A prospective study of acute idiopathic neuropathy. III. Immunological studies.

Abstract

SUMMARYThe immune responses of 100 patients who presented with an acute idiopathic neuropathy were compared with those of age and sex matched controls. Blood lymphocytes and their subsets were counted with a fluorescent activated cell sorter. CD8 + (putative suppressor) lymphocytes were significantly reduced in the first week of the disease but total lymphocytes, total T and CD4 + (putative helper) cells were not altered. This reduction depended on the nature of the preceding infection. Serum complement C3 and C4 concentrations remained normal and immune complexes were rarely detected with a Clq binding assay. Complement-fixing antibodies to human peripheral nerve antigens were discovered in the serum of 7% of patients but only 1% of controls. Complement-fixing antibodies to galactocerebroside were not discovered in any sera. Enzymelinked immunoassays detected increased antibody responses to galactocerebroside but none at all to human P2 myelin protein in the patient sera. Forty u1 of serum from five patients injected into the sciatic nerves of rats did not induce significantly more demyelination than the serum from control patients. It is concluded that auto-immune responses can only be detected by these techniques in a small minority of patients with acute idiopathic neuropathy.The close histological resemblance between experimental allergic neuritis (EAN) and the Guillain-Barre syndrome (GBS) suggests a common auto-immune pathogenesis. The major antigen responsible for inducing EAN is a basic myelin protein, P2, MW 15000 D, which is located predominantly in the myelin of the peripheral rather than central nervous system.1-3 EAN can be transferred with lymph node cells"4 or a CD4+ T cell line.5 Cell-mediated immune mechanisms are important in pathogenesis because the development of EAN is inhibited by procedures which deplete T cells6 but antibodies to P2 can readily be detected in the blood of rats and rabbits with EAN.34 Of other myelin components which might induce an auto-immune response the most studied is galactocerebroside; rabbits immunised with this glycolipid develop a demyelinating neuropathy, lacking the perivascular lymphocyte infiltration of EAN and GBS, probably owing to complement-fixing antibodies directed against

Lymphocyte Subset Proportions in Guillain-Barré Syndrome Patients Treated with Plasmapheresis

“…The reduction in CD19+ cells after PP may be associated with the decrease of immunoglobulins or circulating autoantibodies. Although these findings are not consistent with the immunofluorescence microscopic data of Hughes et al [5], Winer et al [7], or Lisak et al [6], or with recent flow-cytometric studies by Dahle et al [8]and Sindern et al [9], we found some relationships between clinical effects and the changes in lymphocyte subsets. That is, the changes in CD19+ and CD3+ subsets were rapid in the group of improvers.…”

“…Several investigators have studied the proportions of circulating T cell subsets in the peripheral blood of GBS patients [5, 6, 7, 8, 9]. They found deviations from the normal pattern, although the results were inconsistent.…”

T cell reactivity to P0, P2, PMP-22, and myelin basic protein in patients with Guillain-Barre syndrome and chronic inflammatory demyelinating polyradiculoneuropathy

“…No significant differences in proliferative reactivity to either P2 [14][15][16][17][18][19][20][21][22][23][24][25] or P2 61-70 were seen between patients with GBS or CIDP, and healthy controls or patients with ON. When GBS patient reactivity to either P2 [14][15][16][17][18][19][20][21][22][23][24][25] or P2 61-70 (19%) was compared with the reactivity of healthy controls and patients with ON grouped collectively (9%), significance was reached (p = 0.029).…”

“…When GBS patient reactivity to either P2 [14][15][16][17][18][19][20][21][22][23][24][25] or P2 61-70 (19%) was compared with the reactivity of healthy controls and patients with ON grouped collectively (9%), significance was reached (p = 0.029). Similarly, reactivity of patients with CIDP to either of these peptides (20.7%) was significantly greater than that of healthy controls and patients with ON grouped collectively (p = 0.018).…”

Guillain-Barré Syndrome in the Elderly

“…Another difference in laboratory findings was the increased eleva tion of immunoglobulin G in the old. The interpretation of this is difficult, in view of the changes in immunological function associ ated with ageing [26,27], The duration of hos pital stay was greater in the elderly though, contrary to what has been asserted [23,28], older patients did not have a significantly [16] 1955-1961 3 0.6 (0.1-1.5) Guam [17] 1960-1966 5 1.9 (0.6-3.9) Olmsted County [18] 1935-1968 29 1.6(1.2-2.2) Israel [19] 1969-1972 89 0.8 (0.6-0.9) San Joaquin. Calif. [20] 1972 worse prognosis as regards death or perma nent disability.…”