Glycosphingolipids are now known to be rapidly converted to mediators for inflammatory reactions or to signaling molecules that control inflammatory events in the nervous system. The present study aimed to explore whether disturbed glycolipids and sphingolipids metabolism in the nervous system is present in patients with a neuroinflammatory disorder, Encephalo-Myelo-Radiculo-Neuropathy (EMRN), because most EMRN patients have been reported to exhibit autoantibodies against neutral glycolipids. We recruited 8 patients with EMRN, 6 patients with Parkinson’s disease (PD), and 20 neurologically normal control subjects for serum determination of the active fragment of C5 complements. For sphingolipids and glycolipids determination with LC-MS/MS in CSF, we recruited 8 patients with EMRN, 6patients with PD, and 6 neurologically normal controls. ELISA for activated peripheral C5 complement, C5a and mass spectrometric analysis of cerebrospinal fluid were performed as previously reported1. Furthermore, all sera from the patients with EMRN were subjected to Far-Eastern blot analysis employing neutral glycolipids such as lactosylceramide (LacCer) as antigens. The data disclosed a significant upregulation of active fragment C5 complement, C5a levels in sera as well as a significant accumulation of species-specific ceramides but not sphingomyelin in cerebrospinal fluid from EMRN patients2. Furthermore, we confirmed the occurrence of anti-neutral glycolipids antibodies, especially anti-LacCer antibodies in all EMRN patients.