Several patients who had a progressive
clinical course involving both the central and peripheral nervous systems have been reported, but the diagnostic marker has been remained uncertain. More recently, such patients were reported to have namely "encephalomyeloradiculoneuropathy (EMRN)" associated with anti-neutral
glycosphingolipid (GSL)
antibodies. These
antibodies were reported to disappear from the serum in the recovery phase, but whether this finding applies to the cerebrospinal fluid (CSF) remains uncertain. We describe a 67-year-old man with EMRN in whom we measured anti-neutral GSL
antibodies in serial serum and CSF samples. During the disease course, the optical densities of the positive band against the background intensity ratio (-<0.3; ±≥0.3 to <0.6; +≥0.6 to <1.0; 2+≥1.0 to <2.0; 3 +≥2.0) for serum and CSF anti-
lactosylceramide (LacCer)
antibodies were found to be as follows: 2+ and 1+ at the first admission, ± and - when the consciousness level improved after
immunotherapy, - and 1+ at clinical relapse, and ± and - when the consciousness level improved after
immunotherapy. This is the first time to document that clinical relapse occurred in EMRN, and at this time the negative anti-LacCer
antibodies in CSF after the first course of
immunotherapy turned positive, but this was not seen in serum samples.