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Abnormal serum factors in Guillain-Barré syndrome

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Abstract

The Guillain-Barré Syndrome (GBS) is generally considered to be a cell-mediated immunopathologic disease of the peripheral nervous system (PNS), although the evidence for this is indirect. Both in vitro and in vivo studies of sera from experimental animals with autoimmune demyelinating neuropathies suggest that serum factors, including antibodies to PNS myelin and/or Schwann cells, may be important in the pathogenesis of some of these disorders. More recently, similar in vitro and in vivo techniques, including the production of demyelination following intraneural injection in the rat have been employed to study sera from patients with GBS. The results of these studies demonstrate the presence of factor(s), as yet not fully characterized, that may be important in mediating demyelination. Moreover, in some patients with chronic or relapsing demyelinative inflammatory neuropathies and monoclonal gammopathy, there is evidence of antimyelin antibodies to PNS myelin. Further studies of serum from patients with acute GBS and these other neuropathies may clarify the role of serum factors in acquired inflammatory diseases of the PNS.

Sommario

La sindrome di Guillain-Barré è generalmente considerata come una malattia del sistema nervoso periferico di tipo immunopatologico con mediazione cellulare anche se la prova di questa interpretazione è indiretta. Sia in vitro che in vivo le indagini sui sieri di animali sperimentali con neuropatie autoimmuni demielinizzanti dimostrano che le componenti del siero, inclusi gli anticorpi alla mielina e o alle cellule di Schwann, sono importanti nella patogenesi di alcune malattie di questo tipo. Più recentemente tecniche simili in vitro e in vivo sono state applicate per studiare il siero di pazienti con sindrome di Guillain-Barré e tra queste tecniche in particolare quella della iniezione intraneurale nei topi capace di produrre demielinizzazione. I risultati di questi studi dimostrano la presenza di uno o più fattori per ora non ancora ben caratterizzati, che devono ritenersi importanti nel provocare demielinizzazioni. Inoltre in alcuni pazienti affetti da neuropatie infiammatorie demielinizzanti croniche ricorrenti e in gammopatie monoclonali, si evidenziano anticorpi antimielina del sistema nervoso periferico. Ulteriori studi sul siero di pazienti conforme di Guillain-Barré acuta e di queste altre neuropatie serviranno a chiarire il ruolo dei fattori del siero nelle malattie infiammatorie del sistema nervoso periferico.

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  1. Department of Neurology, School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA

    Lisak R. P., Brown M. J. & Summer A. J.

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Some of the original work cited in this review was supported in part by the United States Public Health Service National Institute of Neurologic and Communicative Diseases and Stroke, the Kroc Foundation, the National Multiple Sclerosis Society (U.S.A.) and the Muscular Dystrophy Association of America.

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Lisak, R.P., Brown, M.J. & Summer, A.J. Abnormal serum factors in Guillain-Barré syndrome. Ital J Neuro Sci 4, 265–272 (1983). https://doi.org/10.1007/BF02043478

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