PMC5893640_01
Thoracic magnetic resonance imaging (MRI). T2-weighted thoracic MRI showed abnormal high signals in the spinal cord (A-C). After 5 months, the high signals were diminished on the follow-up thoracic MRI (D,E).
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Spinal magnetic resonance imaging (MRI) showed abnormal high-intensity signals in the thoracic spinal cord (Figure 1).
Clinical course and serum and cerebrospinal fluid anti-lactosylceramide antibodies.
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The clinical course is shown in Figure 2.
Serial Far-Eastern blot analyses. Neutral glycosphingolipids (GSLs), such as lactosylceramide, glucosylceramide, and galactosylceramide (GalCer) were applied onto thin-layer chromatography plates (TLC plates) to obtain the respective pure neutral GSLs, and they were developed as described previously. The separated neutral GSLs were transferred to a regular polyvinylidene difluoride membrane (PVDF membrane) with a thermal blotter (ATTO, Tokyo, Japan). The positive band(s) were detected with a chemiluminescence detection system. The separated GSLs were probed with serum from a healthy volunteer [(1): negative control] and with serum from a patient with encephalomyeloradiculoneuropathy [(2): positive control]. Panel (3) shows the separated GSLs from the patient at the following multiple time-points: serum at the first admission, cerebrospinal fluid (CSF) at the first admission, serum at the second admission, CSF at the second admission, serum after final immunotherapy, and CSF after final immunotherapy. Positive bands were detected with serum and CSF from the patient at the first admission and CSF from the patient at the second admission. A positive band was weakly detected with serum from the patient after final immunotherapy. Sera and CSF are usually 1,000- and 5-times diluted with the antibody reaction buffer, respectively. Lactosylceramide and glucosylceramide were applied to (a) lane, whereas GalCer was applied to each (b) lane of the TLC plates.
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During the disease course, the optical density ratios of serum and CSF anti-LacCer antibodies were as follows: 2+ and 1+ at the first admission, +- and 1+ when the consciousness level decreased despite steroid pulse therapy, +- and - when the consciousness level improved after the first IVIg treatment, - and 1+ at clinical relapse, and +- and - when the consciousness level improved after increasing the dose of prednisolone and administering the third IVIg treatment (optical density ratio: -<0.3; +->=0.3 to <0.6; +>=0.6 to <1.0; 2 +>=1.0 to <2.0; 3 +>=2.0) (Figure 3).