TY - JOUR
T1 - Gangliosides act as onconeural antigens in paraneoplastic neuropathies
AU - De Toni, Luca
AU - Marconi, Silvia
AU - Nardelli, Ettore
AU - Alberti, Daniela
AU - Borsellino, Giovanna
AU - Fracasso, Giulio
AU - Bach, Simona
AU - Bertolasi, Laura
AU - Santo, Antonio
AU - Bassi, Antonella
AU - Tramonti, Daniela
AU - Battistini, Luca
AU - Bonetti, Bruno
PY - 2004/11
Y1 - 2004/11
N2 - We describe two patients with progressive neuropathy and lung cancer in whom gangliosides (GS) may represent the oncoantigens. Patient 1 had motor neuropathy, high titers of IgG 1 and IgG 3 to GD1a and GM1, and expansion of circulating γ-δ T lymphocytes, a T-cell subset responding to glycolipids. Patient 2 presented with Miller-Fisher-like syndrome and IgG 3 activity to disialo-GS. In both cases, decreased autoimmune responses and stabilization of neuropathy were accomplished by tumor treatment. By immunohistochemistry, patient 1's IgG bound to his own tumor and to structures of normal nervous system expressing GD1a or GM1. Infiltration of IgG in the same neural structures was found at his autopsy. Regarding cellular immunity, the proportion of γ-δ T lymphocytes infiltrating carcinoma from patient 1 was significantly higher than in neoplastic controls. These results indicate that GS may represent onconeural antigens in paraneoplastic neuropathy (PNN); their expression on neoplastic tissue may elicit autoimmune responses, which also target neural structures.
AB - We describe two patients with progressive neuropathy and lung cancer in whom gangliosides (GS) may represent the oncoantigens. Patient 1 had motor neuropathy, high titers of IgG 1 and IgG 3 to GD1a and GM1, and expansion of circulating γ-δ T lymphocytes, a T-cell subset responding to glycolipids. Patient 2 presented with Miller-Fisher-like syndrome and IgG 3 activity to disialo-GS. In both cases, decreased autoimmune responses and stabilization of neuropathy were accomplished by tumor treatment. By immunohistochemistry, patient 1's IgG bound to his own tumor and to structures of normal nervous system expressing GD1a or GM1. Infiltration of IgG in the same neural structures was found at his autopsy. Regarding cellular immunity, the proportion of γ-δ T lymphocytes infiltrating carcinoma from patient 1 was significantly higher than in neoplastic controls. These results indicate that GS may represent onconeural antigens in paraneoplastic neuropathy (PNN); their expression on neoplastic tissue may elicit autoimmune responses, which also target neural structures.
KW - γ-δ T lymphocytes
KW - Autoantibodies
KW - Gangliosides
KW - Onconeural antigens
KW - Peripheral neuropathy
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U2 - 10.1016/j.jneuroim.2004.07.020
DO - 10.1016/j.jneuroim.2004.07.020
M3 - Article
C2 - 15465609
AN - SCOPUS:4644263291
VL - 156
SP - 178
EP - 187
JO - Journal of Neuroimmunology
JF - Journal of Neuroimmunology
SN - 0165-5728
IS - 1-2
ER -