Acute motor axonal neuropathy (AMAN) …

Francesco Michelassi MD PhD

Peripheral Neuropathies

Updated 05.22.2025
Released 11.27.2003
Expires For CME 05.22.2028

Acute motor axonal neuropathy

Author: Francesco Michelassi MD PhD

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Editor: Louis H Weimer MD

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Acute motor axonal neuropathy

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Introduction

Overview

Acute motor axonal neuropathy is the most frequent axonal variant of Guillain-Barré syndrome and is often used synonymously with the term “axonal Guillain-Barré syndrome.” This review describes clinical and electrodiagnostic features of acute motor axonal neuropathy and compares it with the acute inflammatory demyelinating polyradiculoneuropathy variant of Guillain-Barré syndrome. The pathology and pathogenesis of this disorder are discussed to highlight the theme of molecular mimicry.

Key points

	• Acute motor axonal neuropathy is a variant of Guillain-Barré syndrome (GBS) with predominant motor axon dysfunction or injury, and it is strongly associated with antecedent Campylobacter jejuni infection and the presence of anti-ganglioside antibodies.
	• In North America and Europe, acute motor axonal neuropathy is much less frequent than acute inflammatory demyelinating polyradiculoneuropathy.
	• Electrodiagnostic studies (preferably serial) are required to distinguish axonal variants, including acute motor axonal neuropathy, from demyelinating forms of Guillain-Barré syndrome.
	• Although some patients without significant axon degeneration may recover quickly, acute motor axonal neuropathy in general may be associated with longer time to recovery and higher risk of incomplete recovery compared to the acute inflammatory demyelinating polyradiculoneuropathy variant.
	• Treatment should include early intravenous immunoglobulins or plasmapheresis as well as supportive therapy.
	• Novel therapeutic approaches under investigation include the modulation of neonatal Fc receptors (FcRn) to alleviate neuroinflammation in acute motor axonal neuropathy and other Guillain-Barré syndrome subtypes.

Historical note and terminology

Guillain-Barré syndrome is a pathophysiologically heterogeneous peripheral nerve disorder of autoimmune origin. There are several variants of this condition, and a classification of this syndrome is included in Table 1. Acute motor axonal neuropathy variant of Guillain-Barré syndrome is a paralytic condition presenting with an acute, ascending, and flaccid paralysis. This is distinguished from acute inflammatory demyelinating polyneuropathy primarily by electrophysiological studies. In 1986, Feasby and colleagues reported five cases of Guillain-Barré syndrome with electrically inexcitable motor nerves. Autopsy of one patient showed significant and marked axonal degeneration in the ventral roots and peroneal nerves without demyelination. Feasby and colleagues were the first to suggest a possible variant of Guillain-Barré syndrome characterized by acute axonal neuropathy (41).

In 1981, Baoxun and colleagues reported on 156 patients admitted to a hospital in Beijing, China, for Guillain-Barré syndrome; 68.6% of the patients had onset between July and October (17). In addition, 75.6% were less than 30 years of age, with the majority from rural areas. These observations suggested a seasonal propensity for children to develop Guillain-Barré syndrome in China. McKhann and colleagues used the terminology “Chinese paralytic syndrome” to refer to children and young adults from predominantly rural areas presenting with acute flaccid paralysis in seeming epidemics during summer and fall months.

Electrodiagnostic studies in 22 of 36 patients with Chinese paralytic syndrome showed reduction in compound muscle action potential amplitudes, suggesting axonal abnormalities. There was little to no prolongation of distal motor latencies or slowing of motor nerve conduction velocities to suggest demyelination, except in one patient who also had abnormal sensory studies and was thought to have acute inflammatory demyelinating polyneuropathy (103). McKhann and colleagues later coined the phrase “acute motor axonal neuropathy” instead of Chinese paralytic syndrome (102).

Acute motor-sensory axonal neuropathy was later used by Griffin and colleagues to differentiate cases of Guillain-Barré syndrome with electrodiagnostic features of axonal damage involving both motor and sensory fibers, such as Feasby and colleagues reported in 1986 (50). Although Griffin and colleagues suggested that patients with acute motor-sensory axonal neuropathy may have a more severe disease than acute motor axonal neuropathy, they also recognized similar pathological characteristics of the motor and sensory fibers, suggesting that the disorders may be within the same disease spectrum.

Table 1. Classification of Guillain-Barré syndrome

Paralytic forms
	Demyelinating electrophysiology
		Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
	Axonal electrophysiology
		Acute motor axonal neuropathy (AMAN) Acute motor-sensory axonal neuropathy (AMSAN) AIDP with secondary axonal degeneration
Regional or focal paralytic forms
		Fisher syndrome Oropharyngeal Acute ophthalmoplegia
Non-paralytic forms
		Sensory ataxic variant Acute pandysautonomia

Clinical manifestations

Presentation and course

Acute motor axonal neuropathy presents as an acute, flaccid, symmetrical ascending paralysis with increased cerebrospinal fluid protein, suggestive of Guillain-Barré syndrome. Sensory impairment is minimal, and autonomic involvement less common. Peak severity is reached within 5 to 9 days (41). Weakness may involve dysphagia, dysarthria, and facial diplegia and may progress to flaccid quadriplegia and respiratory failure. Extraocular muscle involvement rarely occurs (102). Focal motor involvement may occur, as seen in by a small study from South India reporting that 12 patients with acute motor axonal neuropathy out of 84 patients with Guillain-Barré syndrome had prominent finger extensor weakness with relative preservation of finger flexors, wrist flexors, and wrist extensors (45). Reflexes are typically absent in advanced stages of the disease, and deep tendon reflex responses correlate with the severity of weakness; however, normal to increased reflexes have been reported in rare cases (179; 87; 26; 139; 114). In fact, although areflexia or hyporeflexia is a diagnostic criterion for Guillain-Barré syndrome. However, it should be known that in rare instances, all Guillain-Barré syndrome subtypes can initially present with hyperreflexia (162). Guillain-Barré syndrome patients with hyperreflexia are more frequently associated with antecedent diarrhea than upper respiratory tract infection and with acute motor axonal neuropathy than acute inflammatory demyelinating polyneuropathy. Among patients with Guillain-Barré syndrome with hyperreflexia, most have generalized hyperreflexia, and antiganglioside antibodies are frequently present.

Autonomic dysfunction is uncommon in acute motor axonal neuropathy, unlike in other subtypes of Guillain-Barré syndrome (11). However, cases of autonomic dysfunction in acute motor axonal neuropathy have been reported and include bowel, bladder, erectile dysfunction, and even severe pulmonary hypertension (138; 133).

Patients with acute motor axonal neuropathy can rarely have concomitant CNS involvement, including CNS demyelination, altered consciousness and loss of brainstem reflexes, cerebellar ataxia, and atypical sensory symptoms (175; 16; 94; 109; 137; 105; 91).

Rarely, acute motor axonal neuropathy may present asymmetrically (31; 100; 136).

Prognosis and complications

Progression beyond involvement of the nodes of Ranvier and motor nerve terminals is the most important determinant of prognosis in acute motor axonal neuropathy, with rapid recovery within 12 months seen in patients with disease limited to the nodes and terminals (155). Once axonal degeneration occurs, the most important determinant is the site of axonal injury and the extent of the involvement of the axons. Patients with injury confined to distal motor axons can recover rapidly, whereas patients with significant disease burden in spinal roots or proximal nerve trunks are more likely to recover slowly and be left with residual muscle weakness. According to Sung and colleagues, 75% of patients with acute motor axonal neuropathy in the study were unable to walk independently at 1 month after admission (150). Hiraga and colleagues found that 14% of patients with acute motor axonal neuropathy were unable to walk independently at 6 months, but this fell to 5% at 12 months. By 57 months, all patients could walk independently (62).

Long-term follow-up on large cohorts of patients with acute motor axonal neuropathy is not available. Residual symptoms were noted up to 6 years after syndrome onset in a group of patients with Guillain-Barré syndrome, not separated by subtype, questioned from the Dutch Guillain-Barré syndrome trial (22). Of 122 Dutch patients, 84 (69%) had no symptoms or only minor neurologic symptoms. Twenty-four patients were able to walk more than 10 meters without assistance. Fourteen were bedbound or only able to walk with support or a walker. Psychosocial status changes concerning work and daily living activities were seen in 63% of patients. Thirty-eight percent experienced a change in work status, with many able to return to work.

The prognosis of patients with acute motor axonal neuropathy compared with patients with the acute inflammatory demyelinating polyradiculoneuropathy variant is contested, with more studies suggesting a worse prognosis for acute motor axonal neuropathy. Patients with acute motor axonal neuropathy, compared to patients with acute inflammatory demyelinating polyradiculoneuropathy, have shown a more rapid progression, earlier nadir, and poorer prognosis, with more residual weakness and incomplete recovery (63; 121; 190; 78; 57). However, other studies have found a similar prognosis between acute inflammatory demyelinating polyradiculoneuropathy and acute motor axonal neuropathy (156; 90).

Good prognostic indicators for acute motor axonal neuropathy are milder symptoms at presentation, relatively high amplitude of median, ulnar, deep peroneal, and posterior tibial CMAPs, and high amplitude of median, ulnar, and superficial peroneal SNAPs (150; 125). Predictors of poor prognosis include increased fasting serum and CSF glucose levels, cranial nerve palsy, severe muscle weakness, and the requirement of mechanical assistance (57; 46; 149). Studies have also suggested that useful prognostic indicators are muscle strength at day 10 and functional status at the nadir of the disease course (121; 90).

Serial electrodiagnostic testing is most commonly used to assess the site and extent of axonal injury, though MRI of the muscle may be a useful technique for assessing the extent of motor axonal injury and resultant clinical course. Good concordance between clinical, electrophysiological, and MRI findings has been reported (20). Likewise, ultrasound of cervical nerve roots and peripheral nerves was shown to help distinguish axonal (AMAN, AMSAN) from demyelinating (AIDP) Guillain-Barré syndrome variants (108; 93). MRI and nerve ultrasound may, thus, be useful adjunctive techniques in situations where extensive EMG examination is not feasible, as in small children.

Serum neurofilament light chain (NfL) levels are emerging as a biomarker of axonal damage in many diseases of the CNS and PNS. A study examining serum and CSF NfL levels found that patients with Guillain-Barré syndrome and axonal or mixed axonal-demyelinating pathology showed significantly lower CSF/serum NfL ratios indicative of a peripheral nerve origin of NfL (81). Role of serum NfL levels as a prognostic biomarker in Guillain-Barré syndrome has been examined (99). It was found that baseline serum NfL levels are increased in patients with Guillain-Barré syndrome, are associated with disease severity and axonal variants, and have an independent prognostic value. In another study, Altmann and colleagues found that serum NfL levels measured at hospital admission correlated with clinical outcome in patients with Guillain-Barré syndrome (03). The patients with higher baseline serum NfL levels were more likely to have longer hospitalization times or need intensive care.

NfL levels are not specific to axonal damage in the PNS but are also elevated with neuronal and/or axonal injury to the CNS. Therefore, two studies proposed novel biomarkers for the specific detection of peripheral nerve damage in immune neuropathies, including acute motor axonal neuropathy. Keddie and colleagues reported that elevated levels of peripherin, an intermediate filament almost exclusively expressed in the axonal cytoskeleton of PNS neurons, were more specific than NfL in distinguishing peripheral nerve axonal damage from CNS-associated axonal injury, and serum peripherin levels distinguished Guillain-Barré syndrome from CIDP and CNS diseases with good diagnostic accuracy. In Guillain-Barré syndrome, serum peripherin levels mostly peaked monophasically during the acute phase and dropped thereafter, but the Guillain-Barré syndrome cohort size was discussed to be too small to yet assess its utility as a biomarker for routine clinical application (80). Compared to NfL levels, peripherin levels were able to distinguish Guillain-Barré syndrome from chronic inflammatory demyelinating polyneuropathy, with even increased diagnostic accuracy when combining both analytes (77% sensitivity, 89% specificity). Furthermore, unlike NfL, peripherin serum levels were age-independent. Although only five acute motor axonal neuropathy patients were included in this study, peripherin also appears to be a promising biomarker to facilitate the differentiation of axonal from demyelinating Guillain-Barré syndrome variants, but future larger studies are warranted to prove this assertion (80).

Nishimoto and colleagues studied 51 children with Guillain-Barré syndrome in Japan and found that those with antiganglioside autoantibodies were more frequently diagnosed with acute motor axonal neuropathy (64% vs. 11%). Functional status on admission was similar between antibody-positive and antibody-negative children. A slower recovery was found in 78% of the antibody-negative group at follow-up, compared to 29% of antibody-positive patients, leading the authors to suggest that antibody testing might be a helpful prognostic tool (115).

Glial fibrillary acidic protein is a protein expressed in the cytoskeleton of mature astrocytes and nonmyelin-forming Schwann cells. Notturno and colleagues found higher levels of glial fibrillary acidic protein in all patients with Guillain-Barré syndrome, with higher levels noted in patients with acute motor axonal neuropathy. Higher levels corresponded to worsening functional status at 6 months, suggesting glial fibrillary acidic protein could be a possible diagnostic marker for acute motor axonal neuropathy and a predictive tool for recovery (116). The utility of this biomarker to predict prognosis needs to be validated in larger clinical studies.

In another study, CSF levels of sphingomyelin, a sphingolipid expressed in the myelin sheath, distinguished chronic inflammatory demyelinating polyneuropathy and acute inflammatory demyelinating polyradiculoneuropathy from other neurologic diseases and predicted outcome (27). A trend towards higher sphingomyelin levels in acute inflammatory demyelinating polyradiculoneuropathy than in acute motor axonal neuropathy patients was seen, but due to a small acute motor axonal neuropathy cohort size (n=3), follow-up studies are required to assess the utility of CSF sphingomyelin levels to distinguish between axonal and demyelinating Guillain-Barré syndrome variants.

A study by Zhang and colleagues demonstrated that soluble receptor for advanced glycation end products (sRAGE) and high-mobility group box 1 (HMGB1) may be used clinically as biomarkers. In the study, serum HMGB1, IL-6, and TNF-alpha levels in all subtypes of Guillain-Barré syndrome were higher than those in healthy controls, whereas patients with the acute motor axonal neuropathy subtype had significantly lower levels of serum sRAGE compared to healthy controls (188). sRAGE and HMGB1 levels normalized after treatment. Furthermore, an additional study demonstrated significantly higher serum IL-36α and IL-36γ cytokine levels in the acute phase of Guillain-Barré syndrome compared to the remission phase. Further, the serum and CSF levels of IL-36α and IL-36γ in the axonal variants of Guillain-Barré syndrome were higher than in the demyelinating subtypes. (195).

Complications. In addition to long-term poor conditioning and loss of strength, other complications in the more acute and subacute phases include dysautonomia, infection, respiratory dysfunction, deep venous thrombosis, pulmonary embolism, contractures, peroneal nerve compression palsies, hypercalcemia and heterotopic calcification from immobility, decubitus ulcers, anemia, psychological abnormalities, and poor nutrition (134; 104).

In fulminant Guillain-Barré syndrome, defined as involvement of both central and peripheral nervous systems and resulting in a comatose state, patients often have relatively rapid recovery of central nervous system function within days; in contrast, recovery of peripheral nervous system function occurs over the course of years and is often incomplete (105). Due to this phenomenon, a patient can regain normal brain function yet clinically appear brain dead due to the absence of cranial nerve reflexes. In cases of fulminant Guillain-Barré syndrome in which there is a question of brain death, the author recommends performing ancillary testing, such as a 24-hour electroencephalogram or transcranial Doppler. Awareness of the potential clinical manifestations and long-term prognosis of fulminant Guillain-Barré syndrome is crucial for acute management of these patients as well as education and counseling provided to family members and other healthcare providers (129).

Clinical vignette

A 43-year-old man with a history of diabetes presented to the emergency department after 3 days of progressive weakness. The patient reported that 3 days before presentation, he had noted weakness in his feet when he tripped over a curb getting out of his car. Over the following 72 hours, the weakness had ascended his legs and began affecting his arms, to the point where he had difficulty combing his hair or drinking from a glass. The patient had recently returned from traveling abroad and reported 3 days of diarrhea 2 weeks before the start of his weakness.

On arrival, his examination was notable for symmetric quadriplegia, with greater weakness in his legs than in his arms. He was noted to have diffuse hyperreflexia in his arms and legs but no deficits in sensation or coordination. He could walk 10 meters with one-person assist.

The patient was admitted as an inpatient, and given the patient’s quadriplegia and hyperreflexia, the primary team obtained MRIs of his brain and cervical spine, which were unremarkable. Cerebrospinal fluid was notable for elevated protein with a normal number of white blood cells. Over one and a half days, he developed worsening quadriplegia, facial diplegia, dysphagia, and dysarthria, and on repeat testing, his reflexes were absent. Two days later, he required mechanical ventilation.

Initial nerve conduction studies on day 4 showed low compound muscle action potential amplitudes with absent F-waves in the lower extremities and partial conduction block of the ulnar nerve across the right elbow. Repetitive nerve stimulation at 3 and 30 Hz did not reveal decremental or incremental responses. Electromyography showed no evidence of increased insertional or spontaneous activity. He was diagnosed with Guillain-Barré syndrome and treated with IVIG, though the patient was unable to be extubated and underwent placement of tracheostomy and gastrostomy tubes. Serum antibodies eventually returned positive for anti-GD1b antibodies.

One month after presentation, the patient still required mechanical ventilation. Nerve conduction studies at 1 month showed further reduced compound muscle action potential amplitudes with absent F-waves in the upper extremities and no change in conduction velocities. Repeat electromyography showed acute denervation with fibrillation and positive sharp waves in all tested muscles.

At 3 months after presentation, the patient no longer required mechanical ventilation but remained nonambulatory. However, 1 year after presentation, the patient could walk independently for 1 meter.

Discussion. This fictitious case report highlights several common features seen in acute motor axonal neuropathy. Antecedent infections are closely associated with acute motor axonal neuropathy, especially Campylobacter jejuni, which is a common cause of “traveler’s diarrhea.” Compared to demyelinating forms of Guillain-Barré syndrome, acute motor axonal neuropathy progresses rapidly, reaching a nadir within days to a week.

There are no differences in the neurologic examination between axonal and demyelinating forms of Guillain-Barré syndrome, though axonal variants are thought to be more likely to progress to more severe weakness and eventual cranial nerve involvement. Although reflexes are classically reduced or absent, all variants of Guillain-Barré syndrome may initially present with hyperreflexia, which may delay the diagnosis. Pure motor involvement supports the diagnosis of acute motor axonal neuropathy over other variants of Guillain-Barré syndrome. Autonomic dysfunction is less common in acute motor axonal neuropathy.

Albuminocytologic dissociation is classically seen in all variants of Guillain-Barré syndrome. Antiganglioside antibodies are strongly associated with acute axonal motor neuropathy, especially those against GD1b and GM1. Electrodiagnostic studies distinguish between axonal variants and demyelinating ones with reduced compound muscle action potentials and preserved conduction velocities, though conduction block can occur early in the disease. Later on, findings of denervation confirm that the disease has progressed to axonal degeneration.

As with other forms of Guillain-Barré syndrome, treatment involves supportive care and either IVIG or plasmapheresis. Acute motor axonal neuropathy is possibly associated with a more severe initial course, but it is notable for a relatively rapid recovery when compared to other motor neuropathies, such as vasculitis, with most patients regaining ambulation within 1 year.

Biological basis

Etiology and pathogenesis

Clinical and experimental data support that acute motor axonal neuropathy is an antibody-mediated nerve disorder. Like other variants of Guillain-Barré syndrome, antecedent triggering infections are common. Acute motor axonal neuropathy is strongly associated with preceding Campylobacter jejuni infection, particularly in Chinese, Japanese, and Bangladeshi populations (72). The bulk of clinical and experimental evidence supports the hypothesis that target epitopes are cell surface glycans called gangliosides, which are the constituents of the axolemma of the motor fibers. Autoantibodies against gangliosides arise due to molecular mimicry, and this hypothesis is supported by the following observations:

	• C jejuni enteritis is the most commonly recognized antecedent infection in acute motor axonal neuropathy.
	• Acute motor axonal neuropathy is strongly associated with specific antiganglioside antibodies, most commonly GM1 and GD1a.
	• C jejuni isolates from patients with Guillain-Barré syndrome carry relevant ganglioside-like moieties.
	• Gangliosides, the purported target antigens, are enriched in the nerve fibers.
	• Pathological and immunopathological studies in acute motor axonal neuropathy indicate antibody-mediated axonal injury.
	• Experimental studies show that antiganglioside antibodies can induce motor nerve fiber injury, mimicking acute motor axonal neuropathy.

Antecedent events like infections are common in acute motor axonal neuropathy, with Campylobacter jejuni gastroenteritis most frequently associated. Other infections have also been reported to precede axonal variants of Guillain-Barré syndrome, including Haemophilus influenzae, mycoplasma, H1N1 influenza, dengue, Chikungunya, Japanese encephalitis virus, Salmonella infection, Zika virus, Staphylococcus aureus endocarditis, coronavirus (SARS-CoV-2) 2019, HIV, Acinetobacter baumannii, Hepatitis A, Hepatitis E, Epstein-Barr virus, varicella zoster, and Campylobacter Rectus (61; 128; 59; 147; 33; 151; 79; 127; 168; 89; 95; 83; 36; 158; 10; 25; 171; 148; 02; 34; 43; 153; 71; 141; 170; 18).

Amongst these infectious agents, Campylobacter jejuni and H influenzae carry ganglioside-like moieties (107). Campylobacter is discussed further because of its frequent association with acute motor axonal neuropathy and relevance to the molecular mimicry hypothesis.

Campylobacter jejuni is a gram-negative rod and one of the most common causes of bacterial gastroenteritis worldwide (69; 44; 117). Infection with C jejuni is found in 13% to 72% of patients with acute motor axonal neuropathy or Guillain-Barré syndrome (69; 54), with an overall prevalence estimated around 30% (106). The lipopolysaccharide of C jejuni carries ganglioside-like moieties. Several studies have characterized these in Guillain-Barré syndrome and diarrhea-associated C jejuni strains. GM1-, GD1a-, GalNAc-GD1a-, GM1b-, GT1a-, GD2-, GD3-, and GM2-like structures have all been identified (15; 13; 14; 185; 186; 184; 145; 112; 111). It is these structures that are likely to provide the initial stimulus to autoimmune activation and induction of antiganglioside antibodies in patients with post-Campylobacter acute motor axonal neuropathy.

Campylobacter jejuni has been estimated to affect more than 1% of the population per year worldwide, but Guillain-Barré syndrome occurs in approximately 1.5 per 100,000. This suggests that fewer than 0.01% of Campylobacter jejuni cases are associated with Guillain-Barré syndrome (145; 174), raising the hypothesis of host susceptibility. The host properties that confer this susceptibility to Guillain-Barré syndrome after Campylobacter infection remain unknown, but most likely involve the loss of tolerance to self-gangliosides (97; 24). Hypotheses that have been proposed to explain host susceptibility include the presence of specific HLA alleles, promoter polymorphisms in the genes for tumor necrosis factor-alpha and toll-like receptor-4, and polymorphisms in the gene for mannose-binding lectin 2 (183; 132; 194; 126; 77; 74; 75). Additionally, promoter polymorphisms in the genes for tumor necrosis factor-alpha and toll-like receptor-4 have also been associated with increased susceptibility to the acute motor axonal neuropathy variant of Guillain-Barré syndrome but not to the acute inflammatory demyelinating polyradiculoneuropathy variant (194; 126; 77; 74; 75).

Gangliosides, the target antigens of the antiganglioside antibodies induced by antecedent infections, are sialic-containing glycosphingolipids that are widely distributed in the mammalian tissues but are particularly enriched in the nervous system. The ceramide portion of gangliosides anchors them into the plasma membrane, whereas oligosaccharide moieties extend into the extracellular space from the cell surface, making them accessible to antibodies in the environment. Gangliosides are classified based on the number and linkage of the sugar backbone and attached sialic acids. There are many ganglioside species, but GM1, GD1b, GD1a, and GT1b are the most abundant in peripheral nerves. Antiganglioside antibodies are found in the serum of a proportion of patients with Guillain-Barré syndrome. These antibodies are polyclonal, predominantly IgG, and generally complement-fixing IgG1 and IgG3 (119; 66). In patients with acute motor axonal neuropathy, anti-GM1 or -GD1a can be detected in 50% to 60% of patients, and antibodies to minor gangliosides GalNAc-GD1a and GM1b are found in about 10% to 15% of cases (187; 180; 178; 130; 73; 66; 118). In acute motor axonal neuropathy preceded by Zika virus infection, antibodies to GA1 were detected in 19% to 46% of patients in one study (25).

The gangliosides most associated with acute motor axonal neuropathy, GM1 and GD1a, are localized at the nodes of Ranvier and in the motor nerve terminals (144; 47), providing an explanation as to why these structures are affected early in the disease. In acute motor axonal neuropathy, antibodies bind to the nodes of Ranvier and paranodal structures via gangliosides and activate complement, which disrupts cell adhesion molecules necessary for the formation and maintenance of nodal structure and function (152). This disruption of nodal structure correlates with a failure of conduction across affected nodes. The activation of complement at the nodes of Ranvier also causes the recruitment of macrophages, which then insert their processes into the nodal gap and open the normally impermeable periaxonal space to endoneurial constituents, including antibody and complement (154; 55; 122; 172; 152). The paranodal sites of the Schwann cell myelin sheath attachment to the axon are then disrupted, allowing recruitment of macrophages to the periaxonal internodal space. This leads to axonal shrinkage and separation from the Schwann cell plasmalemma. The axon survives for some time before undergoing Wallerian-like degeneration (51). In addition to axonal degeneration, axonal regeneration is inhibited by activation of the Fc gamma receptor on macrophages (189; 58; 143). It is important to emphasize that not all patients develop axonal degeneration, and patients with rapid recovery likely develop pathological changes restricted to the nodes of Ranvier.

The motor nerve terminal is another site of injury in acute motor axonal neuropathy, and this part of the motor axon is susceptible to antibody-mediated injury because it lies outside of the blood-nerve barrier (64). In a series of studies, Willison and colleagues showed that antiganglioside (including anti-GD1a) antibodies bind to nerve terminals and cause complement-dependent decreased quantal acetylcholine release, resulting in neuromuscular blockade and a calcium-dependent degeneration of the motor nerve terminal (173; 49; 124; 120; 48). The clinical, pathological, and electrophysiologic effects in this model are almost entirely abrogated by the application of an inhibitor of the C5 component of complement (eculizumab), which prevents formation of the membrane attack complex (56). Nerve injury restricted to the nerve terminals provides another potential rationale to explain rapid recovery, as the degenerated axon must grow a short distance to reconnect with target muscle fibers and restore function (64). As such, antibody and complement-dependent injury that is restricted to the nodes or motor nerve terminals may explain patients who demonstrate rapid recovery. In contrast, a variable extent of axonal degeneration is seen in patients with a prolonged disease course.

It is not well understood how antiganglioside antibodies lead to specific involvement of the motor axons while sparing the sensory axons in acute motor axonal neuropathy, as many biochemical studies indicate that there are no consistent differences in ganglioside content of motor and sensory fibers. Gong and colleagues have suggested that the accessibility of the gangliosides to antibodies may vary between motor and sensory nerves, and variation in susceptibility to injury may contribute to distinctions between acute motor axonal neuropathy and acute motor-sensory axonal neuropathy (47). Supporting this hypothesis, several authors have demonstrated preferential immunohistochemical staining of motor nerve fibers with monoclonal anti-GD1a antibodies in rodents and humans (37; 32; 47; 96). However, similar differences in staining were not detected with GM1. It has been hypothesized that acute motor axonal neuropathy and acute motor-sensory axonal neuropathy share a common immunological profile, and both are likely part of a spectrum of immune-mediated forms of attack on nerve axons (51; 181).

Acute motor axonal neuropathy (early changes)

(A) Deposition of C3d at the nodes of Ranvier (arrows). (B) Macrophages overlying and inserting processes at the nodes of Ranvier (arrow). (C) Normal node of Ranvier (arrow). (D) Lengthening of the node (arrow) with overlying m...
Acute motor axonal neuropathy (late changes)

(A) C3d deposition on internodal axolemma (arrows). (B) Macrophage in the periaxonal space and lying adjacent to an axon. (C) Left fiber: macrophage (M) surrounding a shrunken but intact axon (A) inside normal-appearing myelin ...

The earliest pathological changes in acute motor axonal neuropathy are subtle and affect the nodes of Ranvier of motor fibers in the ventral roots (51). These changes consist of lengthening of the nodal gap. Recruitment of macrophages to the nodes occurs early on. At the immunopathological level, it is noted that early in the pathogenetic process, IgG binds at the nodes of Ranvier, leading to activation of complement, suggested by C3d deposition, which causes macrophage recruitment (55). These macrophages then insert their processes into the nodal gap and open the periaxonal space, which is normally impermeable, to endoneurial constituents, including antibody and complement. Immunopathological analysis at this stage shows deposition of IgG and complement activation marker C3d in periaxonal space and on the internodal axolemma in late cases (55). Macrophages are then recruited to the periaxonal internodal space after the disruption of paranodal sites of the Schwann cell myelin sheath attachment to the axon. This leads to axonal shrinkage and separation from Schwann cell plasmalemma. The axon survives for some time before undergoing Wallerian-like degeneration (51).

Differential diagnosis

Confusing conditions

The differential diagnosis of acute ascending paralysis includes acute myelopathy, such as from spinal cord compression, infarction, infection, or transverse myelitis. The presence of bowel or bladder dysfunction or a sensory level favors a spinal cord abnormality over a peripheral neuropathy.

A neuromuscular junction abnormality, such as myasthenia gravis or botulism, should be considered. Although some forms of Guillain-Barré syndrome, such as Miller-Fisher syndrome, present with ophthalmoparesis, acute motor axonal neuropathy rarely has ocular findings. Botulism generally presents with dilated, unreactive pupils and constipation. Electrodiagnostic studies with repetitive stimulation can be helpful.

West Nile virus infection may produce weakness, including an axonal polyneuropathy or a poliomyelitis-type illness on electromyography and nerve conduction studies (113). These patients should present with a febrile illness along with mental status changes at the time of weakness, although encephalopathy is not always evident. Poliomyelitis is a consideration in geographical locales where polio is still not eradicated (92), but most patients have a gastroenteritis and fever with an acute onset of weakness that tends to be asymmetrical. Lyme disease should have a history of tick bite and erythema migrans. Other infections, such as diphtheria, may present with acute paralysis, and tick paralysis should improve with the removal of the parasite. Paralytic rabies can also mimic axonal Guillain-Barré syndrome (146).

Other considerations should include toxin exposure, such as heavy metals and organophosphates, that can have a similar presentation. Acute porphyria should have a predominantly motor axonopathy, but other symptoms such as abdominal complaints, psychiatric disturbances, and seizures are common (135).

Associated or underlying disorders

Development of autoantibodies to gangliosides, either spontaneously or due to antecedent infection, is thought to underlie the pathogenesis of acute motor axonal neuropathy. As such, exposure to infectious agents with ganglioside-like moieties is associated with the development of acute motor axonal neuropathy, as discussed more thoroughly in the “Etiology and Pathogenesis” section.

Diagnostic workup

Electrodiagnostic examination is required to distinguish acute motor axonal neuropathy from other forms of Guillain-Barré syndrome. Ho and colleagues proposed the electrodiagnostic criteria (Table 2) (65).

Diagnosis of acute inflammatory demyelinating polyneuropathy must have one of the following in two or more nerves during the first two weeks of illness:

	• Conduction velocity less than 90% of the lower limit of normal if amplitude is greater than 50% of the lower limit of normal; less than 85% if the amplitude is less than 50% of the lower limit of normal.
	• Distal latency greater than 110% of the upper limit of normal if amplitude is normal; greater than 120% of the upper limit of normal if amplitude is less than the lower limit of normal.
	• Evidence of unequivocal temporal dispersion.
	• F-response latency greater than 120% of normal.

Diagnosis of acute motor axonal neuropathy:

	• No evidence of demyelination as defined above.
	• Decrease in compound muscle action potential amplitude to less than 80% of the lower limit of normal.

These criteria have been widely accepted. Hadden and colleagues further modified the classification to include a ratio of the proximal compound muscle action potential amplitude to the distal compound muscle action potential amplitude of less than 0.5 to be further evidence of demyelination (53). However, acute motor axonal neuropathy may show early partial motor conduction block with no further evidence of demyelination or later remyelination (26). The principal abnormalities of acute motor axonal neuropathy are reduced distal compound muscle action potential amplitude and absent F-wave responses (102). When there is electrodiagnostic evidence of acute motor axonal neuropathy and amplitudes of sensory nerve action potentials are below the lower limit of normal, the diagnosis of acute motor-sensory axonal neuropathy should be considered (182).

Table 2. Electrophysiological Separation of Guillain-Barré Syndrome Variants

	AIDP	AMAN/AMSAN
Conduction velocity	<90% if amp > 50% <85% if amp < 50%	> 90% if amp > 50% > 85% if amp < 50%
Distal latency	> 110% if amp normal > 120% if amp reduced	< 120%
F-wave latency	> 120% ULN	< 120% ULN
CMAP amplitude	pCMAP/dCMAP ratio < 0.5 and dCMAP 20% LLN	dCMAP < 80% LLN in at least two nerves
Sensory		Sensory Amp < 10% LLN in AMSAN; normal in AMAN

If initial studies are normal and the diagnosis is in question, the nerve conduction studies should be repeated in a few days. Some advocate mandatory serial electrophysiological studies to eliminate diagnostic uncertainty, even if initial studies appear conclusive (160). Uncini and colleagues studied 55 patients with Guillain-Barré syndrome subtypes who had serial electrodiagnostic recordings. The diagnosis was changed in 24% of patients after the follow-up electrodiagnostic study, and the main shift was from equivocal or demyelinating electrophysiology to an axonal subtype of Guillain-Barré syndrome. This shift was related to reversible conduction failure and the length-dependent compound muscle action potential amplitude reduction (161). In another case report, a patient had motor conduction blocks in all peripheral nerves in electrophysiological studies and was diagnosed as having acute inflammatory demyelinating polyradiculoneuropathy. Later, reduction of CMAP amplitudes in the posterior tibial nerve, absence of CMAPs in the median, ulnar, and peroneal nerves, and loss of motor conduction blocks were found on repeat electrophysiological studies. According to these findings, the patient’s diagnosis was changed to acute motor axonal neuropathy. The authors suggest that motor conduction blocks may appear in the early stage of acute motor axonal neuropathy and they disappear on serial studies; hence, it is better to repeat electrodiagnostic studies in patients with Guillain-Barré syndrome (176). The difficulty in distinguishing axonal from demyelinating Guillain-Barré syndrome variants within the first four days after disease onset was also pointed out by another study (21). This study stressed the need for serial electrophysiological assessments to delineate acute motor axonal neuropathy from AIDP.

Several studies have demonstrated that relative “sparing” of sural SNAP on nerve conduction studies is one of the most specific findings for Guillain-Barré syndrome (38). Sural-sparing is defined as an observed decrease in the median or ulnar SNAP that is greater than the observed decrease in sural SNAP. Additionally, calculating the percentage change in median and ulnar SNAP and comparing it with the change in sural SNAP may enable the electrodiagnostician to detect subclinical sural-sparing and increase the yield of nerve conduction studies in the early stages of Guillain-Barré syndrome. Umapathi and colleagues recommend that if the initial nerve conduction studies of a patient with suspected Guillain-Barré syndrome demonstrate an abnormal sural SNAP with normal upper limb SNAP, then the electrodiagnostician should question the diagnosis, regardless of Guillain-Barré syndrome subtype (159). However, sural sparing may be less common in axonal variants, as one study demonstrated that although 53% of patients with acute inflammatory demyelinating polyradiculoneuropathy presented with sural sparing, sural sparing was present in only 8% of patients with acute motor axonal neuropathy (29).

Another study used CMAP scan to differentiate between axonal and demyelinating forms of Guillain-Barré syndrome (40). Plotting the CMAP amplitudes against a range of stimulus intensities results in a dose-response curve that defines the CMAP scan. Patients with acute motor axonal neuropathy and AIDP have pronounced differences in CMAP scan stimulus intensity parameters at the onset of disease, which allows for differentiation between these Guillain-Barré syndrome subtypes.

Repetitive stimulation should be performed only to assess if neuromuscular junction abnormality, such as botulism or myasthenia gravis, is the cause of the patient’s acute flaccid paralysis.

Cauda Equina Conduction Time (CECT) is another technique investigated to differentiate demyelinating and axonal types of Guillain-Barré syndrome. Matsumoto and colleagues performed a study to compare CECT in nine demyelinating and seven axonal Guillain-Barré syndrome patients. They used Magnetic Augmented Translumbosacral Stimulation (MATS) to activate nerves at both the proximal and distal sites of the cauda equina for the measurement of CECT. In this study, although motor conduction velocity at the peripheral nerve trunk was normal in patients with demyelinating Guillain-Barré syndrome and in patients with axonal variants of Guillain-Barré syndrome, only patients with demyelinating Guillain-Barré syndrome had prolonged CECT (101).

Ultrasound of cervical nerve roots and peripheral nerves is another diagnostic tool to diagnose Guillain-Barré syndrome and distinguish between AMAN/AMSAN and AIDP: AMAN/AMSAN patients revealed milder cervical root enlargement than AIDP patients, and vagal nerve enlargement correlated with autonomic dysfunction in a Chinese study (93). The 2023 EAN/PNS guidelines on diagnosis and treatment of Guillain-Barré syndrome recommend nerve ultrasound (and/or nerve/plexus MRI) as additional diagnostic tools in atypical Guillain-Barré syndrome cases (164).

Cerebrospinal fluid can be evaluated to help with the diagnosis of Guillain-Barré syndrome. After the first week of symptoms, cerebrospinal fluid protein may be elevated in most patients; a normal cerebrospinal fluid protein level within the first week of symptoms does not exclude Guillain-Barré syndrome. Typically, cerebral spinal fluid white cells are less than 10, but counts up to 50 cells per mm3 have been recorded in Guillain-Barré syndrome (12). CSF profile in acute motor axonal neuropathy is expected to conform to the preceding observations for Guillain-Barré syndrome in general. A study correlated the CSF total protein levels with different subtypes of Guillain-Barré syndrome (23). This study found significantly higher CSF total protein levels in Guillain-Barré syndrome cases classified as demyelinating compared to axonal subtypes.

Although the presence of antiganglioside antibodies is common in Guillain-Barré syndrome, especially GM1 and GD1a with the acute motor axonal neuropathy variant, their presence is neither specific nor sensitive for distinguishing between axonal and demyelinating variants of Guillain-Barré syndrome. The EAN/PNS guidelines do not recommend routine testing of serum antibodies against gangliosides (164). However, detecting antibodies against multiple gangliosides arranged in a complex rather than against each ganglioside separately may improve sensitivity and specificity. One paper by Thomma and colleagues reported that 92.6% of patients with Guillain-Barré syndrome were positive for at least one antiganglioside complex antibody. Furthermore, using models based on antibodies to antiganglioside complexes rather than single gangliosides increased sensitivity for detecting Guillain-Barré syndrome and acute motor axonal neuropathy (62% to 83% and 53% to 72%, respectively), while preserving sensitivity (79% to 81% and 93% to 89%, respectively) (157).

Stool cultures or serological studies for Campylobacter jejuni may be considered in patients who present with recent gastroenteritis, especially because the HS2, HS4c, HS19, HS23/26c, HS41, and HS1/44c Campylobacter jejuni capsular subtypes are associated with acute motor axonal neuropathy (60).

If clinically indicated, urine porphyrins, heavy metals, Lyme titers, West Nile, autoimmune serologies, botulism serologies, and imaging of the spinal cord should be considered to help exclude other causes of acute paralysis.

Management

Despite the availability of two specific immunotherapies, namely, IVIG and plasma exchange, the mainstay of management in Guillain-Barré syndrome or acute motor axonal neuropathy remains the provision of supportive care (including admission to ICU) during the acute phase to prevent complications and facilitate recovery. There are no controlled studies of immunomodulatory therapy in the primary axonal variants of Guillain-Barré syndrome, but anecdotal experience indicates that both plasma exchange and IVIG are beneficial. To date, there is no difference in immunomodulatory treatments in acute motor axonal neuropathy and other subtypes of Guillain-Barré syndrome (09; 64; 53), which was affirmed by the 2023 EAN/PNS guidelines on diagnosis and treatment of Gullain-Barré syndrome (164). Due to the ease of its administration and broader patient acceptability, IVIG is used more frequently for the treatment of Guillain-Barré syndrome. The 2023 EAN/PNS guidelines strongly recommend that patients with Guillain-Barré syndrome or acute motor axonal neuropathy be referred to specialized centers with an ICU experienced in managing patients with acute flaccid paralysis. Patients with a rapidly progressing disease, bulbar involvement, and low muscle strength, especially in proximal muscles, are at risk for mechanical ventilation and should be admitted early to an ICU for surveillance (164). This is important because without optimal supportive or intensive care, this group of disorders still carries a significant risk of mortality, and the simplicity of IVIG treatment occasionally prevents appropriate referral to centers experienced in managing Guillain-Barré syndrome.

Plasmapheresis improves disability and speeds recovery in Guillain-Barré syndrome compared to supportive treatment alone (04; 05). When using plasmapheresis, the French Cooperative Group suggests treating patients with mild Guillain-Barré syndrome with two exchanges and moderate to severe forms with four exchanges. There was no additional benefit in receiving six versus four exchanges in severe cases (08). The EAN/PNS guidelines recommend two plasma exchange cycles early after disease onset (less than 2 weeks) for mildly affected patients who are able to walk unaided and four to five plasma exchange cycles in severely affected patients (164). In some centers, immunoadsorption (IA) is performed instead of plasma exchange due to purported better tolerability, but randomized controlled trials on immunoadsorption in Guillain-Barré syndrome are currently lacking (98), and treatment with immunoadsorption is not recommended by the 2023 EAN/PNS guidelines (164).

The dose of intravenous immunoglobulin is generally set at 2 grams per kilogram and is generally divided into five doses of 400 milligrams per kilogram per dose. The infusion rate should not exceed 200 milliliters per hour or 0.08 milliliters per kilogram per minute. Dalakas suggested giving 1 gram per kilogram for 2 consecutive days and stated he had not found more adverse side effects with a 2-day infusion (35), but the EAN/PNS guidelines favored the 5-day standard therapy over the 2-day course due to a lower probability of treatment-related fluctuations (164).

No statistical significance was noted when plasmapheresis was compared to intravenous immunoglobulin (163) or when plasmapheresis was followed by intravenous immunoglobulin (09); therefore, the 2023 EAN/PNS guidelines stated equality of IVIG and plasma exchange to treat Guillain-Barré syndrome (164). Most large Guillain-Barré syndrome trials include patients with moderate to severe disease. Given the expense of plasmapheresis and intravenous immunoglobulin treatment, electing not to treat those with mild disease is certainly understandable and in many centers is the protocol. Because the French Cooperative Group found a faster recovery rate in treating those with mild disease, the 2023 EAN/PNS guidelines recommend treating all cases of acute motor axonal neuropathy with either intravenous immunoglobulin or plasmapheresis within two weeks of onset or earlier (164).

Kuwabara and colleagues suggest intravenous immunoglobulin may be superior to plasmapheresis in cases of acute motor axonal neuropathy with positive anti-GM1 autoantibodies. The patients treated with IVIG had significantly lower Hughes grade scores 1, 3, and 6 months after onset and a higher probability of regaining independent locomotion at 6 months. Rapid recovery was more frequent, and delayed recovery was less frequent in the IVIG subgroup (86). The authors postulate that if autoantibodies are pathogenic, intravenous immunoglobulin can displace antibodies bound to motor nerves, possibly preventing complement activation (192). Plasmapheresis would only remove free-circulating antibodies (177).

Historically, relapse after treatment, called treatment-related fluctuation, was thought to occur in about 5% to 10% of patients, irrespective of treatment modality. However, it is now known that about 5% of patients who present with Guillain-Barré syndrome are later found to have acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). As such, the 2023 EAN/PNS guidelines recommend retreatment with the original modality in patients who have relapsed after initial improvement, but to consider changing the diagnosis from Guillain-Barré syndrome to A-CIDP in patients with three or more treatment-related fluctuations (164). For patients who show no initial improvement or who have poor prognosis, there was no improvement in a second IVIG course in both the observational International Second IVIG Dose (ISID) study and the Second intravenous Immunoglobulin Dose in patients with Guillain-Barre Syndrome with poor prognosis (SID-GBS) trial (166; 169), which is why the 2023 EAN/PNS guidelines strongly recommend against a second course of IVIG in treatment-refractory patients because it is associated with minimal benefit and increased serious adverse events (164).

Steroids, whether administered orally or intravenously, have been found to be ineffective in treating Guillain-Barré syndrome (68; 06) and are not recommended in the acute stages of the disease. However, 500 milligrams of intravenous methylprednisolone given for five days along with five days of intravenous immunoglobulin was found to be more effective than intravenous immunoglobulin alone in an open study of 25 patients (07). A randomized, double-blind, placebo-controlled study with 233 individuals with Guillain-Barré syndrome compared therapy with intravenous immunoglobulin to intravenous immunoglobulin with 500 milligrams of intravenous methylprednisolone. There was no statistical difference in improvement between the two groups. However, a trend toward a decreased number of days until independent walking was noted in the steroid-treated group (average 28 days vs. 56 days). The authors stated further investigation using intravenous immunoglobulin with methylprednisolone would be warranted (165). Nevertheless, the 2023 EAN/PNS guidelines recommended against a combined use of IVIG and methylprednisolone due to a lack of efficacy of this combinational therapy (164).

Although eculizumab, an inhibitor of complement activation that prevents the formation of the membrane attack complex, was originally a promising emerging treatment for patients with Guillain-Barré syndrome, a phase III trial demonstrated that it did not improve motor function compared to placebo, and the 2023 EAN/PNS guidelines recommend against the use of eculizumab in Guillain-Barré syndrome (164; 85). Another complement inhibitor, ie, humanized antibody against the C1q component of complement (88), is being developed as an add-on therapy with intravenous immunoglobulin for the treatment of Guillain-Barré syndrome (NCT04035135).

An additional treatment approach relevant to Guillain-Barré syndrome pathogenesis is the antagonism of neonatal Fc receptor (FcRn). FcRn is central to IgG homeostasis and catabolism. FcRn antagonism shortens the half-life of IgG, including circulating pathogenic IgG antineural autoantibodies. FcRn antagonism can reduce antibody-mediated inflammatory nerve injury in experimental models of Guillain-Barré syndrome (191). The FcRn inhibitor efgartigimod has been shown in clinical trials to improve symptoms in both myasthenia gravis and chronic inflammatory demyelinating polyradiculoneuropathy, and it has been approved by the FDA for the treatment of myasthenia gravis (67; 01). Several case reports and series have since reported an improvement in recovery in patients with Guillain-Barré syndrome who have received efgartigimod, and a phase II clinical trial studying the efficacy and safety of efgartigimod in Guillain-Barré syndrome is underway (NCT05701189) (193; 196; 30). Given the ample clinicopathologic evidence of autoantibody-mediated nerve injury in Guillain-Barré syndrome, particularly its axonal variants, the use of FcRn inhibitors may extend to this group of disorders.

In addition to intravenous immunoglobulin or plasmapheresis, supportive therapy should be instituted immediately. Patients with oropharyngeal weakness with an inability to protect their airway or a vital capacity of less than 15 milliliters per kilogram should be considered for elective endotracheal intubation. These patients, as well as those with autonomic instability, should also be monitored in the intensive care unit. Prevention of infection, such as pneumonia or urinary tract infection, should be utilized. Deep venous thrombosis and pulmonary embolism prophylaxis, in addition to adequate nutrition by nasogastric or gastrostomy tube, should be addressed (134). Prevention of contractures and decubitus ulcers should be instituted early in immobilized patients with passive range-of-motion exercises and frequent repositioning. Rehabilitative measures such as physiotherapy should be started during the acute phase of the disease and continued afterwards because late recovery can occur (164). Strenuous exercise may cause paradoxical weakness, so therapy should be aimed at improving overall function and introducing strengthening exercises slowly (19). Orthotics should be used for optimal positioning and strength. In addition, complications from long-term illness, such as hypercalcemia of immobilization and anemia, should be monitored (104).

Outcomes

There are two patterns of recovery in patients with acute motor axonal neuropathy. The first is a rapid recovery after plasmapheresis or intravenous immunoglobulin, which may be related to reversible immune-mediated conduction failure at the nodes of Ranvier or in motor fibers. The second pattern is a slower, more likely incomplete recovery, which suggests Wallerian-like degeneration in motor axons (64; 84). Studies suggest that at 1 month after hospital admission, 25% of patients with acute axonal motor neuropathy can walk independently, which increases to 84% at 6 months after hospital admission, and 95% at 1 year after admission. In one study, all patients had achieved independent walking by 5 years (62; 150).

Special considerations

Pregnancy

According to national registries in Sweden, Guillain-Barré syndrome risk is lower during pregnancy and increases postpartum (76). There were a few case reports of intravenous immunoglobulin treatment for Guillain-Barré syndrome during 23 to 33 weeks of gestation. Both women responded to treatment and delivered healthy infants (140). In a study, Sharma and colleagues performed a retrospective observational analysis to find a correlation between pregnancy and Guillain-Barré syndrome. They estimated the incidence of Guillain-Barré syndrome in pregnancy in their cohort was between 1.2 and 1.9 cases per 100,000. Their study indicated that the risk of Guillain-Barré syndrome increases in the third trimester and in the first two weeks after delivery. They analyzed 47 patients with pregnancy and Guillain-Barré syndrome and suggested that early diagnosis and prompt intensive supportive care in these cases can improve the prognosis for both the mother and fetus (142). Improvement after plasmapheresis has also been reported (70), but no known clinical trials comparing intravenous immunoglobulin and plasmapheresis exist in pregnancy.

Anesthesia

Specific case reports concerning anesthesia and acute motor axonal neuropathy are unknown to these authors. Perel and colleagues give several recommendations if anesthesia is needed for patients with Guillain-Barré syndrome. Because of decreased sympathetic activity from autonomic instability, they suggest continuous monitoring of the electrocardiogram, blood pressure, and central venous pressure even for minor procedures. Rapid changes in upright position should be avoided, and even minor changes in position should be performed carefully. Medications such as barbiturates and phenothiazines should not be administered, given previous reports of circulatory collapse. Caution should be used with positive pressure ventilation because significant peripheral pooling without reflex venous constriction may occur. They also recommend using sympathomimetic agents if anesthetic agents are needed. Even when low-spinal or epidural analgesia is needed, volume expansion with intravenous fluids should be used and hypotension treated promptly (123). Feldman reported a specific case of cardiac arrest after succinylcholine administration for a Cesarean section in a patient one month after recovery from Guillain-Barré syndrome. Arterial blood showed severe hyperkalemia. Because cholinergic receptors proliferate at extraneuromuscular junction sites after neurologic injury, neuromuscular blockade can increase serum potassium when there is a large amount of muscle involvement (42).

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Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Author

Francesco Michelassi MD PhD

Dr. Michelassi of Colmbia University Irving Medical Center has no relevant financial relationships to disclose.
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Editor

Louis H Weimer MD

Dr. Weimer of Columbia University received a consultant honorarium from Roche and Ovid Therapeutics.
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Former Authors

Tonya DiTrapani-Stephenson MD
Yi Pan MD PhD
Duaa Jabari MD
Pedro Mancias MD
Sameera Salman Ghauri MBBS
Parveen Athar MD
Suur Biliciler MD
Billie Hsieh MD
Ali Reza Shoraka MD
Martin Svacina MD
Alina Sprenger-Svacina MD
Thy Nguyen MD
Kazim Sheikh MD

Patient Profile

Age range of presentation: 1 month to 65+ years
Sex preponderance: Some reports male>female, >2:1

Some reports male=female
Heredity: none
Population groups selectively affected: Chinese population showed predominately younger patients from rural areas.
Occupation groups selectively affected: none

ICD & OMIM codes

ICD-10: Guillain-Barré syndrome: G61.0

Polyneuropathy, unspecified: G62.9
ICD-11: Acute inflammatory demyelinating polyneuropathy: 8C01.0

Polyneuropathy, unspecified: 8C0Z

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Acute motor axonal neuropathy

Associated Disorders

Campylobacter jejuni infection
Guillain-Barré syndrome
Haemophilus influenzae infection

Differential Diagnosis

acute myelopathy
spinal cord compression
infarction
infection
inflammatory-like transverse myelitis
- neuromuscular junction abnormality
- myasthenia gravis
- botulism
- West Nile virus infection
- poliomyelitis
- Lyme disease
- diphtheria
- tick paralysis
- toxin exposure (heavy metals and organophosphates)
- acute porphyria

Also Relevant

Acute inflammatory demyelinating polyradiculoneuropathy
Autoantibodies: disease markers
Clinical evaluation of peripheral neuropathies
Efgartigimod alfa-fcab
Efgartigimod alfa and hyaluronidase-qvfc
- Guillain-Barre syndrome in children
- Motor and multifocal motor neuropathies
- Zika virus: neurologic complications

Clinical Categories

Peripheral Neuropathies

Web References

Guidelines

AAN: Immunotherapy for Guillain-Barre syndrome

Acute motor axonal neuropathy

Cite this article

Introduction

Overview

Key points

Historical note and terminology

Table 1. Classification of Guillain-Barré syndrome

Clinical manifestations

Presentation and course

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Confusing conditions

Associated or underlying disorders

Diagnostic workup

Table 2. Electrophysiological Separation of Guillain-Barré Syndrome Variants

Management

Outcomes

Special considerations

Pregnancy

Anesthesia

Media

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

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Questions or Comment?

Also in This Category

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Toxic peripheral neuropathies

Organophosphate neuropathy

Diphtheritic neuropathy

Efgartigimod alfa and hyaluronidase-qvfc

Acrylamide neuropathy

Whipple disease

Neurolymphomatosis

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