS enabling forecasting of the patients with poorest long term outcome.