Peripheral neuropathy associated with monoclonal IgM antibody to glycolipids with a terminal glucuronyl-3-sulfate epitope.

Twenty-nine patients with paraproteinaemia, 12 with neuropathy and 17 without a previous record of neurological symptoms were clinically characterized. All 12 neuropathy patients had a moderate to severe sensorimotor demyelinating neuropathy. The patients were examined with regard to serum antibodies to gangliosides, including GM1, GD1a, GD1b, GT1b, and LM1, and other acidic glycolipids, including LK1 and sulphatide, of human brain and peripheral nerve.

Susceptibility to demyelinating polyneuropathy in plasma cell dyscrasia may be influenced by amino acid position 9 of the HLA-DR beta chain.

Fifty-five patients with plasma cell dyscrasias were investigated by genomic typing for HLA-DR and -DQ genes by restriction fragment length polymorphism, neurophysiology and for presence of anti-myelin-associated glycoprotein (MAG) antibodies. In 26 patients, a polyneuropathy (PN) of demyelinating type was established. Among these individuals, an association was found with the presence of a tryptophan amino acid residue at position 9 of the DR beta chain (P < 0.

Clinical, neurophysiological and immunological evidence of polyneuropathy in patients with monoclonal gammopathies.

In this study we estimated the prevalence of polyneuropathy (PN) in patients with monoclonal gammopathies. 31 patients with monoclonal gammopathies (19 with monoclonal gammopathy of uncertain significance (MGUS), 10 with multiple myeloma (MM), and 2 with Waldenström's macroglobulinemia), were studied by clinical and neurophysiological examination, blood tests to exclude other causes of PN, ELISA assays to detect antibodies to peripheral nerve myelin (PNM), and antibodies to myelin associated glycoprotein (MAG). 11 of 31 patients (36%) had a clinical PN, 3 (10%) had a probable PN (signs but no symptoms), and 4 (13%) had a subclinical PN (only neurophysiological signs of PN).

Ig-secreting cells pass the blood-brain barrier: studies on kappa and lambda light chain secreting cells in plasma cell dyscrasia.

To study if immunoglobulin (Ig)-secreting cells actively pass the blood-brain barrier (BBB), 15 patients with monoclonal gammopathy underwent bonemarrow (BM) iliac crest aspiration biopsy, peripheral blood (PB) sampling and cerebrospinal fluid (CSF) analysis. With an enzyme-linked immunospot assay we investigated the number and ratio of mononuclear cells secreting Ig with kappa and lambda light chains in the three different compartments. A statistically significant (P < 0.

Autoantibodies versus clinical symptoms in blood donors.

A group of 255 blood donors was analyzed for the presence of serum autoantibodies, e.g., antinuclear antibodies, smooth muscle antibodies, antimitochondrial antibodies, antiparietal cell antibodies and antireticulin antibodies by indirect immunofluorescence microscopy.

Immunochemical and clinical effects of immunosuppressive treatment in monoclonal IgM neuropathy.

A pathogenic role of the M protein in monoclonal IgM neuropathy has been suggested. This is based among other things on a close relation between immunosuppressive treatment, lowered concentration of M protein, and clinical effect. We studied five patients with monoclonal IgM and antibodies to peripheral nerve myelin.

Comparison of seven formulae and isoelectrofocusing for determination of intrathecally produced IgG in neurological diseases.

Seven different formulae and agarose isoelectrofocusing (AIF) using immunolabelling for IgG were compared for their ability to discriminate between intrathecally produced IgG and transudated IgG in cerebrospinal fluid. All reference limits were set to a specificity of 97.5% (reference group, n = 211).

Cerebral ischemia associated with anticardiolipin antibodies.

Eight patients, 3 with systemic lupus erythematosus (SLE) or "SLE-like" disease, 1 with sarcoidosis, and 4 with no connective tissue disease had transient ischemic attacks (TIA) or cerebral infarctions associated with high levels of anticardiolipin antibodies (ACA). Cerebral ischemic events included amaurosis fugax, recurrent hemispheric TIA, cerebral infarction, and multi-infarction dementia. Treatment with acetylsalicylic acid was ineffective in 3 patients.

Antibodies to peripheral nerve myelin may occur without clinical neuropathy in healthy persons.

To study the role of autoantibodies against peripheral nerve myelin (PNM), sera from 255 healthy blood donors were investigated by enzyme-linked immunosorbent assay (ELISA), and persons with anti-PNM antibodies were further studied clinically and by neurography, 25 blood donors (10%) had anti-PNM antibodies of IgM, IgG or IgA isotype. The 12 blood donors with anti-PNM antibodies of IgM isotype showed only slightly decreased nerve conduction velocities compared to 12 blood donors without anti-PNM antibodies. Two blood donors with anti-PNM antibodies of IgM and IgG isotype respectively showed a clinical, previously undiagnosed polyneuropathy (PN), verified by neurography.

Lyme arthritis: oligoclonal anti-Borrelia burgdorferi IgG antibodies occur in joint fluid and serum.

The antibody response to Borrelia (B.) burgdorferi, and to measles virus as control antigen, was analysed by agarose isoelectric focusing (AIF) and immunoblot of joint fluid and serum from 10 patients with Lyme arthritis and 10 controls with rheumatoid arthritis. Among the Lyme arthritis patients, six had oligoclonal anti-B.

Acute peripheral facial palsy: CSF findings and etiology.

CSF and serum were examined in acute and convalescence phase from 56 patients with acute idiopathic peripheral facial palsy. CSF protein analysis, viral and borrelia serology were performed. Borrelia infection was found in 9/56 cases and was often associated with inflammatory CSF findings.

Antibodies to myelin-associated glycoprotein are found in cerebrospinal fluid in polyneuropathy associated with monoclonal serum IgM.

Antibodies to myelin-associated glycoprotein (MAG) have been demonstrated in the serum samples from about half the patients with polyneuropathy associated with serum IgM monoclonal component. We examined cerebrospinal fluid (CSF) and serum samples from 13 patients with this disease by enzyme-linked immunosorbent assay for anti-MAG IgM antibodies. We detected these antibodies in both CSF and serum samples in 10 of the patients; in three of them the antibodies were at higher levels in the CSF.

Correlation between dynamic posturography, clinical investigation, and neurography in patients with polyneuropathy.

Dynamic posturography (PG) is a new objective method to study functional performance in diseases affecting balance. It measures muscle response latencies and sway angles to standardized alterations of a moveable platform and moveable surroundings. Twenty-eight patients with polyneuropathy (PN) were studied by clinical investigation, vibrametry, neurography, and dynamic PG.

Absence of autoantibodies in primary fibromyalgia.

Sera from patients with primary fibromyalgia (223 sera, 210 women; 13 men) were analyzed, by immunofluorescence microscopy, for the presence of antibodies directed against cell nuclei (ANA), smooth muscle, mitochondria and other tissue antigens present in cryostat sections of rat organs (liver, kidney and stomach). Sera from blood donors (255 sera, 75 women; 180 men) served as a comparative group. The occurrence of serum autoantibodies in patients with fibromyalgia did not differ significantly from the reference group.

Immunoglobulins within the central nervous system in primary Sjögren's syndrome.

Cerebrospinal fluid (CSF) and sera from 17 patients with primary Sjögren's syndrome (PSS) with or without clinical evidence of nervous system involvement were studied. Intrathecal IgG synthesis as measured by oligoclonal IgG bands on agarose isoelectric focusing or elevated IgG index in CSF was found in 6 of 8 patients with clinical nervous system involvement but also in 5 of 9 patients without clinical nervous system involvement. Elevated IgM-index in CSF was found in 7 of 8 patients with clinical nervous system involvement and in 6 of 9 patients without clinical nervous system involvement.

No increased relapse frequency in acute Guillain-Barré syndrome after plasma exchange.

Relapse frequency in 23 patients with acute Guillain-Barré syndrome (GBS) treated with plasma exchange (PE) was studied. Fifteen patients showed clear improvement, and 4 patients some improvement during the PE period, whereas 4 patients did not improve in immediate connection to the PE treatment. During the follow up time of 6-96 months (mean 36 months), only one of the 23 patients had a relapse, which appeared after 3 months.

Neuropathy and myopathy in primary Sjögren's syndrome: neurophysiological, immunological and muscle biopsy results.

Seventeen consecutive patients with primary Sjögren's syndrome (PSS) received neurophysiological examination, analysis of antibodies against peripheral nerve-myelin (PNM) and muscle biopsy, to show the prevalence and characteristics of peripheral neuropathy (PN) and myopathy; 3 PSS cases showed a clinical mild sensorimotor polyneuropathy, 1 of them had been treated with cytostatic drugs; 1 had mononeuropathia multiplex; and 1 clinical carpal tunnel syndrome. In these 5 patients neurophysiological investigation verified the clinical diagnosis of peripheral nerve involvement; 2 with PN had serum-antibodies against PNM; 1 of IgM-, and 1 of IgA-isotype. Muscle biopsies were taken from 15 PSS patients; 11 showed inflammatory myositis or inflammatory perivascular infiltrates and 3 showed signs of denervation.

Mononuclear cell types in cerebrospinal fluid and blood of patients with multiple sclerosis. Quantitation by immunoenzyme microassay with panel of monoclonal antibodies.

Phenotypic distribution of mononuclear cells in cerebrospinal fluid (CSF) and peripheral blood from patients with multiple sclerosis (MS) and, for reference, patients with acute aseptic meningoencephalitis (AM), and in blood only from healthy controls, was studied with an immunoenzymatic microassay enabling analysis even in the presence of a normal CSF cell count. In MS, increased CD5+ (pan-T) cell proportion in CSF compared with blood was not reflected by changes of CD4+ or CD8+ cells, while in AM, an increase of CD4+ cells was registered. Therefore, a population of CD5+, CD4-, and CD8- cells may be anticipated to exist in CSF of patients with MS.

Leukocyte types in cerebrospinal fluid and peripheral blood enumerated immunoenzymatically in aseptic meningitis and the Guillain-Barré syndrome.

To study celltype distribution simultaneously in peripheral blood (PB) and cerebrospinal fluid (CSF) from patients with aseptic meningitis (AM) (n = 14) and Guillain-Barré syndrome (GBS) (n = 9) we used an immunoenzymatic method that enabled the use of several monoclonal antibodies, also in CSF samples with normal cellcounts. In both patient groups a different cell-distribution in CSF compared to PB was found with regard to pan T cells (CD5+/anti-Leu1+), T cell subpopulations (CD4+/anti-Leu3+, CD8+/anti-Leu2+), B cells (OKB2+, OKB7+), monocytes/macrophages (CD11+/OKM1+) and HLA/DR expressing cells, whereas the distribution of HLA/DC+ cells was similar in CSF and PB. Thus, the CSF cell distribution does not reflect the distribution in PB.

IgM monoclonal gammopathy and neuropathy in two siblings.

A sister and a brother with a progressive mixed axonal and demyelinating polyneuropathy were found to have a monoclonal IgM gammopathy of kappa and lambda type, respectively. Sural nerve and cutaneous nerve specimens obtained by biopsy showed deposits of IgM on myelin sheets. Sera from both patients contained antibodies directed to bovine peripheral nerve myelin as determined by ELISA technique and to normal human peripheral nerve myelin as demonstrated by indirect immunofluorescence histochemistry.

Occurrence and isotype of antibodies against peripheral nerve myelin in serum from patients with peripheral neuropathy and healthy controls.

Antibodies against peripheral nerve myelin have previously been demonstrated in serum from patients with peripheral neuropathy and IgM paraproteinaemia, and a causal relationship has been suggested. Using enzyme-linked immunosorbent assay (ELISA), anti-myelin antibodies were found in sera from eight of 16 patients with polyneuropathy and paraproteinaemia, but also in 17% of 109 patients with peripheral neuropathy lacking monoclonal immunoglobulin, including five of 10 patients with Charcot-Marie-Tooth disease, and in 16% of 142 blood donors. The antibodies were mostly of IgM class in the two neuropathy groups, while blood donors had mostly IgA antibodies, and a few subjects of each group had antimyelin antibodies of two different isotypes.

Cerebrospinal fluid immunoglobulins and beta 2-microglobulin in lymphoproliferative and other neoplastic diseases of the central nervous system.

Humoral immune aberrations may occur in the cerebrospinal fluid (CSF) of patients with lymphoproliferative and other neoplastic diseases infiltrating the central nervous system (CNS). Such aberrations may be of diagnostic importance. We therefore studied CSF and serum from 47 patients with lymphoproliferative diseases and from 16 patients with various nonlymphoid neoplasias; 17 patients and 12 patients, respectively, had neoplastic CNS involvement.