Severe Guillain‐Barré syndrome: sorting out the pathological hallmark in an electrophysiological axonal case

Abstract

We describe a clinicopathological study of a patient presenting with severe and electrophysiological axonal Guillain-Barré syndrome (GBS). An 83-year-old man had a 2-day history of distal acroparesthesias and ascending weakness culminating in quadriplegia, the patient dying 1 month after onset. On day 3, motor conduction velocity (MCV) and distal motor latency values were normal or minimally delayed; most F waves were present with latencies normal or barely delayed. Compound muscle action potential (CMAP) amplitudes were variably reduced. On day 10, there was reduction of CMAPs with relative preservation of MCV. On histological study, the density of myelinated fibers was normal in L5 ventral and dorsal roots, where outstanding lesions included dark fibers, scattered macrophage infiltration, and occasional images of de-remyelination or axonal degeneration. In the fifth spinal nerve, there was widespread loss of myelinated fibers with focal areas showing almost complete fiber loss and variable fascicular combination of extensive de-remyelination and axonal degeneration. Wallerian-like degeneration predominated in femoral and sciatic nerves. Peripheral neuron cell bodies showed central chromatolysis. We conclude that the pathological hallmark of this electrophysiological axonal GBS case is extensive but variable de-remyelination of proximal nerve trunks with superimposed nerve ischemia and axonal degeneration.

Very early electrodiagnostic findings in Guillain‐Barré syndrome

“…Electrophysiological diagnosis of GBS in early stages may often be difficult because electrodiagnostic findings may be inconclusive and not necessarily suggestive of demyelination unless combined, so multiple nerves need to be tested ( Albers et al, 1985 ; Alam et al, 1998 ; Gordon and Wilbourn, 2001 ; Vucic et al, 2004 ) . At early stages, pathological hallmarks of AIDP and AMAN may be indistinguishable ( Izumo et al, 1985 ; Ropper and Adelman, 1992 ) , and the observation of serial electrophysiological changes is important to accurately delineate and define the electrophysiological pattern and the GBS subtype ( Hadden et al, 1998 ; Hiraga et al, 2005 ; Berciano et al, 2009 ; Uncini et al, 2010 ) . Here, we report the electrodiagnostic findings of 18 patients at very early stages of the disease.…”

“…CB was significantly more frequent than prolonged latency in F‐waves. The absence of F‐waves may be related with muscle inactivity ( Taniguchi et al, 2008 ) or may occur in both axonal and demyelinating GBS ( Kimura and Bultzer, 1975 ; Ho et al, 1997 ; Kuwabara et al, 2000 ; Vucic et al, 2004 ; Hiraga et al, 2005 ; Berciano et al, 2009 ) . However, the presence of TD, prolonged latency of R1 response of BR, or multiple A‐waves in five of our patients with blocked F‐wave responses support the presence of an underlying demyelinating process.…”

“…This pattern is an atypical finding in any diffuse polyneuropathy, but its presence in association with an appropriate clinical syndrome indicates an acquired demyelinating neuropathy ( Murray and Wade, 1980 ; Albers et al, 1985 ; Kuwabara et al, 2004 ; Al‐Shekhlee et al, 2007 ) . However, a recent study has shown that although the sural nerve is electrophysiologically preserved, it may be pathologically involved ( Berciano et al, 2009 ) . None of our patients had abnormal sural responses but normal upper limb sensory responses, a finding that would be characteristic of most chronic sensory neuropathies ( Albers et al, 1985 ) .…”

Thickening and Contrast Enhancement of Spinal Roots on MR Imaging in Guillain-Barré Syndrome: Thoughts on Pathologic Background: Fig 1.

“…[2][3][4][5] In 1 study from a patient presenting with pure motor GBS, inflammatory demyelination with secondary axonal degeneration was restricted to ventral spinal roots. 2 Figure 1 illustrates typical findings in the L5 ventral root.…”

“…Precise inflammatory demyelination of these nerve trunks may imply an increase of endoneurial fluid nerve pressure potentially causing endoneurial ischemia and wallerian-like degeneration. [3][4][5] The predominance of inflammatory lesions in spinal roots and particularly in the ventral ones is an outstanding feature in GBS and in experimental allergic neuritis. Antigen differences between motor and sensory neurons probably account for greater and selective motor involvement.…”