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Proximal nerve lesions in early Guillain–Barré syndrome: implications for pathogenesis and disease classification

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Abstract

Guillain–Barré syndrome (GBS) is an acute-onset, immune-mediated disorder of the peripheral nervous system. In early GBS, arbitrarily established up to 10 days of disease onset, patients could exhibit selective manifestations due to involvement of the proximal nerves, including nerve roots, spinal nerves and plexuses. Such manifestations are proximal weakness, inaugural nerve trunk pain, and atypical electrophysiological patterns, which may lead to delayed diagnosis. The aim of this paper was to analyze the nosology of early GBS reviewing electrophysiological, autopsy and imaging studies, both in acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor/motor-sensory axonal neuropathy (AMAN/AMSAN). Early electrophysiology showed either well-defined demyelinating or axonal patterns, or a non-diagnostic pattern with abnormal late responses; there may be attenuated M responses upon lumbar root stimulation as the only finding. Pathological changes predominated in proximal nerves, in some studies, most prominent at the sides where the spinal roots unite to form the spinal nerves; on very early GBS endoneurial inflammatory edema was the outstanding feature. In the far majority of cases, spinal magnetic resonance imaging showed contrast enhancement of cauda equina, selectively involving anterior roots in AMAN. Both in AIDP and AMAN/AMSAN, ultrasonography has demonstrated frequent enlargement of ventral rami of C5–C7 nerves with blurred boundaries, whereas sonograms of upper and lower extremity peripheral nerves exhibited variable and less frequent abnormalities. We provide new insights into the pathogenesis and classification of early GBS.

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Acknowledgments

We thank Mrs. Marta de la Fuente for secretarial help. This study was Supported by CIBERNED and IDIVAL.

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Authors and Affiliations

  1. Service of Neurology, University Hospital “Marqués de Valdecilla (IDIVAL)”, University of Cantabria, “Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)”, 39008, Santander, Spain

    José Berciano, María J. Sedano & Ana L. Pelayo-Negro

  2. Service of Clinical Neurophysiology, University Hospital “Marqués de Valdecilla (IDIVAL)”, University of Cantabria, “Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)”, 39008, Santander, Spain

    Antonio García & Pedro Orizaola

  3. Service of Radiology, University Hospital “Marqués de Valdecilla (IDIVAL)”, University of Cantabria, “Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)”, 39008, Santander, Spain

    Elena Gallardo

  4. Department of Anatomy and Cell Biology, University of Cantabria, IDIVAL, “Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)”, 39008, Santander, Spain

    Miguel Lafarga & María T. Berciano

  5. Department of Neurology and Immunology, Erasmus MC University Medical Centre Rotterdam, PO Box 2040, 3000 CA, Rotterdam, Netherlands

    Bart C. Jacobs

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  1. José Berciano
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Berciano, J., Sedano, M.J., Pelayo-Negro, A.L. et al. Proximal nerve lesions in early Guillain–Barré syndrome: implications for pathogenesis and disease classification. J Neurol 264, 221–236 (2017). https://doi.org/10.1007/s00415-016-8204-2

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