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 Detalle_Publicacion

Proximal nerve lesions in early Guillain?Barre´ syndrome: implications for pathogenesis and disease classification

Abstract: Guillain?Barre´ syndrome (GBS) is an acuteonset, 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 nondiagnostic 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.

 Fuente: J Neurol. 2017 Feb;264(2):221-236

Editorial: Springer

 Fecha de publicación: 01/02/2017

Nº de páginas: 16

Tipo de publicación: Artículo de Revista

 DOI: 10.1007/s00415-016-8204-2

ISSN: 0340-5354,1432-1459

Autores/as

JOSE ANGEL BERCIANO BLANCO

MARIA JOSE SEDANO TOUS

ANA LARA PELAYO NEGRO

GARCÍA, ANTONIO

MIGUEL ANGEL LAFARGA COSCOJUELA

MARIA TERESA BERCIANO BLANCO

JACOBS, BART C.