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Central nervous system (CNS) involvement, including encephalopathy, encephalitis, leptomeningitis, and pachymeningitis, in rheumatoid arthritis (RA) is rather rare. We report the case of a 61-year-old female with a history of RA in remission for 7 years, who presented with numbness, weakness of the left upper limb, dysarthria, and headache. Magnetic resonance imaging (MRI) of the brain showed meningeal enhancement in the frontal, parietal, and temporal lobes. Cerebrospinal fluid (CSF) examination detected high levels of both rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibody (ACPA), with a high ACPA-immunoglobulin G index (> 2.0). She was diagnosed with rheumatoid meningitis. Following combined therapy with oral prednisolone and intravenous infusion of cyclophosphamide, her symptoms promptly improved. After treatment, RF and ACPA levels in the CSF were reduced, and MRI showed improvement of the meningeal structures. This case, along with existing literature, suggests that the ACPA level in the CSF may serve as a useful marker for diagnosing of CNS involvement in RA, as well as an index of effectiveness of the associated treatment.

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We report a 68-year-old man without clinical history of rheumatoid arthritis who presented with acute bilateral palsy of the IX and X cranial nerves secondary to pachymeningitis confirmed on cranial MRI. Rheumatoid factor in both serum and cerebrospinal fluid and anti-agalactosyl IgG antibody in serum were positive. The radiographs of hands and feet revealed signs of early rheumatism. The dural biopsy specimen showed chronic inflammation with infiltration of lymphocytes and histiocytes. A diagnosis of rheumatoid cranial pachymeningitis was made. Treatment with long-term corticosteroid was excellently effective.

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Paired serum and cerebrospinal fluid (CSF) specimens from 30 multiple sclerosis (MS) patients and 30 matched neurological control (NC) patients were quantitatively tested for IgM-class rheumatoid factor (RF). Significantly elevated RF levels were found in serum from 6 MS patients and 12 NC patients. Seven of the latter 12 patients had a diagnosis of recurrent head pain. RF was detected in CSF from 2 MS patients and 2 NC patients. In 3 of the 4 cases, this could be explained by blood-brain barrier damage or normal diffusion of RF into CSF. Intrathecal RF synthesis was found in the remaining MS patient. These results suggest that RF production may be related to underlying pathological mechanisms in at least some MS and NC patients. They also confirm that RF must be carefully accounted for in etiological studies designed to demonstrate pathogen-specific IgM antibodies in, for example, MS patients.

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Rheumatoid pachymeningitis is a rare complication of rheumatoid arthritis. This disease was confined to the dura and pia-arachnoid of the lumbar cord in our patient. Her neurologic deficits responded to surgical decompression and corticosteroid therapy. Radiologic evidence and the differences in cell count, protein, and glucose content between lumbar and cisternal cerebrospinal fluid indicate that rheumatoid pachymeningitis can be localized to a discrete region of the central nervous system. Elevated immunoglobulins, IgM and IgG rheumatoid factors, low molecular weight IgM, and immune complexes were found in the cerebrospinal fluid and implicate an immune reaction in the pathogenesis of this disease, which is probably similar to inflammatory processes involving other organs in rheumatoid arthritis.

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Paired samples of serum and concentrated cerebrospinal fluid (CSF) from 53 patients with multiple sclerosis (MS) and from 36 patients with other neurological diseases were adjusted to an equal concentration of IgG and examined for occurrence of auto-antibodies. Anti-nuclear antibodies, anti-mitochondrial antibodies, and rheumatoid factor were not detected with increased frequency in the materials studied. Smooth-muscle antibody (SMA) was, however, detected in CSF and serum from three patients with MS and one patient with chronic lymphocytic meningoencephalitis, and in the CSF only from one additional patient with MS. The SMA activities of the CSF were 4 to 16 times higher than in the matching sera, suggesting a local SMA synthesis within the central nervous system. The SMA was associated with electrophoretically restricted fractions of IgG, but an association between SMA and oligoclonal IgG of the CSF could not be demonstrated in the SMA-positive patients by the methods used.

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