Acute Motor Axonal Neuropathy Subtype Research Articles

Clinical characteristics and functional outcomes of pediatric Guillain-Barré syndrome admitted to the Neuro-intensive care unit: a decade-long retrospective observational study.

Guillain-Barré Syndrome (GBS) remains a significant contributor to acute flaccid paralysis in pediatric patients worldwide. Despite its impact, studies focusing on pediatric GBS requiring intensive care unit (ICU) management are limited. This study aimed to address this gap by exploring the clinical and outcome characteristics of pediatric GBS necessitating ICU care. This retrospective observational study, spanning a decade, analyzed the records of 75 pediatric GBS patients admitted to the Neuro-ICU of a tertiary care center in South India. Data included demographics, prodromal symptoms, clinical features, investigations, treatment modalities, and outcomes. The majority (55/75) of patients were male, with a median age of 12 years. The highest incidence of GBS requiring ICU admission was in the monsoon season. Prodromal symptoms were observed in 56%. Most patients (93.33%) presented with typical GBS symptoms, and 40% had respiratory distress on ICU admission. Acute motor axonal neuropathy (AMAN) was the most common subtype. Approximately 80% required mechanical ventilation, with a median duration of 22.5 days. No in-hospital mortality was recorded. At discharge, most patients had a GBS disability score of 4, improving to 2 at a median follow -up of 228 days. Pediatric GBS patients requiring ICU care exhibit distinctive characteristics, including a higher prevalence of AMAN subtype, seasonal clustering, and favorable outcomes with intensive treatment. The absence of in-hospital mortality underscores the effectiveness of prompt ICU admission and dedicated Neuro-intensive care.

Complete genome sequence of Edwardsiella tarda strain GBS0709 isolated from a Japanese patient with the acute motor axonal neuropathy subtype of Guillain-Barré syndrome.

The complete genome sequence of Edwardsiella tarda strain GBS0709, isolated from an 81-year-old Japanese patient with the acute motor axonal neuropathy subtype of Guillain-Barré syndrome, was determined. It comprised a 3,632,068 bp circular chromosome and a 5,386 bp plasmid. The overall guanine and cytosine content was 57.3%.

Therapeutic Plasma Exchange in a Patient With Acute Motor Axonal Neuropathy Subtype of Guillain-Barre Syndrome and Systemic Lupus Erythematosus

Objective To highlight treatment resistance of concurrent Guillain-Barre syndrome (GBS) and active systemic lupus erythematosus (SLE). Background Coincidence of SLE and GBS is an uncommon and complicated course of autoimmune disease. Treatment is further complicated by pregnancy exacerbation of SLE. Design/Methods We report a case of acute motor axonal neuropathy (AMAN) subtype of GBS in a young pregnant woman in her early 20s presenting with SLE. Patient was 10 weeks pregnant at presentation. Lumbar puncture study and electrical muscle stimulation (EMS) were consistent with acute motor axonal neuropathy subtype of GBS. Patient also had increased proteinuria and renal biopsy performed that was consistent with lupus nephritis. Despite treatment with IVIG and pulse dose corticosteroids, the patient had minimal neurological improvement with respiratory decline requiring intubation. Her pregnancy was terminated at this point and a course of therapeutic plasma exchange (TPE) was started followed by cyclophosphamide. Results The patient responded to the combination of therapy and had slow but gradual neurologic recovery as well as improvement of proteinuria. Conclusions Concurrent GBS and active SLE in the setting of pregnancy may be more treatment resistant, and combination therapy including TPE, immunosuppression, and termination of pregnancy may be indicated. For patients with concurrent GBS and SLE, especially axonal subtype of GBS and during pregnancy, TPE should be considered as a primary treatment option with respect to both efficacy and safety.

Outcome of Guillain-Barré syndrome following intravenous immunoglobulin compared to natural course.

Intravenous immunoglobulin (IVIg) is recommended in Guillain-Barré syndrome (GBS), but its efficacy may vary in different subtypes. We report the outcomes of patients with GBS following IVIg treatment compared to the natural course (NC). We also compare the effect of IVIg treatment in different subtypes of GBS. From a cohort of 528 GBS subjects, we have extracted 189 patients who received IVIg and compared their outcomes with 199 age- and peak disability-matched patients who did not receive IVIg, plasmapheresis, or corticosteroid. Disability was assessed using the 0-6 Guillain-Barré Syndrome Disability Scale (GBSDS). Clinical and neurophysiological subtypes were recorded. The primary outcome was functional disability at 6 months, which was categorized as complete (GBSDS ≤ 1), partial (GBSDS 2-3), or poor (GBSDS > 3). The secondary outcomes were in-hospital death, duration of hospitalization, and mechanical ventilation. In-hospital death (2.6% vs. 2%, p=0.74) and 3-month poor recovery (20.7% vs. 18%) were similar in the IVIg and NC groups. At 6 months, however, a lesser proportion of patients in the IVIg group had poor recovery (2.2% vs. 8.3%, p=0.026). The outcomes of IVIg and NC were compared in 72 acute motor axonal neuropathy (AMAN) and 256 acute inflammatory demyelinating polyradiculoneuropathy (AIDP) patients. IVIg therapy did not alter the outcome in AMAN but resulted in a lesser proportion of poor recovery at 6 months in AIDP (0.8% vs. 6.6%, p=0.03). IVIg is beneficial in AIDP variants of GBS but not in the AMAN subtype. A customized treatment may be cost-effective until a randomized controlled trial is conducted in AMAN.

Acute Motor Axonal Neuropathy Accompanied with Delayed Facial Diplegia

Facial nerve palsy is one of major accompanying features in Guillain-Barré syndrome (GBS). In most of the cases, facial weakness develops simultaneously with other symptoms such as motor weakness, sensory change and other cranial neuropathies. However, facial palsy also occurs after the nadir of neurological deficits or even after the beginning of limb weakness improvement, called delayed facial palsy (DFP). DFP has been reported in Miller Fisher syndrome, but it rarely found from the acute motor axonal neuropathy subtype of GBS. Recently, we experienced a patient who diagnosed acute motor axonal neuropathy accompanying with delayed facial diplegia.

Guillain-Barr\xe9 syndrome triggered by surgery in a Chinese population: a multicenter retrospective study

BackgroundSurgery is a potential trigger of Guillain-Barré syndrome (GBS), a disorder which leads to an autoimmune-mediated attack of peripheral nerves. The present study was designed to explore clinical features of post-surgical GBS compared with those of general GBS in order to provide better clinical advice to patients undergoing surgery.MethodsThe medical records of GBS patients who were seen at 31 tertiary hospitals in southern China between January 1, 2013 and September 30, 2016 were retrospectively analyzed. Post-surgical GBS was defined as symptoms of GBS within 6 weeks after surgery. Clinical features of post-surgical GBS are described and are compared with general GBS.ResultsAmong the 1001 GBS patient cases examined in this study, 45 (4.5%) patient cases exhibited symptoms of GBS within 6 weeks of undergoing surgery. Within this group, 36 (80.0%) patients developed initial symptoms of limb weakness. The average interval between surgery and symptom onset was 13.31 days. The most common type of surgery which triggered GBS was orthopedic surgery, followed by neurological surgery. Compared to general GBS, post-surgical GBS was characterized by a higher proportion of severe patients (Hughes functional grading scale (HFGS) score ≥ 3) upon admission and at nadir, higher HFGS scores at discharge, and longer hospital stays. Post-surgical GBS patients also had a significantly higher frequency of the acute motor axonal neuropathy subtype (37.9 vs. 14.2, respectively; P = 0.001).ConclusionSurgery is probably a potential trigger factor for GBS, especially orthopedic surgery. Infections secondary to surgery may play a role. The possibility of preceding (post-operative) infections was not excluded in this study. Clinical presentation of post-surgical GBS is characterized by a more severe course and poorer prognosis, and should be closely monitored.Trial registrationchicTR-RRc-17,014,152.

How Different is AMAN from AIDP in Childhood GBS? A Prospective Study from North India.

To compare the clinical profile and short-term outcome of children with axonal and demyelinating subtypes of childhood Guillain Barré syndrome (GBS). This is a prospective observational study conducted in a tertiary care teaching hospital in North India. Consecutive children with Guillain Barré syndrome were recruited to compare the clinical profile and short term outcome among the subtypes. Among 9847 children admitted to the emergency, 95 had acute flaccid paralysis; 57 of whom had GBS. Electrophysiological studies were completed in 57; of whom 20 had acute inflammatory demyelinating polyneuropathy (AIDP); 19 had acute motor axonal neuropathy (AMAN); 12 had non-reactive nerves; five were unclassifiable; 1 had acute motor sensory axonal neuropathy (AMSAN). More children in AMAN group had preceding gastroenteritis (4 vs. 2), while AIDP group had upper respiratory infections (12 vs. 7). Ataxia was only seen in AIDP subtype while wrist drop, foot drop and hyperreflexia were seen only with AMAN subtype. Respiratory muscle involvement (6 vs. 3) and artificial ventilation (5 vs. 2) was more in AMAN. At discharge, children with AIDP were less likely to be non-ambulant (12 vs. 6, p = 0.036). Mean disability scores at hospital discharge (4.9 ± 1.2 vs. 4 ± 0.9, p = 0.015) and at last follow-up (0.7 ± 1.01 vs. 0.05 ± 0.2, p = 0.016) were higher in AMAN. Children with AIDP were more likely to achieve normalcy on follow-up (19 vs. 12, p = 0.023). Children with AMAN appear to have a more severe clinical course; higher short-term morbidity; and slower recovery than those with AIDP.

F30. Axonal variants of Guillain Barre Syndrome are predominant among nepalese population: A tertiary care center study

Introduction Guillain Barre Syndrome (GBS) is an acute, frequently severe, and fulminant polyradiculoneuropathy that is autoimmune in nature. Our aim is to classify GBS subtypes and evaluate the clinical severity. Methods All consecutive patients recruited prospectively in the study were of age ⩾ 16 years and were being admitted in department of Neurology of Tribhuvan University Teaching Hospital, Kathmandu, Nepal from 2016 March to 2017 February. All demographic, historical and clinical data were collected. Nerve conduction study, cerebrospinal fluid analysis along with clinical features were evaluated to assess the diagnostic certainty of Brighton Criteria. Overall disability sum score and GBS disability score were assessed in all patients. Results A total of 46 patients were included: male patients being predominant (70% vs 30%), mean age = 36.5 ± 16.2, range = 16–80 years. Thirty-two patients (70%) were axonal variant, acute motor axonal neuropathy being more common (18 patients), and 14 patients were acute motor and sensory axonal neuropathy. 14 patients (30%) were demyelinating type, out of which 11 patients had both motor and sensory features, and only 3 patients were pure motor demyelinating type. Axonal variants were found to have higher Overall disability sum score during nadir (p = 0.024) and discharge (p = 0.004), and also higher GBS disability score during nadir (p = 0.012) and discharge (p = 0.021). Acute motor axonal neuropathy (AMAN) had higher GBS disability score than acute inflammatory demyelinating polyneuropathy (AIDP) at nadir (p = 0.034) and discharge (p = 0.039). Conclusion AMAN subtype are predominant among Nepalese population. Axonal variants of GBS are clinically severe than demyelinating variants.

Antibodies to single glycolipids and glycolipid complexes in Guillain-Barré syndrome subtypes.

To comprehensively investigate the relationship between antibodies to single glycolipids and their complexes and Guillain-Barré syndrome subtypes and clinical features. In acute sera from 199 patients with Guillain-Barré syndrome, immunoglobulin G (IgG) antibodies to glycolipids and ganglioside complexes were tested using ELISA against individual antigens from single glycolipids including gangliosides (LM1, GM1, GM1b, GD1a, GalNAc-GD1a, GD1b, GT1a, GT1b, GQ1b) and a neutral glycolipid, asialo-GM1 (GA1), and antigens from the combination of 2 different glycolipids. Based on serial nerve conduction studies, the electrodiagnoses were as follows: 69 demyelinating subtype, 85 axonal subtypes, and 45 unclassified. Significant associations were detected between acute motor axonal neuropathy subtype and IgG antibodies to GM1, GalNAc-GD1a, GA1, or LM1/GA1 complex. Reversible conduction failure was significantly associated with IgG antibodies to GM1, GalNAc-GD1a, GD1b, or complex of LM1/GA1. No significant association was demonstrated between acute inflammatory demyelinating polyneuropathy and any of the glycolipids or ganglioside complexes. Anti-ganglioside complex antibodies alone were detected in 7 patients (5 axonal subtype). The current study demonstrates that antibodies to single glycolipids and ganglioside complexes are associated with acute motor axonal neuropathy or acute motor conduction block neuropathy but not acute inflammatory demyelinating polyneuropathy. This study provides Class II evidence that antibodies to glycolipids are increased in patients with acute motor axonal neuropathy and acute motor conduction block neuropathy but not acute inflammatory demyelinating polyneuropathy.

Childhood Guillain–Barré syndrome subtypes in northern India

This prospective cross-sectional study was conducted at a tertiary care research centre in North India to describe the frequency and clinical characteristics of subtypes of childhood Guillain–Barré syndrome. Among the 68 children enrolled, 65 were finally diagnosed with Guillain–Barré syndrome (median age, 60months); 45 (69%) were boys. The most common subtype was acute motor axonal neuropathy in 27 patients (41.5%, 95% confidence interval [CI] 29–54), followed by acute inflammatory demyelinating polyneuropathy in 15 (23%, 95% CI 13.5–35), and acute motor sensory axonal neuropathy in three (4.6%, 95% CI 1–13). Twelve patients (18.5%, 95% CI 10–30) had inexcitable nerves, and eight (12.4%, 95% CI 5.5–23) were unclassifiable. Those with acute inflammatory demyelinating polyneuropathy were more likely to have had a preceding upper respiratory tract infection. The acute motor axonal neuropathy subtype peaked in incidence during the winter and monsoon months.

Clinical, electrophysiological subtypes and antiganglioside antibodies in childhood Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) has been the most common cause of flaccid paralysis in children after the decline in the incidence of poliomyelitis. There are not any published data from the Indian subcontinent documenting electrophysiological patterns and antiganglioside antibodies in pediatric GBS. The study population included children with GBS referred for electrodiagnostic evaluation and also children with GBS admitted to our institute between August 2006 and July 2007. Nerve conduction studies were done to determine GBS subtypes and serum antiganglioside antibodies were measured using enzyme-linked immunosorbent assay (ELISA). Clinical and electrophysiological features were correlated with antiganglioside antibody results. Of the 43 (male to female ratio = 2.1:1) children studied, 97.6% had motor weakness, 76.7% had cranial nerve palsies, 13.9% had autonomic disturbances and respiratory paralysis was found in 9.3% children. Antecedent illness was recorded in 69.8% children. The GBS subtype distribution was as follows: acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 21 (48.8%), acute motor axonal neuropathy (AMAN) in 19 (44.2%), and 3 (6.9%) children were unclassified. The severity of illness was similar in both AMAN and AIDP subtypes and the recovery in both the subtypes was complete without any significant difference in the duration of recovery. Preceding diarrheal illness was more common in AMAN subtype as compared to AIDP subtype (57.9% vs. 4.7%, P = 0.007). Sensory symptoms were more common in AIDP subtype than in AMAN subtype (66.6% vs. 21%, P = 0.03}. The commonest ganglioside antibody was IgM GM2. Anti GM3 antibodies were exclusively seen in children with AMAN and IgG GD1b was significantly associated with (36.7 vs. 4%; P = 0.007) AMAN subtype. IgG GT1b was identified in 50% of patients with AIDP as compared to 22.7% in patients with AMAN. In this study, AMAN subtype accounted for a significant proportion of pediatric GBS. AMAN was associated with diarrhea and specific antiganglioside antibodies. Recovery in children with GBS was complete, irrespective of the subtype.

Association of TLR4 Asp299Gly and Thr399Ile polymorphisms with Guillain-Barré syndrome in Northern Indian population

Molecular mimicry between Campylobacter jejuni lipopolysaccharide and host gangliosides induces an immune response leading to axonal damage and Guillain-Barré syndrome (GBS). TLR polymorphisms are associated with many autoimmune diseases. The role of the TLR4 gene in GBS susceptibility largely remains unknown. We investigated TLR4 polymorphism in GBS. One hundred and twenty GBS patients and 150 healthy controls were included. TLR4 (Asp299Gly and Thr399Ile) genes were studied by PCR-RFLP. TLR4 (Asp299Gly) polymorphism was significantly associated with GBS ( p, 0.045; OR, 8.75; 95% CI, 1.05–72.88); only acute motor axonal neuropathy (AMAN) was associated with Gly299Gly homozygote ( p, 0.027; OR, 12.40; 95% CI, 1.33–115.77) and Thr399Ile ( p, 0.019; OR, 3.42; 95% CI, 1.22–9.54) heterozygote, and TLR4–399Ile allele ( p, 0.045; OR, 2.63; 95% CI, 1.02–6.75) compared to controls. In conclusion, TLR4 (Asp299Gly) polymorphism is associated with an increased susceptibility to GBS. Besides Asp299Gly, AMAN subtype is also associated with Thr399Ile polymorphism.

Rituximab-responsive Guillain–Barré syndrome following allogeneic hematopoietic SCT

Rituximab-responsive Guillain–Barré syndrome following allogeneic hematopoietic SCT