Severe axonal degeneration in acute Guillain-Barré syndrome: Evidence of two different mechanisms?

doi:10.1016/0022-510X(93)90324-R

Journal of the Neurological Sciences

Volume 116, Issue 2, June 1993, Pages 185-192

https://doi.org/10.1016/0022-510X(93)90324-R Get rights and content

Abstract

Four cases of severe acute Guillain-Barré syndrome (GBS) characterized by severe axonal degeneration are presented. All had electrically inexcitable motor nerves as early as 4 days after onset. The disease was rapid in onset and the residual disability was severe. Two different types of pathology were seen. Nerve biopsies in 3 cases showed severe axonal degeneration without inflammation or demyelination. Autopsy in one of these cases showed that the dorsal and ventral roots were also significantly affected. These cases illustrate the primary axonal form of GBS. Nerve biopsy in the fourth case at day 15 showed marked inflammation and demyelination with axonal degeneration. Contralateral nerve biopsy at day 75 showed almost complete loss of axons. This case illustrates another type of axonal degeneration, that which occurs secondary to inflammation and demyelination.

References (33)

T.E. Feasby
Inflammatory-demyelinating polyneuropathies
T.E. Feasby et al.
Complement depletion suppresses Lewis rat experimental allergic neuritis
Brain Res.
(1987)
E. Hall et al.
Preservation of motor nerve function during early degeneration by the 21-aminosteroid anti-oxidant U74006F
Brain Res.
(1990)
A.K. Asbury
Diagnostic consideration in Guillain-Barré syndrome
Ann. Neurol.
(1981)
A.K. Asbury et al.
The inflammatory lesion in idiopathic polyneuritis: its role in pathogenesis
Medicine (Balt.)
(1969)
B. Babior
Oxygen-dependent microbial killing by phagocytes
New Engl. J. Med.
(1978)
W.F. Brown et al.
Conduction block and denervation in Guillain-Barré polyneuropathy
Brain
(1984)
W.F. Brown et al.
Electrophysiological changes in the acute axonal form of Guillain-Barré syndrome
Muscle Nerve
(1993)
V. Chaudhry et al.
Wallerian degeneration in human nerves: serial electrophysiological studies
Muscle Nerve
(1992)
D. Cornblath et al.
Motor conduction studies in Guillain-Barre syndrome: description and prognostic value
Ann. Neurol.
(1988)

D. Currie et al.

Guillain-Barré syndrome in children: evidence of axonal degeneration and long-term follow-up

Arch. Phys. Med. Rehab.

(1990)

C. Dinarello et al.

Lymphokines

New Engl. J. Med.

(1987)

P.J. Dyck

Pathologic alterations of the peripheral nervous system of man

T.E. Feasby et al.

An acute axonal form of Guillain-Barré polyneuropathy

Brain

(1986)

R. Gilliatt et al.

Nerve conduction during wallerian degeneration in the baboon

J. Neurol. Neurosurg. Psychiat.

(1972)

J. Griffin et al.

Macrophage responses in inflammatory demyelinating neuropathies

Ann. Neurol.

(1990)

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With respect to timing of electrodiagnostic studies in evaluating axonal injury, the previously mentioned phenomena are of clinical relevance. In severe acute axonal injury, nerve conduction in segments distal from the axonal injury will remain intact for up to several days, referable to the latent phase of Wallerian degeneration (van der Meche et al., 1991; Feasby et al., 1993; Capasso et al., 2003; Uncini and Kuwabara, 2012). Within days, SNAP and CMAP amplitudes diminish in a retrograde or “dying-back” pattern.
Axonal neuropathies encompass a wide range of acquired and inherited disorders with electrophysiologic characteristics that arise from the unique neurophysiology of the axon. Accurate interpretation of nerve conduction studies and electromyography requires an in-depth understanding of the pathophysiology of the axon. Here we review the unique neurophysiologic properties of the axon and how they relate to clinical electrodiagnostic features. We review the length-dependent Wallerian or “dying-back” processes as well as the emerging body of literature from acquired axonal neuropathies that highlights the importance of axonal disease at the nodes of Ranvier. Neurophysiologic features of individual inherited and acquired axonal diseases, including primary nerve disease as well as systemic immune mediated, metabolic, and toxic diseases involving the peripheral nerve, are reviewed. This comprehensive review of electrodiagnostic findings coupled with the current understanding of pathophysiology will aid the clinician in the evaluation of axonal polyneuropathies.
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The advance in understanding that changed this viewpoint was the appreciation that distinct, clinical-pathological phenotypes could be delineated within the Guillain-Barré syndrome spectrum, the main phenotypes of which are termed acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy (figure 2). Although this distinction of Guillain-Barré syndrome phenotypes does not negate the idea of bystander axonal injury, it does clarify the point that axons themselves can be the primary target for autoimmune injury, rather than being injured as a secondary phenomenon.37 Clinical variants such as Miller Fisher syndrome are now classified within the Guillain-Barré syndrome family of disorders.
Guillain-Barré syndrome is the most common and most severe acute paralytic neuropathy, with about 100 000 people developing the disorder every year worldwide. Under the umbrella term of Guillain-Barré syndrome are several recognisable variants with distinct clinical and pathological features. The severe, generalised manifestation of Guillain-Barré syndrome with respiratory failure affects 20–30% of cases. Treatment with intravenous immunoglobulin or plasma exchange is the optimal management approach, alongside supportive care. Understanding of the infectious triggers and immunological and pathological mechanisms has advanced substantially in the past 10 years, and is guiding clinical trials investigating new treatments. Investigators of large, worldwide, collaborative studies of the spectrum of Guillain-Barré syndrome are accruing data for clinical and biological databases to inform the development of outcome predictors and disease biomarkers. Such studies are transforming the clinical and scientific landscape of acute autoimmune neuropathies.
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To assess the longitudinal changes of nerve ultrasound in Guillain–Barré syndrome (GBS) patients.
We prospectively recruited 17 GBS patients and 17 age and gender-matched controls. Serial studies of their nerve conduction parameters and nerve ultrasound, documenting the cross-sectional areas (CSA), were performed at admission and repeated at several time points throughout disease course.
Serial nerve ultrasound revealed significantly enlarged CSA in median, ulnar and sural nerves within the first 3 weeks of disease onset. Longitudinal evaluation revealed an improvement in the nerve CSA with time, reaching significance in the ulnar and sural nerves after 12 weeks. There was no significant difference between the demyelinating and axonal subtypes. There was also no significant correlation found between nerve CSA and neurophysiological parameters or changes in nerve CSA and muscle strength.
In GBS, serial studies of peripheral nerve ultrasound CSA are helpful to detect a gradual improvement in the nerve size.
Serial nerve ultrasound studies could serve as a useful tool in demonstrating nerve recovery in GBS.
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Autoimmune neuropathies occur when immunologic tolerance to myelin or axonal antigens is lost. Even though the triggering factors and the underling immunopathology have not been fully elucidated in all neuropathy subsets, immunological studies on the patients' nerves, transfer experiments with the patients' serum or intraneural injections, and molecular fingerprinting on circulating autoantibodies or autoreactive T cells, indicate that cellular and humoral factors, either independently or in concert with each other, play a fundamental role in their cause. The review is focused on the main subtypes of autoimmune neuropathies, mainly the Guillain–Barré syndrome(s), the Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), the Multifocal Motor Neuropathy (MMN), and the IgM anti-MAG-antibody mediated neuropathy. It addresses the factors associated with breaking tolerance, examines the T cell activation process including co-stimulatory molecules and key cytokines, and discusses the role of antibodies against peripheral nerve glycolipids or glycoproteins. Special attention is given to the newly identified proteins in the nodal, paranodal and juxtaparanodal regions as potential antigenic targets that could best explain conduction failure and rapid recovery. New biological agents against T cells, cytokines, B cells, transmigration and transduction molecules involved in their immunopathologic network, are discussed as future therapeutic options in difficult cases. This article is part of a Special Issue entitled: Neuromuscular Diseases: Pathology and Molecular Pathogenesis.
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A retrospective analysis was performed to document the clinical and electrophysiological features of Guillain-Barré syndrome (GBS) subtypes using different diagnostic criteria.
One hundred GBS patients were included. Clinical and laboratory features were analyzed, and patients were classified according to four sets of diagnostic criteria. Electrodiagnostic criteria were also analyzed.
A total of 69 patients met Asbury and Cornblath's criteria, 96 met Van der Meché’s criteria, 99 met Wakerley's diagnostic classification and 86 met level 1 or 2 of the Brighton criteria. Rates of GBS subtypes were: 69% classic GBS; 8% Miller–Fisher syndrome; 12% paraparetic GBS; 2% pharyngeal–cervical–brachial GBS; and 9% unclassified. Those for electrodiagnostic subtypes were 52% demyelinating and 9% axonal according to Hadden's criteria vs 41% demyelinating and 41% axonal as per Rajabally's criteria.
In this study of case distribution within the GBS spectrum of a retrospective cohort of French patients, the application of new diagnostic criteria enabled accurate diagnoses and classifications of the different subtypes, and also increased the recognition of axonal GBS.
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Research articleSevere axonal degeneration in acute Guillain-Barré syndrome: Evidence of two different mechanisms?

Abstract

Brain Res.

Brain Res.

Diagnostic consideration in Guillain-Barré syndrome

Ann. Neurol.

The inflammatory lesion in idiopathic polyneuritis: its role in pathogenesis

Medicine (Balt.)

Oxygen-dependent microbial killing by phagocytes

New Engl. J. Med.

Conduction block and denervation in Guillain-Barré polyneuropathy

Brain

Electrophysiological changes in the acute axonal form of Guillain-Barré syndrome

Muscle Nerve

Wallerian degeneration in human nerves: serial electrophysiological studies

Muscle Nerve

Motor conduction studies in Guillain-Barre syndrome: description and prognostic value

Ann. Neurol.

Guillain-Barré syndrome in children: evidence of axonal degeneration and long-term follow-up

Arch. Phys. Med. Rehab.

Lymphokines

New Engl. J. Med.

Pathologic alterations of the peripheral nervous system of man

An acute axonal form of Guillain-Barré polyneuropathy

Brain

Nerve conduction during wallerian degeneration in the baboon

J. Neurol. Neurosurg. Psychiat.

Macrophage responses in inflammatory demyelinating neuropathies

Ann. Neurol.

Research article
Severe axonal degeneration in acute Guillain-Barré syndrome: Evidence of two different mechanisms?