Acute Inflammatory Demyelinating Polyradiculoneuropathy Patients Research Articles

Comparison of ulnar, median, and sural sensory nerve conduction studies between demyelinating and axonal forms of Guillain-Barré Syndrome

Background: Guillain-Barré syndrome (GBS) is an important neurological disease that can cause disability. Axonal GBS and acute inflammatory demyelinating polyradiculoneuropathy (AIDP) are two important subgroups.
 Methods: Patients with clinical and electrodiagnostic features compatible with GBS were included in this retrospective study. The patients were divided into two groups neurophysiologically as Axonal GBS and AIDP. Medical research council (MRC) scores of the patients' muscles, median / ulnar / posterior tibial / peroneal / sural nerve conduction study findings were included in the analyses. Sural sparing pattern was considered as the absence of median/ulnar compound nerve action potential (CNAP) and present sural nerve CNAP (SS-M/SS-U).
 Results: Twelve AIDP and 10 Axonal GBS patients were included in the study. MRC scores were not different between the two groups (p=0.895). CNAPs of the right median and ulnar nerves were smaller in AIDP patients than in Axonal GBS patients (p

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.

A wave is a marker in demyelination subtype in children′s Guillain-Barré syndrome and it correlates with short-term prognosis

Objective To explore the A wave value in neuroelectrophysiological subtype of Guillain-Barre syndrome(GBS)and the clinical severity and short-term prognosis of acute inflammatory demyelinating polyradiculoneuropathy(AIDP). Methods From March 2014 to March 2017, a total of 56 children with GBS at Department of Neurology of Wuhan Children′s Hospital Affiliated to Tongji Medical College, Huazhong University of Science & Technology were enrolled.The patients were divided into AIDP subtype(40 cases) and axonal GBS subtype(16 cases) according to the results of electrophysiological examination.According to whether there was existence of A wave or not, the GBS children were divided into 2 groups.The first group was the A wave in GBS group(18 cases), and the second group was non-A wave in GBS group(38 cases). In order to explore classification value for GBS with A wave, clinical data including age, gender, history of prodromal infection, cranial nerve dysfunction, autonomic nerve involvement and conduction blocks were analyzed.To explore A wave value in clinical severity and short-term prognosis of AIDP, the age, gender, clinical severity, conduction blocks, short-term prognosis of the 2 groups were analyzed in A wave with AIDP (18 cases) and non-A wave with AIDP(22 cases). Results Compared with non-A wave GBS patients, A wave GBS patients had more conduction blocks(10 cases vs.2 cases, χ2=18.021, P=0.000). Age, sex, precedent infections, cranial nerve involvement, autonomic nerve involvement were not significantly statistically different(all P>0.05). A wave was only seen in AIDP subtype(18 cases), and the percentage of A wave in AIDP was 45%(18/40 cases). There was no A wave in axonal GBS.Compared with non-A wave in AIDP, A wave in AIDP patients had more conduction blocks(10 cases vs.2 cases, χ2=9.924, P=0.002), poorer clinical motor function[(3.39±1.09) scores vs.(2.50±1.01) scores, t=2.667, P=0.011]and short-term prognosis[(2.06±0.64) scores vs.(1.55±0.60) scores, t=2.607, P=0.013]. Conclusions A wave is correlated with demyelination subtype in children′s Guillain-Barre syndrome and poor short-term prognosis in AIDP. Key words: A wave; Child; Guillain-Barre syndrome

Clinical and nerve conduction features in Guillain−Barré syndrome associated with Zika virus infection in Cúcuta, Colombia

Zika virus (ZIKV) infection has been associated with an increased incidence of Guillain-Barré syndrome (GBS) but the relative frequency of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and axonal GBS subtypes is controversial. Twenty GBS patients diagnosed according to the Brighton criteria during the ZIKV outbreak in Cúcuta, Colombia, were evaluated clinically and electrophysiologically. The electrodiagnosis of GBS subtypes was made according to a recently described criteria set that demonstrated a high diagnostic accuracy on the basis of a single test. The electrophysiological features of 34 Italian AIDP patients were used as control. All patients had symptoms compatible with ZIKV infection before the onset of GBS and ZIKV infection was laboratory confirmed through a plaque reduction neutralization test (PRNT90 ) in 100% of patients. The median time from onset of ZIKV infection symptoms to GBS was 5 days (interquartile range 1-6 days). Cranial nerve palsy was present in 85% of patients (facial palsy in 75%, bulbar nerve involvement in 60%), autonomic dysfunction in 85%, and 50% of patients required invasive mechanical ventilation. AIDP was diagnosed in 70% of patients. 40% of nerves of AIDP patients showed a prevalent distal demyelinating involvement but this pattern was not different from the Italian AIDP patients without ZIKV infection. Guillain-Barré syndrome associated with ZIKV infection in Cúcuta is characterized by a high frequency of cranial nerve involvement, autonomic dysfunction and requirement of mechanical ventilation indicating an aggressive and severe course. AIDP is the most frequent electrophysiological subtype. Demyelination is prevalent distally but this pattern is not specific for ZIKV infection.

Guillain‐Barré syndrome: subtypes and predictors of outcome from India

There is a paucity of large studies evaluating the subtypes of Guillain-Barré syndrome (GBS) and their outcome from Southeast Asia. We report cliniconeurophysiological subtypes of GBS and their correlation with triggering events and 3-month outcome from northern India. Three hundred and twenty eight consecutive patients with GBS were clinically evaluated, including their triggers, severity, autonomic involvement, cranial nerve palsy, and respiratory paralysis. Nerve conduction study (NCS) was repeated at 3 weeks if the initial study was normal. They were categorized into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), inexcitable motor nerve, and equivocal. Clinically, 204 (62.2%) patients had pure motor, 106 (32.3%) motor sensory, 16 (4.9%) Miller Fisher syndrome, and 2 (0.6%) pure sensory GBS. Based on NCS, 242 (73.8%) had AIDP, 44 (13.4%) AMAN, 15 (4.6%) AMSAN, 8 (2.4%) inexcitable motor nerves, and 27 (8.2%) equivocal GBS. AIDP patients were older, more common in summer, had lesser peak disability, and better outcome compared to those with AMAN. Eleven (3.4%) patients died and 48 (14.6%) had poor outcome at 3 months. The poor outcome was related to severity, dysautonomia, and inexcitable motor nerves. AIDP is the commonest variant of GBS in our study and has better outcome compared to AMAN.

A-waves in Guillain–Barré syndrome: Correlation with electrophysiological subtypes and antiganglioside antibodies

ObjectiveTo investigate the relationship of A-waves with conventional electrophysiological subtypes of Guillain–Barré syndrome (GBS), as well as with anti-ganglioside antibodies. MethodsThe subjects consisted of 30GBS patients who were classified into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy, and unclassified based on the results of nerve conduction studies. “Abundant A-waves” were defined for the upper-limb nerves (median and ulnar nerves) using receiver–operator characteristic curves. The presence or absence of IgG anti-ganglioside antibodies was also noted. ResultsAbundant A-waves at weeks 3–6 from onset were observed in 64% of the 14 AIDP patients and 0% of 16 non-AIDP patients, and in 60% of 15 antibody-negative patients and 0% of 15 antibody-positive patients. In the earlier period, this relationship was less clear. The correlation between the conventional electrophysiological subtypes and antibodies was present, but was much weaker. ConclusionsAbundant A-waves in GBS after the acute phase were strongly associated with demyelination that was not mediated by antiganglioside antibodies, possibly through the mechanism of proximal re-excitation induced by electrical inhomogeneities due to segmental demyelination. SignificanceAbundant A-waves are promising as a novel reliable marker of demyelination.

Association between Helicobacter pylori infection and acute inflammatory demyelinating polyradiculoneuropathy

The aim of this study was to investigate a possible association between Helicobacter pylori infection and acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Of 17 consecutive patients with Guillain-Barre syndrome (GBS), 13 patients (six females; mean age 50 +/- 24 years) with AIDP were investigated. Clinical status was evaluated according to Hughes' score, and electrophysiological tests were performed within 2 weeks from disease onset. Helicobacter pylori infection was detected histologically and serum H. pylori-specific IgG antibodies were analysed by ELISA. Twenty asymptomatic patients (12 females; mean age 63 +/- 8 years), undergoing upper gastrointestinal endoscopy for investigation of mild iron deficiency anaemia, served as controls. Helicobacter pylori was found in 12 of 13 AIDP patients (92%), and in 10 of 20 controls (50%), (P = 0.02). Electrophysiological studies showed demyelination in all AIDP patients. High levels of anti-H. pylori IgG antibodies correlated with advanced clinical status. Five of seven AIDP patients with high levels of anti-H. pylori IgG antibodies had delayed F-wave latencies, indicating affection of proximal segments of peripheral nerves. Helicobacter pylori infection seems to be more frequent in AIDP patients. Anti-H. pylori titre might reflect advanced clinical status. Anti-H. pylori IgG antibodies are also associated with involvement of the proximal parts of peripheral nerves in AIDP.

Neurophysiologic findings in early acute inflammatory demyelinating polyradiculoneuropathy

Background: Patients with early acute inflammatory demyelinating polyradiculoneuropathy (AIDP) may not meet the current neurophysiologic criteria. Objective: To document neurophysiologic findings in early AIDP. Methods: Clinical and neurophysiologic data from 38 AIDP patients, assessed within 10 days of symptom onset were reviewed. Results: In addition to absent H reflexes and abnormal F-wave responses in the acute stage of AIDP, abnormalities of blink reflexes, upper limb sensory responses abnormalities with spared sural responses, presence of distal CMAP dispersion, and A-waves were frequently observed. Established demyelinating neurophysiologic parameters were infrequently seen. Conclusions: Abnormalities of H reflexes and F responses were most frequently noted in early AIDP. Additionally, distal temporal dispersion, prolonged or absent blink reflexes, and A-waves were often present in the acute stage of AIDP when classic diagnostic criteria of AIDP were not satisfied. Significance: Neurophysiologic studies in early AIDP frequently reveal abnormalities that are not specific of primary demyelinating neuropathy.

Epidemiological, clinical, and electrodiagnostic findings in childhood Guillain-Barré syndrome: a reappraisal.

We evaluated 61 children with Guillain-Barré syndrome, 14 months to 14 years of age, admitted to the Hospital Nacional de Pediatria in Buenos Aires. According to the electrodiagnostic findings, they fit into two groups, those with acute motor axonal neuropathy (AMAN) (18 patients) and those with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (43 patients). Ninety percent of the children with AMAN resided in suburban or rural areas without running water, whereas half of the AIDP patients lived in a metropolitan district. Summer and winter months showed a higher incidence of both variants. Children with AMAN were younger, evolved more acutely, reached a higher maximum disability score, required assisted ventilation more often, had lower mean level of cerebrospinal fluid protein, improved more slowly, and had a poorer outcome 6 months and 12 months after onset. Electrophysiological findings in those with AIDP revealed a pattern of severe diffuse slowing in children 5 years old or younger and a multifocal pattern in children 6 years old or older. This difference was not reflected in the clinical picture. In contrast, AMAN showed a uniform pattern with normal sensory conduction, severely reduced compound muscle action potential amplitude, near normal conduction velocity, and early denervation. Epidemiological, clinical, electrodiagnostic, cerebrospinal fluid, and prognostic data indicate that these variants of Guillain-Barré syndrome should be regarded as different entities.

The distribution of CD1 molecules in inflammatory neuropathy

The CD1 molecules have been shown to present non-protein antigens, such as complex lipids to Mycobacteria, and may be important in presenting glycolipids which are involved in inflammatory neuropathies. To study the expression of CD1 molecules in peripheral nerve, we examined nerve biopsies from two patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), five with acute axonal neuropathy, six with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), nine with chronic axonal neuropathy, six with vasculitic neuropathy and three with no histological abnormality. Immunocytochemical studies showed strong labelling of CD1b on endoneurial macrophages (CD68+) and on myelinated nerve fibres in both AIDP patients, but it was rarely observed in the other patients. Weaker staining was seen on endoneurial macrophages and/or other endoneurial cells in some of the patients with other peripheral neuropathies, but none of the control nerves. CD1a had a weaker, but similar pattern. There was endoneurial infiltration of CD4+ and CD8+ T cells in the AIDP and CIDP nerves and sometimes in the other peripheral neuropathy nerves, but not in the normal nerves. Most T cells had alpha beta+ T cell receptors (TCR), but gamma delta+ TCR T cells were found in the nerves of both AIDP patients and sometimes in the nerves of other patients with peripheral neuropathy. Staining for mannose receptor was almost universal, being more intense in AIDP, chronic axonal neuropathy and vasculitis patients. We conclude that CD1 molecule expression is upregulated in peripheral neuropathy, especially in association with inflammation.

T cell receptor Vβ gene usage in Guillain-Barré syndrome

We set out to determine whether the T cell receptor (TCR) Vβ gene usage in acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is restricted. We separated activated from non-activated peripheral blood T cells with anti-IL2 receptor (anti-CD25) antibody-labelled magnetic beads from four AIDP patients and four normal control (NC) subjects. The TCR Vβ gene usage of circulating activated and non-activated T cells was heterogeneous in all the patients and controls, but the activated T cells of all four of the AIDP patients showed a more limited usage of Vβ genes and enhanced Vβ 15 usage, as compared to the non-activated T cells. This was not seen in the healthy controls. The activated and non-activated T cells from a patient with acute motor and sensory axonal neuropathy (AMSAN) showed a similar Vβ gene usage to that of the controls. From a further patient with AIDP, we studied the Vβ gene usage of short-term T cell lines reactive to the peripheral nerve myelin proteins P2, P0 and the P0 peptide amino acid sequence 194–208. The Vβ gene usage of the lines was heterogeneous, with enhanced usage of Vβ 15 in the cell line responsive to the P0 peptide. We conclude that T cells activated during the immune response associated with AIDP preferentially used Vβ 15, which may indicate a restricted response to a common antigen, or a role for an as yet undefined superantigen in the pathogenesis of AIDP. © 1997 Elsevier Science B.V. All rights reserved.