Anti-neurofascin antibody is frequently present in patients with CCPD. Recognition of this antibody may be important, because patients with CCPD who are antibody positive respond well to IV immunoglobulin or plasma exchange.

BackgroundRheumatoid meningitis (RM) is a rare disorder that often develops during a remission phase of rheumatoid arthritis (RA). This is the first study to demonstrate differences in regard to immunological disturbance between blood and cerebrospinal fluid (CSF) samples obtained from a patient with RM using flow cytometry.Case presentationA 36-year-old woman with RA and generalized myasthenia gravis (MG) developed RM during a remission phase. Although both RA and MG were stable and well controlled, she noticed fever, headache, and transient sensory disturbance. Blood and CSF examination findings suggested aseptic meningitis, while brain magnetic resonance imaging revealed restricted portions of meningitis and associated cortical lesions, compatible with a diagnosis of RM. The dose of oral prednisolone was increased, which ameliorated the symptoms within 1 week along with improvement in CSF findings. This patient exhibited features of RM that were manifested in a manner independent of the activity of RA. An investigation of cellular immunity using CSF specimens with flow cytometry showed differences in regard to the pathogenesis of inflammation in the CSF as compared to outside of the central nervous system. In contrast to results obtained with paired blood samples, CSF cells at the peak stage of RM showed a marked increase in CCR3+ Th2 cells and marked decrease in CD8+ cells, suggesting an immunoregulatory disturbance in the CSF. Those findings indicated a CSF-specific activation of humoral immunity, resulting in augmentation of meningeal inflammation, as shown by excess synthesis of intrathecal IgG and markedly elevated interleukin-6 level. Results of the present detailed investigation of lymphocyte subsets revealed a discrepancy regarding the process of inflammation in this RM patient between CSF and blood samples.ConclusionsRM is not a simple reflection of the immune status of RA, as the pathogenesis seems related to, at least in part, CSF-specific immunological dysregulation.

HLA genotype-clinical phenotype correlations are not established for multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD). We studied HLA-DRB1/DPB1 genotype–phenotype correlations in 528 MS and 165 NMOSD cases using Japan MS/NMOSD Biobank materials. HLA-DRB1*04:05, DRB1*15:01 and DPB1*03:01 correlated with MS susceptibility and DRB1*01:01, DRB1*09:01, DRB1*13:02 and DPB1*04:01 were protective against MS. HLA-DRB1*15:01 was associated with increased optic neuritis and cerebellar involvement and worsened visual and pyramidal functional scale (FS) scores, resulting in higher progression index values. HLA-DRB1*04:05 was associated with younger onset age, high visual FS scores, and a high tendency to develop optic neuritis. HLA-DPB1*03:01 increased brainstem and cerebellar FS scores. By contrast, HLA-DRB1*01:01 decreased spinal cord involvement and sensory FS scores, HLA-DRB1*09:01 decreased annualized relapse rate, brainstem involvement and bowel and bladder FS scores, and HLA-DRB1*13:02 decreased spinal cord and brainstem involvement. In NMOSD, HLA-DRB1*08:02 and DPB1*05:01 were associated with susceptibility and DRB1*09:01 was protective. Multivariable analysis revealed old onset age, long disease duration, and many relapses as independent disability risks in both MS and NMOSD, and HLA-DRB1*15:01 as an independent risk only in MS. Therefore, both susceptibility and protective alleles can influence the clinical manifestations in MS, while such genotype–phenotype correlations are unclear in NMOSD.

576http://msj.sagepub.com consequences of MS-related fatigue that might guide future studies to focus on specific questions.

Objective Systemic inflammation is known to be associated with Alzheimer's disease (AD) advancement. The aim of the present study was to examine the relationships of cognitive function in AD patients with the presence of systemic inflammation and immunological alterations. Methods Eight patients with AD and nine elderly individuals as a control group were enrolled, none of whom had a history of autoimmune diseases or were suffering from an infection. C-reactive protein, interleukin-6 and tumor necrosis factor-a levels in serum were measured, while lymphocyte subsets were also determined using a flow cytometry system. In addition, two of the AD patients received repeated cognitive function and blood testing on an outpatient basis. Results In the AD group, the percentages of CD3 À CD16 + CD56 + (natural killer) and CD4 + CCR5 + (type 1 helper T) cells, and CD8 + CD11a + (cytotoxic T) lymphocytes were increased, whereas the percentages of CD19 + (B lymphocytes) and CD4 + CD25 bright CD127 dim (regulatory T) cells were decreased as compared with the controls. Systemic inflammation, represented by elevated C-reactive protein and interleukin-6, was shown to aggravate cognitive function in both patients who underwent follow-up outpatient examinations. However, lymphocyte subsets did not change in parallel with cognitive function. Conclusions Inflammatory acute clinical events have the potential to reduce cognitive function in AD patients, which could be partially attributed to a decrease in regulatory T cells in the blood. Furthermore, increased numbers of natural killer cells, cytotoxic T lymphocytes and type 1 helper T cells in these patients might reflect the presence of chronic inflammation, thus forming an immunological background.