The European journal of medicine · 1993
Amyotrophic lateral sclerosis an enigmatic disease with B-cellular and anti-retroviral immune responses.
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Westarp M E, Fuchs D, Bartmann P, Hoff-Jörgensen R, Clausen J, Wachter H, Kornhuber H H
Аннотация
OBJECTIVES: The most frequent sporadic adult motor neuron disease, amyotrophic lateral sclerosis, affects more men, follows no epidemiologic pattern, and was long considered a pure spinal cord disorder. It now becomes evident that the disease is characterized by spinal, cerebral and extra-neuromuscular changes including B-cellular responses and ultrastructural skin alterations. Do these parameters identify subgroups or correlate with the male preponderance of the disease?
METHODS: We analyzed age at and site of onset, sex, duration of clinical disease, and human foamy retroviral seroreactivity in 47 consecutive patients with a definite diagnosis of amyotrophic lateral sclerosis. The results were compared with antivisna seroreactivity, immunoglobulin isotypes, circulating immune complexes, neopterin and beta 2-microglobulin as well as skin biopsies in respective subsets of the same 47 patients.
RESULTS: Seroreactivity to recombinant human spuma retrovirus (HSRV) envelope and/or capsid protein was positive in 20/47 amyotrophic lateral sclerosis patients, and 28/30 competed with specific retroviral antibodies on maedi-visna antigen. Anti HSRV-seronegative patients had lower immunoglobulin IgG3 isotype concentrations, while HSRV-gag plus HSRV-env antibody positives demonstrated highest circulating IgG immune complexes. All 11 amyotrophic lateral sclerosis patients partially reacting to recombinant HSRV-env or HSRV-gag antigen were men, and male amyotrophic lateral sclerosis patients tended to have higher total cerebrospinal fluid protein levels. Neopterin and beta 2-microglobulin as markers of a cellular immune activation remained basically normal in serum and cerebrospinal fluid.
CONCLUSION: We suggest a particular B-lymphocytic and retroviral involvement in this enigmatic, relentlessly progressing, at present untreatable and most frequent neurological system degeneration. To our opinion this situation justifies the search for novel anti-retroviral therapeutic strategies.
METHODS: We analyzed age at and site of onset, sex, duration of clinical disease, and human foamy retroviral seroreactivity in 47 consecutive patients with a definite diagnosis of amyotrophic lateral sclerosis. The results were compared with antivisna seroreactivity, immunoglobulin isotypes, circulating immune complexes, neopterin and beta 2-microglobulin as well as skin biopsies in respective subsets of the same 47 patients.
RESULTS: Seroreactivity to recombinant human spuma retrovirus (HSRV) envelope and/or capsid protein was positive in 20/47 amyotrophic lateral sclerosis patients, and 28/30 competed with specific retroviral antibodies on maedi-visna antigen. Anti HSRV-seronegative patients had lower immunoglobulin IgG3 isotype concentrations, while HSRV-gag plus HSRV-env antibody positives demonstrated highest circulating IgG immune complexes. All 11 amyotrophic lateral sclerosis patients partially reacting to recombinant HSRV-env or HSRV-gag antigen were men, and male amyotrophic lateral sclerosis patients tended to have higher total cerebrospinal fluid protein levels. Neopterin and beta 2-microglobulin as markers of a cellular immune activation remained basically normal in serum and cerebrospinal fluid.
CONCLUSION: We suggest a particular B-lymphocytic and retroviral involvement in this enigmatic, relentlessly progressing, at present untreatable and most frequent neurological system degeneration. To our opinion this situation justifies the search for novel anti-retroviral therapeutic strategies.