Acute Motor Axonal Neuropathy Patients Research Articles

Sural Sparing Pattern and Sensory Ratio as Electrodiagnostic and Prognostic Markers in Pediatric Guillain-Barré Syndrome.

We aimed to evaluate the presence of sural sparing pattern (SSP) and sensory ratio in pediatric Guillain-Barré syndrome (GBS), their distribution to subtypes, and their relationship with demographic and clinical features with a focus on the disability and muscle strength. This single-center retrospective study was conducted on pediatric GBS patients of both sexes with 2 years follow-up and two nerve conduction studies in which SSP and sensory ratio were calculated. Three subgroups of SSP were formed by separate calculation of median (SSP-m) and ulnar (SSP-u) and both median and ulnar sensory nerve action potentials (SNAPs; SSP-total). Muscle strength and disability were evaluated with the Medical Research Council (MRC) sum score and Hughes functional grading scale (HFGS), respectively. SSP total was identified in 70.6% (n: 24) of the patients, while sensory ratio >1 was observed in 20 (66.7%) patients. Patients with SSP-m, SSP-u, SSP-total, or sensory ratio >1 had higher HFGS scores, while patients with SSP-m, SSP-u, or SSP-total had lower MRC sum scores. SSP parameters were significantly associated with muscle strength and disability scores in acute motor axonal neuropathy patients. Both SSP and sensory ratio can be used for diagnostic and prognostic purposes. Disability and muscle strength are associated with SSP and sensory ratio in pediatric GBS.

Guillain-Barré Syndrome in Northern China: A Retrospective Analysis of 294 Patients from 2015 to 2020.

Acute motor axonal neuropathy (AMAN) was first reported to be the main subtype of Guillain-Barré syndrome (GBS) in northern China in the 1990s. About 30 years has passed, and it is unknown whether the disease spectrum has changed over time in northern China. We aimed to study the epidemiological, clinical, and electrophysiological features of GBS in northern China in recent years. We retrospectively analyzed the medical records of GBS patients admitted to the Second Hospital of Hebei Medical University in northern China from 2015 to 2020. A total of 294 patients with GBS were enrolled, with median age 53 years and 60.5% of participants being male, and a high incidence in summer and autumn. AMAN was still the predominant subtype in northern China (40.1%). The AMAN patients had shorter time to nadir, longer hospitalization time, and a more severe HFGS score at discharge than acute inflammatory demyelinating polyneuropathies (AIDP) (p < 0.05). With SPSS multivariable logistic regression analysis, we found the GBS disability score (at admission), dysphagia, and dysautonomia were independent risk factors for GBS patients requiring MV (p < 0.05). In comparison with other regions, the proportion of AMAN in northern China (40.1%) was higher than in eastern (35%) and southern (19%) China. AMAN is still the predominant subtype in northern China after 30 years, but there have been changes over time in the GBS spectrum since the 1990s. There are regional differences in GBS in China.

Elevated monocyte to high-density lipoprotein cholesterol ratio correlates with clinical severity in acute inflammatory demyelinating polyradiculoneuropathy patients.

While monocyte to high-density lipoprotein cholesterol ratio (MHR) has been reported to be associated with nervous system lesions, the role of MHR has not been determined in patients with Guillain-Barré Syndrome (GBS). The purpose of our study was to explore the role of MHR in patients with GBS. A total of 52 GBS patients were involved in the study retrospectively, including patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). We used Hughes Functional Grading Scale (HFGS) score to evaluate functional status in GBS patients. Among patients with different subtypes of GBS, MHR was significantly elevated in those with demyelination compared to patients without demyelination (p < 0.001); AIDP patients had an increased MHR compared with AMAN or AMSAN patients (p = 0.001; p = 0.013). There was a positive correlation between MHR and HFGS score (r = 0.463, p = 0.006) in AIDP patients, but not in AMAN or AMSAN. Multiple linear regression analysis revealed that MHR was independently associated with HFGS score (beta = 0.405, p = 0.013) in AIDP patients. Our study suggests that MHR as an inflammatory marker is elevated in patients with AIDP compared to AMAN or AMSAN patients, while MHR has a positive correlation with clinical severity in AIDP patients, suggesting that MHR may provide an additional information to reflect the pathophysiology of AIDP.

Collaborative studies of U.S.-China neurologists on acute motor axonal neuropathy.

Collaborative studies of U.S.-China neurologists on acute motor axonal neuropathy.

Clinical and Electrophysiological Features of Chronic Motor Axonal Neuropathy.

To determine the clinical and electrophysiological characteristics of chronic motor axonal neuropathy (CMAN) and identify the associated similarities and differences between CMAN, acute motor axonal neuropathy (AMAN), and motor neuropathy secondary to amyotrophic lateral sclerosis. The study described clinical and electrophysiological features of five patients with CMAN and compared with 20 AMAN patients, 42 amyotrophic lateral sclerosis patients and 41 healthy controls. To compare the distribution of different nerve involvement in the same limb, split ratio was introduced. Split ratio of upper limb = amplitude of compound muscle action potential abductor pollicis brevis (APB)/amplitude of compound muscle action potential abductor digiti minimi, and split ratio of lower limb = amplitude of compound muscle action potential extensor digitorum brevis/amplitude of compound muscle action potential abductor hallucis. Chronic motor axonal neuropathy patients manifested lower motor neuron syndrome with positive IgG anti-monosialoganglioside antibodies and good outcome. The CMAN patients shared similar clinical manifestation with AMAN patients except for disease course and higher Medical Research Council scores. Compared with healthy controls, the split ratio of lower limb was higher in both CMAN and AMAN, despite comparable split ratio of upper limb. There was significant difference between CMAN group and amyotrophic lateral sclerosis group in nerve involvement presented as split hand and split leg signs in amyotrophic lateral sclerosis and reverse split leg sign in CMAN. Chronic motor axonal neuropathy associated with monosialoganglioside might be a "mild" AMAN with chronic onset by similar clinical and electrophysiological features. There was a unique pattern of nerve involvement presenting as reverse split leg sign in both CMAN and AMAN.

Clinical Characteristics and Electrodiagnostic Features of Guillain-Barré Syndrome Among the Pediatric Population.

Guillain-Barré syndrome is an immune-mediated peripheral neuropathy characterized by different clinical manifestations. We aimed to describe the clinical features, seasonal distribution, subtypes, and electrodiagnostic characteristics of Iranian children with Guillain-Barré syndrome. In this prospective study, a total of 30 children with Guillain-Barré syndrome were evaluated. All demographic features were collected and electrodiagnostic study was assessed. Twelve participants were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy and 18 patients were identified with acute motor axonal neuropathy. The initial findings showed that a significant number of patients (23 cases, P = .003) resided in rural areas. Our results showed a higher incidence of Guillain-Barré syndrome in summer and autumn months. No significant difference was observed between the seasonal distribution of acute inflammatory demyelinating polyradiculoneuropathy and acute motor axonal neuropathy subtypes. Antecedent history of pulmonary infections was recorded in 14 children with Guillain-Barré syndrome. Electrophysiological findings revealed a pattern of prolonged F wave latency with reduced persistency, absence of sensory nerve action potential, reduced compound muscle action potential amplitude, prolonged distal motor latency, reduced nerve conduction velocity, and abnormal temporal dispersion or conduction block in most patients with acute inflammatory demyelinating polyradiculoneuropathy. However, reduced compound muscle action potential amplitude, F wave with normal latency and reduced persistency, normal sensory nerve action potential amplitude, normal distal latency, normal sensory nerve conduction velocity, and conduction block or temporal dispersion were observed in most acute motor axonal neuropathy patients. The data support a correlation between Guillain-Barré syndrome incidence with seasonal variation and living area. Further studies should assess the Guillain-Barré syndrome features in pediatric population.

Electrophysiological Subtypes and Prognostic Factors of Guillain-Barre Syndrome in Northern China

Objectives: To assess the epidemiology of different electrophysiological subtypes of Guillain-Barre syndrome (GBS) and investigate the factors affecting the prognosis of the acute motor axonal neuropathy (AMAN) subtype in northern China.Methods: According to the National Institute of Neurological Disorders and Stroke diagnostic criteria for GBS, 104 consecutive GBS patients were recruited from the Department of Neurology of the Second Hospital of Hebei Medical University, China from 2014 to 2018.Results: Based on nerve conduction studies (NCSs), AMAN was the most common subtype in Northern China, accounting for 58 patients (55.8%). AMAN patients had significantly higher prevalence of antecedent diarrhea, longer duration of hospitalization, and slightly slower recovery than those with acute inflammatory demyelinating polyneuropathy (AIDP), but there was no statistical difference in disease severity or short-term prognosis between AMAN and AIDP. Based on multivariate regression analysis, AMAN patients with antecedent diarrhea (OR = 0.16, 95% CI: 0.03–0.756, p = 0.021) or conduction blocks (CBs) (OR = 0.033, 95% CI: 0.001–0.787, p = 0.035) had dramatically better short-term prognosis. Decreased compound action potential with distal stimulation (dCMAP) amplitude was associated with significant slower speed of recovery(OR = 8.31, 95% CI: 2.55–27.10, p = 0.02).Conclusion: AMAN is still the most common subtype of GBS in northern China. A decline in dCMAP amplitude is predictive factor of a slow recovery and poor outcome of GBS. Diarrhea and CBs may be the factors for better short-term prognosis in AMAN patients in Northern China.

Guillain-Barré syndrome in a local area in Japan, 2006-2015: an epidemiological and clinical study of 108 patients.

Many epidemiological studies of Guillain-Barré syndrome (GBS) and Fisher syndrome (FS) have been conducted in Europe and America. In contrast, epidemiological studies are rare in Asia where the GBS subtypes differ from those in Western countries. This study was undertaken to clarify the incidence of GBS and FS in a local area in Japan as well as their seasonal trends. Seventy-one GBS and 37 FS patients were recorded from 2006 to 2015 in an area of approximately 1.5 million inhabitants in Japan. The incidence, seasonal trends and clinical features of GBS and FS were examined. The incidence rate of GBS was 0.42 cases per 100000 person-years and that of FS was 0.22 cases per 100000 person-years. The incidence of GBS increased with age and FS affected predominantly patients aged from 45 to 64years old. There was some seasonal clustering of acute motor axonal neuropathy (AMAN) and FS in spring and summer, but it was not significant. AMAN and FS patients had a high frequency of preceding infection (AMAN, 68% gastrointestinal infection; FS, 65% upper respiratory infection). Antecedent respiratory infection was significantly associated with FS as an outcome. Serum antibodies to ganglioside GM1 were detected in 71% of AMAN patients and antibodies to GQ1b were detected in 81% of FS patients. Our study offers evidence of a lower incidence of GBS and a higher incidence of FS in a local area in Japan than in Western countries.

Anti-ganglioside antibodies in Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy in Chinese patients.

In this study we investigated the relationships between anti-ganglioside antibodies and Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP). Samples from 48 Chinese patients diagnosed with GBS and 18 patients diagnosed with CIDP were retrospectively reviewed. In the GBS patients, 62.5% were classified as having acute inflammatory demyelinating polyneuropathy (AIDP), 27.1% were found to have acute motor axonal neuropathy (AMAN), and 10.4% were unclassified. Serum IgG anti-ganglioside antibodies were detected in 46.2% of the AMAN patients and in 6.7% of the AIDP patients (P < 0.05); 5.6% of the 18 CIDP patients were IgG antibody positive, and 27.8% were IgM antibody positive. Facial palsy and sensory impairment were significantly associated with IgM antibodies. These results suggest that IgG anti-GM1 antibodies are associated with AMAN, but not with AIDP, and that IgM antibodies against GM1, GM2, and GM3 are associated with facial nerve palsy. Muscle Nerve 55: 470-475, 2017.

Electrophysiological subtypes and long term prognosis of Guillain-Barré syndrome

To assess the electrophysiological subtypes of Guillain-Barré syndrome (GBS) acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN), and long-term prognosis in northeastern China. One hundred and eighteen patients with GBS were recruited between 2010 and 2012 and retrospectively reviewed. According to electrodiagnostic criteria, patients were classified as AMAN (49, 46.7%) or AIDP (39, 37.1%), or were unclassified (17, 16.2%). Between the AMAN and AIDP groups, age, sex, and clinical disability did not differ significantly, but the AMAN patients more frequently had preceding gastroenteritis. By 4 weeks after onset, 24.5% of the AMAN patients (12) and 33.3% of the AIDP patients (13) had regained the ability to walk; by 1 year, 77.6% of the AMAN patients (38) and 79.5% of the AIDP patients (31) could walk. Recovery was generally favorable in both subtypes. Immunotherapy was effective in patients with either AIDP or AMAN, and glucocorticoids and immunoglobulin treatment could achieve similar effect. AMAN and AIDP are the main subtypes of GBS in northeastern China. The prognosis of patients with AMAN is similar to that of patients with AIDP. Different immunotherapies have similar effect on long term prognosis.

More severe manifestations and poorer short-term prognosis of ganglioside-associated Guillain-Barré syndrome in Northeast China.

Ganglioside as a neurotrophic drug has been hitherto widely used in China, although Guillain-Barré syndrome (GBS) following intravenous ganglioside treatment was reported in Europe several decades ago. We identified 7 patients who developed GBS after intravenous use of gangliosides (ganglioside+ group) and compared their clinical data with those of 77 non-ganglioside-associated GBS patients (ganglioside− group) in 2013, aiming at gaining the distinct features of ganglioside-associated GBS. Although the mean age, protein levels in cerebrospinal fluid (CSF) and frequency of cranial nerve involvement were similar between the two groups, the Hughes Functional Grading Scale (HFGS) score and the Medical Research Council (MRC) sum score at nadir significantly differed (4.9±0.4 vs 3.6±1.0; 7.7±5.5 vs 36.9±14.5, both p<0.001), indicating a higher disease severity of ganglioside-associated GBS. A higher ratio of patients with ganglioside-associated GBS required mechanical ventilation (85.7% vs 15.6%, p<0.01). The short-term prognosis of ganglioside-associated GBS, as measured by the HFGS score and the MRC sum score at discharge, was poorer (4.3±0.5 vs 2.8±1.1; 17.3±12.9 vs 46.0±13.9, both p<0.001). All the patients in the ganglioside+ group presented an axonal form of GBS, namely acute motor axonal neuropathy (AMAN). When compared with the AMAN patients in the ganglioside− group, more severe functional deficits at nadir and poorer recovery after standard treatment were still prominent in ganglioside-associated GBS. Anti-GM1 and anti-GT1a antibodies were detectable in patients with AMAN while not in patients with the demyelinating subtype of GBS. The concentrations of these antibodies in patients with AMAN were insignificantly different between the ganglioside+ and ganglioside− groups. In sum, ganglioside-associated GBS may be a devastating side effect of intravenous use of gangliosides, which usually manifests a more severe clinical course and poorer outcome.

Clinical and electrophysiological features of Guillain–Barré syndrome in Iran

We evaluated the clinical and electrophysiological characteristics of 121 consecutive patients admitted with Guillain–Barré syndrome (GBS) to a tertiary referral hospital in Tehran, Iran, from 1997 to 2007. The mean age of patients was 38.9 (standard deviation 19.7) years. The predominant subtype of GBS was the demyelinating form. Miller Fisher syndrome was present in 3.3% of patients. There was no significant seasonal clustering among the three subtypes, but axonal variants tend to occur in summer. In contrast with other subtypes, the majority of patients with acute motor-sensory axonal neuropathy (AMSAN) were female (72.3%). AMSAN patients also had significantly longer hospitalization time (p=0.002) and intensive care unit (ICU) admission (p=0.017), while none of the acute motor axonal neuropathy patients needed ICU admission. Involvement of cranial nerves and symmetry of signs were significantly detected in the demyelinating variant (p=0.021 and p=0.040, respectively). The overall mortality was 3.3%.

A multicentre prospective study of Guillain-Barré Syndrome in Japan: a focus on the incidence of subtypes

ObjectiveGuillain–Barré Syndrome (GBS) is classified into the two major subtypes; acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Previous studies have suggested that AIDP is predominant and...

Electrophysiological subtypes and prognosis of Guillain–Barré syndrome in Northeastern China

The purpose of this study was to assess the electrophysiological subtypes and prognosis of Guillain-Barré syndrome (GBS) in northeastern China. Ninety-nine patients with GBS were recruited between 2006 and 2010 and retrospectively reviewed. Sixty-seven percent of patients had acute inflammatory demyelinating polyneuropathy (AIDP). Patients with acute motor axonal neuropathy (AMAN) had more severe symptoms at onset of GBS, and intravenous immunoglobulin (IVIg) was less effective in these patients. The prognosis may have been associated with the severity of the illness and did not differ between AMAN and AIDP patients. Abnormal motor nerve conduction studies (NCS) of the lower limbs and sensory NCS of the upper limbs with normal sural sensory nerve studies were the main electrophysiological features of AIDP. AIDP is the main subtype of GBS, and it has specific electrophysiological characteristics in northeastern China. The prognosis of patients with AMAN was similar to that of patients with AIDP. Moreover, IVIg was more effective in patients with AIDP.

Cav2.1 Voltage-dependent Ca2+ Channel Current is Inhibited by Serum from Select Patients with Guillain-Barré Syndrome

To investigate the pathophysiological mechanisms of immune-mediated peripheral neuropathies, we studied the effects of sera from patients with Guillain-Barré syndrome (GBS) on the Cav2.1 voltage-dependent calcium channel (VDCC) current in Purkinje cells. Using the whole-cell recording technique, Cav2.1 VDCC current was measured in cerebellar Purkinje cells in the presence of serum from GBS patients with acute motor axonal neuropathy (AMAN) or acute inflammatory demyelinating polyneuropathy (AIDP). The AMAN patient sera significantly inhibited the Cav2.1 VDCC current compared with healthy volunteer sera, and this inhibition was fully reversible by washing out the AMAN serum. Similarly, IgG purified from AMAN sera also inhibited the Cav2.1 VDCC current. However, the activation and inactivation kinetics of the Cav2.1 VDCC currents were not affected by serum from an AMAN patient. Moreover, the VDCC current of Purkinje cells was also inhibited by IgG anti-GM1 monoclonal antibody (anti-GM1 mAb). In an immunocytochemical study using double fluorescence staining, Purkinje cells were stained by monoclonal IgG anti-GM1 mAb. In contrast, AIDP patient and healthy volunteer sera did not affect the Cav2.1 VDCC current. These results suggest that in some case of GBS, particularly of AMAN patients with IgG anti-GM1 mAb, muscle weakness may be induced by dysfunction of Cav2.1 VDCC functioning at the motor nerve terminals.

Recovery patterns and long term prognosis for axonal Guillain–Barré syndrome

Background: Little is known about the long term prognosis for patients the severe acute motor axonal neuropathy (AMAN) form of Guillain–Barré syndrome (GBS), unlike those with acute inflammatory demyelinating neuropathy...

Sensory Nerve Conduction in Demyelinating and Axonal Guillain-Barré Syndromes

Guillain-Barré syndrome is divided into acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) based on motor nerve conduction studies. We investigated whether sensory nerve conduction studies contribute to the electrodiagnosis of AIDP and AMAN. In consecutive 59 patients with AIDP (n = 26) or AMAN (n = 33), results of sensory nerve conduction studies in the median, ulnar and sural nerves were reviewed. Sensory nerve conduction abnormalities were found for 85% of AIDP patients and for only 6% of AMAN patients. In AIDP patients, the abnormalities were present in 85% of patients in the median nerves, 85% in the ulnar nerves and 38% in the sural nerves. AMAN is very rarely associated with sensory nerve involvement. Abnormal sensory nerve conduction is supportive of AIDP and is more frequently found for the median and ulnar nerves than sural nerves.

Differences in membrane properties of axonal and demyelinating Guillain-Barré syndromes.

Guillain-Barré syndrome is classified into acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyneuropathy (AIDP) by electrodiagnostic and pathological criteria. In AMAN, the immune attack appears directed against the axolemma and nodes of Ranvier. Threshold tracking was used to measure indices of axonal excitability (refractoriness, supernormality, and threshold electrotonus) for median nerve axons at the wrist of patients with AMAN (n = 10) and AIDP (n = 8). Refractoriness (the increase in threshold current during the relative refractory period) was greatly increased in AMAN patients, but the abruptness of the threshold increases at short interstimulus intervals indicated conduction failure distal to the stimulation (ie, an increased refractory period of transmission). During the 4 week period from onset, the high refractoriness returned toward normal, and the amplitude of the compound muscle action potential increased, consistent with improvement in the safety margin for impulse transmission in the distal nerve. In contrast, refractoriness was normal in AIDP, even though there was marked prolongation of distal latencies. Supernormality and threshold electrotonus were normal in both groups of patients, suggesting that, at the wrist, membrane potential was normal and pathology was relatively minor. These results support the view that the predominantly distal targets of immune attack are different for AMAN and AIDP. Possible mechanisms for the reduced safety factor in AMAN are discussed.

Physiologic-pathologic correlation in Guillain–Barré syndrome in children

To correlate electrophysiologic patterns with sural nerve pathology in children with Guillain-Barré syndrome (GBS). Based on electrophysiologic and pathologic observations, GBS has been divided into demyelinating and axonal subtypes. The acute motor axonal neuropathy (AMAN) involves predominantly motor nerve fibers with a physiologic pattern suggesting axonal damage, whereas the acute inflammatory demyelinating polyneuropathy (AIDP) involves both motor and sensory nerve fibers with a physiologic pattern suggesting demyelination. In this study, we sought to confirm these observations by correlating sural nerve pathology with electrophysiologic findings in GBS patients. Biopsies of sural nerve from 29 of 50 prospectively studied GBS patients were obtained. Nerves were examined by light and electron microscopy, and with immunocytochemistry for macrophages, lymphocytes, and complement activation products. Sural nerves from AMAN patients were normal or had only a few (0.1% to 0.7%) degenerating fibers without lymphocytic infiltration or complement activation. One patient with reduced sural sensory nerve action potential classified as acute motor sensory axonal neuropathy (AMSAN) had many degenerating fibers (2.3%) in the sural nerve. All three AIDP patients displayed active demyelination, and in two patients, lymphocytic infiltration and complement activation products were observed on the abaxonal Schwann cell surface. Classification of Guillain-Barré syndrome subtypes based on motor conduction studies correlates closely with pathologic changes seen in sural nerve. In acute motor axonal neuropathy cases, the sural nerve is almost completely spared pathologically. In acute inflammatory demyelinating polyneuropathy cases, macrophage-mediated demyelination and lymphocytic infiltration are common in the biopsies of sural nerves.

Acute motor axonal neuropathy and acute motor-sensory axonal neuropathy share a common immunological profile.

Griffin and colleagues (Griffin JW, Li CY, Ho TW, Tian M, Gao CY, Xue P, Mishu B, Cornblath DR, Macko C, McKhann GM, Asbury AK. Pathology of motor-sensory axonal Guillain-Barré syndrome. Ann Neurol 1996;39:17-28 [4]) proposed that acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) are part of the spectrum of a single type of immune attack on the axon. In contrast, IgG anti-GM1 antibody is associated closely with AMAN, but whether other IgG anti-ganglioside antibodies are associated with this neuropathy is not clear. We investigated whether IgG anti-ganglioside antibodies can be used as immunological markers to differentiate AMAN from acute inflammatory demyelinating polyneuropathy (AIDP) and whether these autoantibodies are present in AMSAN. The frequencies of anti-GM1, anti-GM1b, and anti-GD1a IgG antibodies in 21 AMAN patients were significantly higher than in 19 AIDP patients. Anti-GM1b and anti-GD1a IgG, as well as anti-GM1 IgG antibodies, therefore are immunological markers for AMAN. The patients with AMSAN had anti-GM1, anti-GM1b, and anti-GD1a IgG antibodies, indicative that AMAN and AMSAN share a common immunological profile.