Proximal nerve lesions in early Guillain-Barré syndrome: implications for pathogenesis and disease classification.
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.
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. joseberciano51@hotmail.com.
José
J
Berciano
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.
María J
MJ
Sedano
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.
Ana L
AL
Pelayo-Negro
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
A
García
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.
Pedro
P
Orizaola
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
E
Gallardo
Department of Anatomy and Cell Biology, University of Cantabria, IDIVAL, "Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)", 39008, Santander, Spain.
Miguel
M
Lafarga
Department of Anatomy and Cell Biology, University of Cantabria, IDIVAL, "Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)", 39008, Santander, Spain.
María T
MT
Berciano
Department of Neurology and Immunology, Erasmus MC University Medical Centre Rotterdam, PO Box 2040, 3000 CA, Rotterdam, Netherlands.
Bart C
BC
Jacobs
D000818:Animals; D020275:Guillain-Barre Syndrome / Q000145:classification* / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D010525:Peripheral Nerves / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*
Acute motor axonal neuropathy; Acute motor-sensory axonal neuropathy; Axonal degeneration; Demyelination; Early Guillain–Barré syndrome; Electrophysiology; Endoneurial fluid pressure; Endoneurial inflammatory edema; Experimental allergic neuritis; Magnetic resonance imaging; Nerve trunk pain; Spinal nerve; Spinal roots; Ultrasonography