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Acute Motor Axonal Neuropathy (AMAN) is an immune-mediated disorder of the peripheral nervous system, part of the spectrum of the Guillain-Barre syndrome (GBS). An infectious event most often triggers it reported a few weeks before the onset. The reported case is of a 56 years-old woman who developed acute motor axonal neuropathy three weeks after respiratory infection with influenza A virus subtype H1N1. Despite early treatment with plasmapheresis and intravenous immunoglobulins, the patient remained tetraplegic, mechanically ventilated for five months, with repetitive unsuccessful weaning trails. The probable cause was considered to be phrenic nerve palsy in the context of acute motor axonal neuropathy. This case highlights that acute motor axonal neuropathy is a severe and life-threatening form of Guillain-Barre syndrome associated with significant mortality and morbidity. Neurological and physical recovery strongly depend on the inter-professional effort in an intensive care unit and neurology professionals.

Introduction/aims Patients with acute motor axonal neuropathy (AMAN) generally have pure motor neuropathy and clinicians usually do not link pain with AMAN. The aim of this retrospective study was to describe the character, location, and intensity of pain in AMAN and acute inflammatory demyelinating polyneuropathy (AIDP) in the acute phase. Methods This was a retrospective study in 44 patients with Guillain-Barre syndrome (GBS) having progressive weakness of more than one limb. The information, including the demographic characteristics, preceding infections, clinical symptoms and signs, severity at nadir, the characteristics of pain, use of analgesics, laboratory and electrophysiological data, and the medical treatment for GBS, were collected from the medical records. Results In 44 patients, 40.9% were diagnosed as AMAN, and 34.1% as AIDP. Pain was more prevalent in AMAN (76.5%) than in AIDP (26.7%, P = .02). Low back and extremities were the most common locations of pain in AMAN (7/13 and 7/13, respectively) and AIDP (2/4 and 2/4, respectively). Discussion Pain was a common symptom in AMAN in the acute stage. The presence or absence of pain is not useful for distinguishing AIDP from AMAN.

Neurological complications are increasingly being reported in dengue fever, and the dengue virus is now recognized as a neurotrophic virus. The damage caused by inflammatory cytokines in the febrile phase and molecular mimicry in the recovery phase is responsible for these neurological manifestations. We report such an unusual neurological complication occurring in a 27-year-old female in the recovery phase of dengue fever, who developed an acute onset of ascending symmetric weakness of all four limbs without any sensory, autonomic, cerebellar, or cranial nerve involvement. She was diagnosed as having an acute motor axonal neuropathy (AMAN) variant of Guillain-Barre syndrome (GBS) based on a nerve conduction study (NCS) showing axonal neuropathy and contrast-enhanced magnetic resonance imaging (CE-MRI) showing root enhancement at the region of the cauda equina. She was treated with intravenous immunoglobulin (IVIG) and showed full recovery from symptoms with treatment. Our case highlights the importance of being aware of such rare neurological complications in dengue fever. Early detection and rapid initiation of treatment can lead to the complete reversal of neurological deficits.

INTRODUCTION/AIMS Ultrasound (US) studies have demonstrated patchy enlargement of spinal and peripheral nerves in Guillain-Barre syndrome (GBS). However, whether ultrasound yields useful information for early classification of GBS has not been established. We aimed to evaluate nerve ultrasound in patients with GBS in northern China and compare the sonographic characteristics between demyelinating and axonal subtypes. METHODS Between November 2018 and October 2019, 38 hospitalized GBS patients within 3 wk of disease onset and 40 healthy controls were enrolled. Ultrasonographic cross-sectional areas (CSA) of the peripheral nerves, vagus nerve, and cervical nerve roots were prospectively recorded in GBS subtypes and controls. RESULTS Ultrasonographic CSA exhibited significant enlargement in most patients' nerves compared with healthy controls, most prominent in cervical nerves. The CSA tended to be larger in acute inflammatory demyelinating polyneuropathy (AIDP) than in acute motor axonal neuropathy (AMAN)/acute motor and sensory axonal neuropathy (AMSAN), especially in cervical nerves (C5: 5.9 ± 1.6 mm2 vs. 7.0 ± 1.7 mm2 , p = .042; C6: 10.5 ± 1.8 mm2 vs. 12.0 ± 2.1 mm2 , p = .033). The chi-squared test revealed significant differences in nerve enlargement in C5 (p

Background Although rare, Guillain-Barré Syndrome (GBS) has a high economic burden, with consequences for families and society. This study aimed to estimate the total cost of GBS, per individual and per variant of the disease, as well as its effect on household income, from the perspective of patients. Methods This was a cost-of-illness study from the perspective of patients and their families, with a time horizon from disease onset to 6 mo after discharge. The total cost of GBS was estimated by bottom-up microcosting, considering direct and indirect costs. Results The median cost of GBS per individual was US$1635.5, with direct costs accounting for 64.3% of this amount. Among the variants analyzed, acute motor sensory axonal neuropathy (US$4660.1) and acute inflammatory demyelinating polyneuropathy (US$2017.0) exhibited the highest costs compared with acute motor axonal neuropathy (US$1635.5) and Miller Fisher Syndrome (US$1464.8). The costs involved compromise more than 20% of the household income of 22 (47.8%) patients. Conclusions This study demonstrated how costly GBS can be. It is hoped that decision-makers will analyze these results with a view to improving the structure of healthcare services.

Background and purpose An incremental number of cases of acute transverse myelitis (ATM) in individuals with ongoing or recent coronavirus disease 2019 (COVID-19) have been reported. Methods A systematic review was performed of cases of ATM described in the context of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection by screening both articles published and in preprint. Results Twenty cases were identified. There was a slight male predominance (60.0%) and the median age was 56 years. Neurological symptoms first manifested after a mean of 10.3 days from the first onset of classical, mostly respiratory symptoms of COVID-19. Overall, COVID-19 severity was relatively mild. Polymerase chain reaction of cerebrospinal fluid for SARS-CoV-2 was negative in all 14 cases examined. Cerebrospinal fluid findings reflected an inflammatory process in most instances (77.8%). Aquaporin-4 and myelin oligodendrocyte protein antibodies in serum (tested in 10 and nine cases, respectively) were negative. On magnetic resonance imaging, the spinal cord lesions spanned a mean of 9.8 vertebral segments, necrotic-hemorrhagic transformation was present in three cases and two individuals had additional acute motor axonal neuropathy. More than half of the patients received a second immunotherapy regimen. Over a limited follow-up period of several weeks, 90% of individuals recovered either partially or near fully. Conclusion Although causality cannot readily be inferred, it is possible that cases of ATM occur para- or post-infectiously in COVID-19. All identified reports are anecdotal and case descriptions are heterogeneous. Whether the condition and the observed radiological characteristics are specific to SARS-CoV-2 infection needs to be clarified.

To compare clinical and nerve conduction studies (NCS) parameters predictive of outcome in children with acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). In this prospective observational study, NCS was done on all children at admission and repeated before discharge. Functional status of patients was graded as per Hughes Disability score. These children were followed up till they achieved independent walking. Clinical and NCS criteria were compared between (a) AMAN and AIDP and (b) two subgroups of children with AMAN—those who achieved early (within 60 d) versus delayed (i.e., after 60 d) walking. Fifty-seven children were initially enrolled, first NCS showed inexcitable nerves in 10, AMAN in 29, acute motor-sensory axonal neuropathy (AMSAN) in 3, AIDP in 13, and 2 were normal. Subsequent NCS showed AMAN in 37, AIDP in 15, AMSAN in 3 patients. There were no deaths, 16 required ventilation. Follow-up till independent walking, was available for 40 patients. AMAN was associated with faster progression, greater peak disability, prolonged hospital stay, and delayed walking (p

The recent outbreak of Zika virus infection increased the incidence of Guillain–Barré syndrome (GBS). Following the first reported case of GBS after Zika virus infection in 2013, there has been a considerable increase in the incidence of GBS in endemic countries, such as French Polynesia and Latin American countries. The association between coronavirus disease 2019 (COVID‐19), caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2), and GBS is another emerging research hotspot. Electrophysiological studies have suggested that GBS patients associated with Zika virus infection or COVID‐19 tend to manifest acute inflammatory demyelinating polyneuropathy, rather than acute motor axonal neuropathy (AMAN). Causative autoantibodies, such as anti‐ganglioside antibodies in AMAN associated with Campylobacter jejuni infection, have not been identified in GBS associated with these emerging infectious diseases. Nevertheless, recent studies suggested molecular mimicry between these viruses and human proteins related to GBS. Recent studies have shown the efficacy of new vaccines, containing artificial messenger RNA encoding the spike protein of SARS‐CoV‐2, against. These vaccines are now available in many countries and massive vaccination campaigns are currently ongoing. Although there are long‐standing concerns about the increased risk of GBS after inoculation of conventional vaccines, the risk of GBS is not considered a legitimate reason to limit administration of currently available vaccines, because the benefits outweigh the risks., In this article, we review the evidence regarding the relationship between emerging infectious diseases (such as Zika virus infection and coronavirus disease 2019) and Guillain–Barré syndrome. The contemporary knowledge about post‐vaccination Guillain–Barré syndrome is also summarized.

Electrophysiology plays a determinant role in Guillain-Barré syndrome (GBS) diagnosis, classification, and prognostication. However, traditional electrodiagnostic (EDX) criteria for GBS rely on motor nerve conduction studies (NCS) and are suboptimal early in the course of the disease or in the setting of GBS variants. Sensory nerve conduction studies, including the sural-sparing pattern and the sensory ratio are not yet included in EDX criteria despite their well-established role in GBS diagnosis. The aim of this review is to discuss the diagnostic value of sensory NCS in GBS, their role in establishing the diagnosis and predicting the outcome according to the various subtypes of the disease.

The relation between severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection and demyelinating Guillain‐Barre syndrome (GBS) has been defined. We aim to report the clinical features of a child with axonal GBS associated with SARS‐CoV‐2. A 6‐year‐old male presented with symmetric ascending paralysis progressed over a 4‐day course and 2 days of fever. He had bilateral lower and upper limb flaccid weakness of 1/5 with absent deep tendon reflexes. He had severe respiratory muscle weakness requiring invasive mechanical ventilation. On admission, SARS‐CoV‐2 returned as positive by real‐time polymerase chain reaction on a nasopharyngeal swab. Cerebrospinal fluid analysis showed elevated protein without pleocytosis. He was diagnosed with GBS associated with SARS‐CoV‐2 infection. The nerve conduction study was suggestive of acute motor axonal neuropathy. Ten consecutive therapeutic plasma exchange sessions with 5% albumin replacement followed by four sessions on alternate days were performed. On Day 12, methylprednisolone (30 mg/kg/day for 5 days) was given. On Day 18, intravenous immunoglobulin (2 g/kg/day) was given and repeated 14 days after due to severe motor weakness. On Day 60, he was discharged from the hospital with weakness of neck flexor and extensor muscles of 3/5 and the upper limbs and the lower limbs of 2/5 on home‐ventilation. Our patient is considered to be the youngest patient presenting with a possible para‐infectious association between axonal GBS and SARS‐CoV‐2 infection. The disease course was severe with a rapid progression, an earlier peak, and prolonged duration in weakness as expected in axonal GBS.

Objective: To analyze patients with Guillain Barre Syndrome (GBS) requiring intensive care unit (ICU) admission, their course, response to treatment and outcome. Methods: A retrospective study conducted at ICU of The Children’s Hospital Lahore in 194 children with diagnosis of GBS from June 2018 to May 2020. Demographic profile, clinical features, treatment, duration of mechanical ventilation, length of ICU stay and outcome was noted. Results: Mean age of children was 5.95 ± 3.1 years with male predominance 136 (70 %). Major proportion were in range of 1-5 years 93(47.9%). Mean duration of ICU stay was 21.3±34.4 days. Out of 194 patients 125(64%) received IVIGs while 112 (57.7%) required mechanical ventilation because of rapidly progressive weakness within 24 hours of admission and those who remained on mechanical ventilation for longer period showed AMSAN (Acute motor sensory axonal neuropathy) type of GBS ( p value

We reported a rare case demonstrating that the hyperbaric oxygen chamber provided faster clinical improvement in a patient with a variant of Guillain-Barre Syndrome (GBS). A patient with progressive, acute weakness of upper extremity locomotor muscles and with difficulty breathing and swallowing was diagnosed with axonal GBS. Despite life-saving conventional therapies, there was no significant improvement until day 5. During hyperbaric oxygen therapy, there were daily gradual improvements until day 20, at which time the patient was capable of walking slowly without using a walking aid.

Ni Komang Sri Dewi Untari,1,2 Kurnia Kusumastuti,3 Guritno Suryokusumo,4 I Ketut Sudiana5 1Department of Hyperbaric, Drs. Med. Rijadi S. Phys. Naval Health Institute, Surabaya, Indonesia; 2Doctoral Program, Faculty of Medicine, Airlangga University, Surabaya, Indonesia; 3Department of Neurology, Faculty of Medicine, Airlangga University, Surabaya, Indonesia; 4Department of Hyperbaric, Faculty of Medicine, Pembangunan Nasional University, Jakarta, Indonesia; 5Department of Pathology Anatomy, Faculty of Medicine, Airlangga University, Surabaya, IndonesiaCorrespondence: Ni Komang Sri Dewi UntariDepartment of Research and Development, Drs. Med. R. Rijadi S., Phys. Naval Health Institute, Surabaya, IndonesiaEmail komang_neuro@yahoo.comAbstract: We reported a rare case demonstrating that the hyperbaric oxygen chamber provided faster clinical improvement in a patient with a variant of Guillain-Barre Syndrome (GBS). A patient with progressive, acute weakness of upper extremity locomotor muscles and with difficulty breathing and swallowing was diagnosed with axonal GBS. Despite life-saving conventional therapies, there was no significant improvement until day 5. During hyperbaric oxygen therapy, there were daily gradual improvements until day 20, at which time the patient was capable of walking slowly without using a walking aid.Keywords: acute motor axonal neuropathy, hyperbaric oxygen

Background: Internationally approved electrodiagnostic criteria for Guillain Barre syndrome lack in children. We intended to compare the diagnostic accuracy of the currently available five electrophysiological criteria for childhood Guillain Barre Syndrome (GBS) at the time of sentinel assessment. Methodology: In this single-center study, data of children diagnosed with GBS between January 2013 to December 2017 were retrieved. Patient charts were reviewed for clinical features, electrophysiological recordings. The electrodiagnostic results (4 motor nerves and two sensory nerves in upper limbs and lower limbs) were reanalyzed and were classified based on Dutch group; Ho; Hadden; Hughes and Rajabally criteria for GBS. Results: During this study period, of the 205 children with clinical features of GBS, 15 children had incomplete electrophysiological data, and four children were excluded due to missing data. The mean age of onset of the 186 children enrolled was 77 months; the median duration from symptom onset to electrodiagnostic evaluation was seven days; pure motor and motor-sensory form of GBS was seen in 71 and 115 children. Based on the Hadden criteria, a demyelinating pattern was noted in 57 children; axonal in 37; Inexcitable in 84 and Equivocal in 8 children. The sensitivity of the various criteria ranged from 71% to 100% for demyelination, 97% to 100% for axonal. The degree of agreement using Hadden and Rajabally criteria for Equivocal subtypes was 0.93. Conclusions: The Rajabally criteria showed the best sensitivity, specificity and diagnostic accuracy for electrodiagnosis of GBS in children when compared against Hadden criteria.

Background: As a global health pandemic, the novel severe acute respiratory syndrome-coronavirus 2 (SARS- CoV2) outbreak began in December 2019 which rapidly spread to more than 200 countries. Respiratory complications and fever are the most obvious symptoms. Sometimes the neurological features are superimposed on the main disease and complicate patientchr('39')s status. Case Presentation: We describe 6 patients with COVID-19 and concomitant quadriparesia who underwent electrodiagnosis using EMG/NCS and results indicated 3 axonal variants of Guillain–Barré syndrome (GBS), including; 2 cases AMAN (acute motor axonal neuropathy), 1 case AMSAN (acute motor and sensory axonal neuropathy), three myopathies, including one combination of CIN/CIM (critical illness neuropathy/critical illness myopathy), one CIM and one acute polymyositis in these cases. Conclusion: Early diagnosis of the neuromuscular disorders of coronavirus could help for correct planning in the treatment of COVID-19 patients. Since GBS and inflammatory myopathies have an autoimmune basis, the immunotherapies such as IVIG, steroids, plasma exchange and other novel treatments as hemoperfusion can promise better and faster recovery in respiratory function and neuromuscular activity among COVID-19 patients who have musculature paralysis concomitantly. However, all these treatments are challenging and further clinical trials should be done to confirm the efficacy and safety of mentioned therapies.

Introduction. The heterogeneity of the forms and severity of Guillain-Barre syndrome explains the variability of recovery: from rapid and complete (in most cases) to slow with the development of persistent residual deficiency (rarely). It is unclear how effective the Erasmus Guillain–Barre syndrome Outcome Scores and its modified version are for different forms of the disease.The aim of the study – to demonstrate the features of recovery in acute motor axonal neuropathy with conduction blocks on the example of 2 clinical cases; to show the possibilities of Erasmus Guillain–Barre syndrome Outcome Scores and its modified version in predicting recovery in this form of the disease.Materials and methods. Data from 2 patients with acute motor axonal neuropathy with motor conduction blocks were retrospectively analyzed. Calculation of the score and assessment of the prognosis of walking recovery by 6 months from the onset of the disease were performed using the online calculator International Guillain-Barre syndrome Outcome Study Prognosis tool in the acute period.Results. In both patients, the forecast of recovery of walking by half a year from the onset of the disease on the Erasmus Guillain–Barre syndrome Outcome Scores and modified Erasmus Guillain–Barre syndrome Outcome Scores scales in the acute period was erroneous. In the first case, the total score on the Erasmus Guillain–Barre syndrome Outcome Scores and its modification in the acute period was 5 and 10 points respectively (poor prognosis), which foreshadowed a long rehabilitation process and incomplete recovery. However, the regression of disorders was dramatic and complete, and by the second month of the disease, only minimal motor disorders remained. In the second patient, on the contrary, the total Erasmus Guillain–Barre syndrome Outcome Scores and its modification during the period of increasing symptoms was 3 and 7 points respectively (good prognosis), while recovery was delayed – only by 5 months from the onset of the disease, the ability to move with support was restored.Conclusion. The Guillain–Barre syndrome is a disease with a favorable prognosis for recovery. However, the prediction of regression of motor disorders should be approached carefully, because in some cases, generally accepted criteria and prognostic scales may not work. Acute motor axonal neuropathy with conduction blocks is a unique form of the disease that has pathophysiological and clinical-neurophysiological features, which should be taken into account when managing this category of patients.

Guillain-Barre syndrome is a post infective polyradiculoneuropathy having heterogenous clinical presentation and various subtypes like acute inflammatory demyelinating polyradiculoneuropathy, acute motor-sensory axonal neuropathy, acute motor axonal neuropathy, pure sensory variant and Miller Fisher syndrome. A prospective study was carried out in patients with Guillain-Barre syndrome admitted to the RNT Medical College & attached Hospital to determine the electrophysiological subtypes and their prognosis in relation to various subtypes, clinical features and treatment. A total of 100 patients was enrolled. In the final analysis there were 74% male and the mean age was 30.4 years. Clinically 97% patients had quadriparesis, 2% had paraparesis and one cases had bibrachial involvement. Cranial nerves and respiratory involvement were seen in 25% and 24% cases respectively. Electrophysiologically the most common type of GBS was AIDP (43%) followed by AMAN (34%) and AMSAN (23%). The prognosis was assessed at one month and found that there was complete recovery in 32% cases and residual weakness in 63% cases. Death occurred in 5% cases because of respiratory involvement

Background The clinical characteristics of electrophysiological subtypes and prognostic factors of Mexican adults diagnosed with Guillain-Barre Syndrome (GBS) have not been described. Materials and methods A single center, ambispective, cohort study was performed (2015–2019). GBS was defined following the Asbury and Cornblath criteria. Electrodiagnosis was made according to Hadden criteria. Clinical, biochemical and electrodiagnostic parameters were described, compared and analyzed using a multivariate model. Only patients who completed a 3-month follow-up were included. Results 137 GBS patients (92 males; mean age 46.6 ± 16.6). 132 (96.3%) underwent an electrodiagnostic assessment. 68 (51.5%) were classified as axonal GBS, with further classified into two groups: acute motor axonal neuropathy (AMAN) 45.4%, and acute motor and sensory axonal neuropathy (AMSAN) 8,6%. The following characteristics were lower in the AMAN group: Medical Research Counsel sumscore (MRC) 30.1 ± 16.3 vs 36.4 ± 14.4, unilateral facial palsy 10% vs 25.9% and albuminocytologic dissociation 41.3% vs. 71.7%. Multivariate analysis found AMAN as an independent predictor of an unfavorable outcome OR: 3.34 (p = 0.03) Conclusions AMAN subtype is the most frequent presentation of GBS in Mexican adult patients and an independent predictor of inability to walk independently at 3 months after discharge.

BACKGROUND: Acute motor axonal neuropathy (AMAN) is a peripheral nerve disorder that attacks motor axons and occurs acutely. AMAN is one type of Guillain–Barre syndrome (GBS) which often attacks men of productive age. Until now, although patients have undergone intravenous immunoglobulin (IVIG) therapy and/or plasmapheresis, long-standing disability remains a problem. In Indonesia, the availability and cost of these therapies are constraints. AIM: Our study aimed to find a proper animal model suitable for AMAN and can be executed in our institution, Naval Health Institute with a hope to find new therapeutic modalities in healing with AMAN. METHODS: GM1 ganglioside immunized in New Zealand male white rabbits with complete Freund’s adjuvant, every 3 weeks until 20 weeks. We evaluated the effects GM1 ganglioside on body weight, functional score, and axon degeneration’s scale. Functional score was examined based on Tarlov’s. Hematoxylin-eosin was used to stain this slide. RESULTS: Rabbits that being immunized with GM1 ganglioside experience a number of neurological signs and symptoms that resemble AMAN, that is, sluggish righting reflex, muscular weakness, flaccid hyper paralysis, and body weight loss. Pathological examination shows extensive degeneration of peripheral nerves, infiltration of macrophages, and perineuritis. CONCLUSION: This histological and clinical findings support that this neuropathy is induced by an autoimmune response delivered by cells that respond to gangliosides.

Hyper-reflexia is occasionally seen in acute motor axonal neuropathy (AMAN), but its pathophysiology is unclear. We report a patient with AMAN following Campylobacter jejuni enteritis, who showed generalized hyper-reflexia, bilateral Hoffmann sign and right Babinski sign. MRI and transcranial magnetic stimulation of the motor cortex disclosed no corticospinal tract involvement. An extensive electrophysiological investigation documented α-motoneuron hyperexcitability and dysfunction of the interneuronal inhibitory circuits in the spinal anterior horn. We propose an immune-mediated damage of the spinal inhibitory interneuronal network as possible mechanism inducing hyper-reflexia in AMAN.

The aim of this review was to analyse the pathophysiology of axonal degeneration in Guillain–Barre syndrome (GBS) with emphasis on early stages (≤ 10 days after onset). An overview of experimental autoimmune neuritis (EAN) models is provided. Originally GBS and acute inflammatory demyelinating polyneuropathy were equated, presence of axonal degeneration being attributed to a “bystander” effect. Afterwards, primary axonal GBS forms were reported, designated as acute motor axonal neuropathy/acute motor–sensory axonal neuropathy. Revision of the first pathological description of axonal GBS indicates the coexistence of active axonal degeneration and demyelination in spinal roots, and pure Wallerian-like degeneration in peripheral nerve trunks. Nerve conduction studies are essential for syndrome subtyping, though their sensitivity is scanty in early GBS. Serum markers of axonal degeneration include increased levels of neurofilament light chain and presence of anti-ganglioside reactivity. According to nerve ultrasonographic features and autopsy studies, ventral rami of spinal nerves are a hotspot in early GBS. In P2-induced EAN models, the initial pathogenic change is inflammatory oedema of spinal roots and sciatic nerve, which is followed by demyelination, and Wallerian-like degeneration in nerve trunks possessing epi-perineurium; a critical elevation of endoneurial fluid pressure is a pre-requisite for inducing ischemic axonal degeneration. Similar lesion topography may occur in GBS. The repairing role of adaxonal Schwann cytoplasm in axonal degeneration is analysed. A novel pathophysiological mechanism for nerve trunk pain in GBS, including pure motor forms, is provided. The potential therapeutic role of intravenous boluses of methylprednisolone for early severe GBS and intractable pain is argued.

We aimed to evaluate the key diagnostic features of Guillain-Barré syndrome (GBS) in Malaysian patients and validate the Brighton criteria. This was a retrospective study of patients presenting with GBS and Miller Fisher syndrome (MFS) between 2010 and 2019. The sensitivity of the Brighton criteria was evaluated. A total of 128 patients (95 GBS, 33 MFS) were included. In the GBS cohort, 92 (97%) patients presented with symmetrical limb weakness. Reflexes were depressed or absent in 90 (95%) patients. Almost all patients (94, 99%) followed a monophasic disease course, with 5 (5%) patients experiencing treatment-related fluctuations. Cerebrospinal fluid (CSF) albuminocytological dissociation was seen in 62/84 (73%) patients. Nerve conduction study (NCS) revealed neuropathy in 90/94 (96%) patients. In GBS patients with complete dataset (84), 56 (67%) patients reached level 1 of the Brighton criteria, 21 (25%) reached level 2, 3 (4%) reached level 3, and 4 (5%) reached level 4. In MFS, the clinical triad was present in 25 (76%) patients. All patients had a monophasic course. CSF albuminocytological dissociation was present in 10/25 (40%) patients. NCS was normal or showed sensory neuropathy in 25/33 (76%) patients. In MFS patients with complete dataset (25), 5 (20%) patients reached level 1 of the Brighton criteria, 14 (56%) reached level 2, 2 (8%) reached level 3, and 4 (16%) reached level 4. Inclusion of antiganglioside antibodies improved the sensitivity of the Brighton criteria in both cohorts. In the Malaysian cohort, the Brighton criteria showed a moderate to high sensitivity in reaching the highest diagnostic certainty of GBS, but the sensitivity was lower in MFS.

The aim of this paper is to analyse the pathological background of very early Guillain-Barré (VEGBS) (≤ 4 days after onset) comparing it with initial stages of experimental autoimmune neuritis (EAN). The pathological hallmark of VEGBS is inflammatory oedema predominating in proximal nerve trunks. In EAN inflammatory oedema precedes the development of demyelination or axonal degeneration; such oedema may increase endoneurial fluid pressure (EFP) stretching the perineurium and constricting the transperineurial microcirculation. Centrofascicular or wedge-shaped areas of nerve ischemia have been reported in GBS and EAN. Additional support for proximal VEGBS pathology comes from electrophysiology showing alterations in late responses as the most frequent features, and ultrasonography illustrating that main changes rely on ventral rami of spinal nerves. Selective inefficiency of the blood-nerve barrier would explain the topography of changes in VEGBS. Increased serum neurofilament light chain concentration has recently been reported in VEGBS, with no difference between demyelinating and axonal subtypes. This is a marker of axonal damage, which could be correlated with endoneurial ischemia caused by increased EFP. Inflammatory oedema of proximal nerve trunks may be pathogenic in VEGBS, and consequently there is a pressing need for therapeutic strategies to stop its rapid impact on the axons.

Neuropatias são condições que acometem o sistema nervoso periférico, podendo estar relacionadas a doenças sistêmicas. A síndrome de Guillain-Barré é o principal exemplo quando se fala em polirradiculoneuropatia aguda, geralmente seu quadro é associado a uma infecção prévia de Campylobacter jejuni sendo a neuropatia axonal motora aguda uma de suas variantes motoras puras e tem sua incidência variante de acordo com o espaço geográfico estudado entre 30 a 65% dos quadros totais de Guillain-Barré. As manifestações dessa variação são paralisia das extremidades de caráter simétrico e ascendente, arreflexia, com acometimento da musculatura respiratória, ocular e facial. Descrevemos um caso no qual o paciente apresentou, após uma infecção de vias aéreas superiores, parestesia de mãos e pés associada a fraqueza de caráter progressiva e ascendente, diplopia e disfagia, evoluindo para insuficiência respiratória aguda, após exames comprobatórios foi iniciado protocolo de tratamento com Hiperimunoglobulina Humana Endovenosa para o tratamento de Síndrome de Guillain-Barré. O paciente evoluiu com tetraplegia flácida e necessidade de ventilação mecânica após isso, no exame de eletroneuromiografia evidenciou-se Neuropatia Axonal Motora Aguda. Por fim novo ciclo de imunoglobulina humana intravenosa obtendo resultado. O paciente recebeu alta hospitalar com sequelas motoras, mantendo acompanhamento domiciliar multiprofissional após 63 dias de internamento em unidade de terapia intensiva.

Axonal variants of Guillain-Barre syndrome (GBS) mainly include acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and pharyngeal-cervical-brachial weakness. Molecular mimicry of human gangliosides by a pathogen's lipooligosaccharides is a well-established mechanism for Campylobacter jejuni-associated GBS. New triggers of the axonal variants of GBS (axonal GBS), such as Zika virus, hepatitis viruses, intravenous administration of ganglioside, vaccination, and surgery, are being identified. However, the pathogenetic mechanisms of axonal GBS related to antecedent bacterial or viral infections other than Campylobacter jejuni remain unknown. Currently, autoantibody classification and serial electrophysiology are cardinal approaches to differentiate axonal GBS from the prototype of GBS, acute inflammatory demyelinating polyneuropathy. Newly developed technologies, including metabolite analysis, peripheral nerve ultrasound, and feature selection via artificial intelligence are facilitating more accurate diagnosis of axonal GBS. Nevertheless, some key issues, such as genetic susceptibilities, remain unanswered and moreover, current therapies bear limitations. Although several therapies have shown considerable benefits to experimental animals, randomized controlled trials are still needed to validate their efficacy.

Background: Coronavirus disease 2019 (COVID-19) is associated with muscle and nerve injuries as a consequence of prolonged critical illness or the infection itself. In this study, we evaluated neuromuscular involvement in patients who underwent post-acute intensive rehabilitation after COVID-19. Methods: Clinical and neurophysiological evaluations, including nerve conduction studies and electromyography, were performed on 21 consecutive patients admitted for rehabilitation after COVID-19. Results: Clinical signs suggesting muscle or nerve involvement (weakness, reduced deep tendon reflexes, impaired sensitivity, abnormal gait) were found in 19 patients. Neurophysiological examinations confirmed neuromuscular involvement in 17 patients: a likely association of critical illness myopathy (CIM) and critical illness polyneuropathy (CIP) was found in 5 patients; CIM alone was found in 4 patients; axonal sensory-motor polyneuropathy was found in 4 patients (CIP in 2 patients, metabolic polyneuropathy in 2 patients); Guillain-Barré syndrome was found in 2 patients (classical demyelinating sensory-motor polyneuropathy and acute motor axonal neuropathy, respectively); peroneal nerve injury was found in 1 patient; and pre-existing L4 radiculopathy was found in 1 patient. Conclusions: Neuromuscular involvement is a very common finding among patients admitted for rehabilitation after COVID-19, and proper investigation should be conducted when muscle or nerve injury is suspected for adequate rehabilitative strategy planning.

Perioperative bilateral medial medullary infarction (BMMI) cases mimicking acute motor axonal neuropathy (AMAN) under general anesthesia have not been reported. We describe a patient who suffered flaccid quadriplegia and could not wean from mechanical ventilation after emergence from general anesthesia in cardiac surgery. A diagnosis of AMAN was considered, but intravenous immunoglobulin showed little efficacy. Magnetic resonance imaging of the patient later revealed BMMI with “snake eyes appearance,” and he was found to have severe vertebral artery stenosis. Considering the association between severe coronary heart disease and cerebrovascular stenosis, we highlight the significance of preoperative evaluation and comprehensive management of the cerebrovascular system for certain patients.

Guillain-Barré syndrome (GBS) in an immune mediated disease that affects peripheral nerves with possible life-threatening complications. GBS has multiple subtypes including acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN), which can make GBS difficult to diagnose. GBS commonly presents after viral infections such as influenza virus, campylobacter jejuni, and zika virus. GBS commonly presents with a prolonged clinical course leading to increased morbidity among affected patients. It is not surprising that COVID-19 has been connected with multiple cases of GBS, which may alter the recovery course for several patients post-COVID. In this report, we present a case of 69-year-old-female who presented with progressive motor weakness and loss of sensation in her extremities after testing positive for antibodies to COVID-19 one-month prior to presentation. Her presentation and treatment of GBS in the setting of COVID-19 is an example of one of the many COVID-19 complications and sheds light on the prolonged recovery course that we may experience as clinicians in the wake of this pandemic.

A 65 years-old woman started developing arthralgias, mild cough and dyspnea. When symptoms progressed to productive cough and moderate exertional dyspnea, she was admitted to the hospital. Thoracic CT revealed diffuse interstitial pneumopathy, pulmonary function tests indicated moderate obstruction (FEV1 = 60%) and bronchoalveolar lavage was suggestive of severe alveolar-hemorrhagic syndrome. Laboratory workup confirmed the presence of antibodies against myeloperoxidase (i.e. p-ANCA) and absence of mycobacterium tuberculosis, hence a diagnosis of granulomatosis with polyangiitis was established. During hospitalization, she developed rapidly progressive paraparesis with retained deep tendon reflexes. Electroneurography highlighted the presence of subacute onset motor axonal polyneuropathy affecting both upper and lower limbs. Under treatment with methylprednisolone, oral cyclophosphamide, alpha-lipoic acid and intravenous immunoglobulins, improvement of neurological status was noted.

Patient: Male, 37-year-old Final Diagnosis: Guillain-Barré syndrome Symptoms: Facial paralysis • paresthesia Medication: — Clinical Procedure: Cerebrospinal fluid analysis • magnetic resonance image • nerve conduction study Specialty: Neurology Objective: Unusual clinical course Background: Guillain-Barré syndrome (GBS) is an autoimmune disease of the peripheral nervous system. It has multiple presentations, demyelinating or axonal, according to the pattern of injury. In general, there are cardinal symptoms, such as areflexia and ascending symmetrical lower limb weakness. GBS has multiple different variants. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common type. Other known variants are acute motor and sensory axonal neuropathy, acute motor axonal neuropathy, acute sensory neuropathy, acute pan dysautonomia, and Miller Fisher syndrome. Case Report: In the present case, the patient had initial symptoms of distal bilateral paresthesia and 12 days later he developed left facial muscle weakness, a decrease in ability to taste, and right facial muscle weakness. Two days later the patient said he did not have limb weakness. On examination, he had bilateral lower motor facial palsy, the power in his upper and lower right limbs was 4/5, and he had bilateral upper limb hyperreflexia. Results of a nerve conduction study were consistent with acute demyelinating polyneuropathy with secondary axonal loss. The patient was treated with immunoglobulin G, 0.4 mg/kg/d for 5 days, and fully recovered. Conclusions: Facial diplegia is one of the GBS variants that presents rarely as pure bilateral facial weakness or is preceded by bilateral lower limb weakness. The present case underscores that patients with GBS may have facial diplegia before weakness. Also, they may not have areflexia as a cardinal feature, and instead, hyperreflexia may be seen. Although hyperreflexia has been reported in association with the acute motor axonal variant, the present case shows that hyperreflexia also can be found with AIDP.

Coronavirus Disease has become a global pandemic after its emergence at the end of 2019 as a cluster of pneumonia. Apart from respiratory symptoms, neurologic complications are also common, mostly in hospitalized patients. More than 80 percent of patients have neurological symptoms during their disease course of which most common is encephalopathy. However, data on neurological complications like Guillain-Barré syndrome associated with coronavirus-2019 are scarce. Here, we report a case of a 64-years-old female patient with typical clinical and electrophysiological manifestations of Acute motor axonal neuropathy variant, who was reported positive with polymerase chain reaction for severe acute respiratory syndrome coronavirus-2, 13 days before the onset of acute bilateral weakness of extremities, areflexia, and normal sensory examination. Cerebrospinal fluid and electrophysiological examination were also suggestive. The neurological symptoms improved during treatment with immunoglobulins. Quick recognition of symptoms and diagnosis is important in the management of Guillain-Barré syndrome associated with coronavirus-2019.

Objective: In the young generations with nitrous oxide abuse (N2O), featured electrophysiological response of the peripheral neuropathy caused by nitrous oxide remains to be defined.Methods: Patien...

A newly introduced term, "axonal conduction block," brought a confusion in the electrodiagnostic diagnosis of Guillain-Barrè syndrome (GBS). I am proposing the term "nodal conduction block" for "axonal conduction block." This unifying concept of nodal conduction block will accommodate both the traditional concept of demyelination as well as the new concept of nodopathy in the "axonal form of GBS,", making the practice of electrodiagnosis much easier.

Albeit primarily a disease of respiratory tract, the 2019 coronavirus infectious disease (COVID-19) has been found to have causal association with a plethora of neurological and neuropsychological effects. However, the pathogenesis of COVID-19-induced neurological manifestations is still in its infancy. Autonomic dysfunction preceding acute motor axonal neuropathy (AMAN) has not been yet associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. We herein report one patient who developed acute onset dysautonomia heralding AMAN during SARS-CoV-2 infection.

Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy characterized by rapidly progressive, essentially symmetric weakness and areflexia in a previously otherwise healthy child. It is the most common cause of acute flaccid paralysis in children, and its reported incidence is 1 to 2/100,000 population. Prior infection is a well-established predating event in GBS. The commonly recognized variants of GBS are acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy, and Miller–Fisher syndrome. AIDP is the most prevalent form. As Guillain–Barrè syndrome represents an important differential diagnosis in infancy with pronounced and progressive hypotonia, we herein report a case of AMAN in a 5-month-old male infant without known exposure to immunomodulating factors or infections.

Objectives To evaluate the outcome of Indian children with Guillain-Barre syndrome who received IV immunoglobulin compared with those who did not receive any specific therapy. Design Single center, prospective cross-sectional study. Setting Tertiary care neurology teaching hospital. Patients Children (≤ 18 yr old) with Guillain-Barre syndrome were included from a prospectively maintained Guillain-Barre syndrome registry from January 2008 to April 2017. Children were classified into acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor-sensory axonal neuropathy, and inexcitable motor nerves based on nerve conduction study. Interventions Out of 138 pediatric Guillain-Barre syndrome, 50 received IV immunoglobulin and another 50 age and peak disability matched controls (who did not receive IV immunoglobulin or plasmapheresis) were selected from the same registry for comparison. Measurements and main results Outcome at 3 and 6 months was defined on the basis of a 0-10 Clinical Grading Scale into complete (Clinical Grading Scale 5) recovery. The primary outcome was proportion of patients with complete recovery at 3 and 6 months in IV immunoglobulin and non-IV immunoglobulin groups. Secondary outcomes included in-hospital deaths, duration of mechanical ventilation, and hospital stay. Subgroup analysis was done in acute motor axonal neuropathy and acute inflammatory demyelinating polyradiculoneuropathy groups. The baseline characteristics were similar except for shorter duration of illness and higher proportion of facial palsy in IV immunoglobulin group. Hospital deaths, duration of mechanical ventilation, hospital stay, and outcome at 3 and 6 months were not different between the two groups. Children with acute motor axonal neuropathy had better recovery at 6 months on IV immunoglobulin (58.3% vs 11.1%; p = 0.03), but not those with acute inflammatory demyelinating polyradiculoneuropathy (58.3% vs 72.2%; p = 0.22). In nonambulatory Guillain-Barre syndrome children, complete recovery at 6 months was similar in IV immunoglobulin and non-IV immunoglobulin group (57.4% vs 57.1%; p = 0.98). Conclusions In Indian children with Guillain-Barre syndrome, the outcome at 6 months in IV immunoglobulin treated group was similar to non-IV immunoglobulin group. Children with acute motor axonal neuropathy responded better to IV immunoglobulin.

OBJECTIVE We report a prospective study analysing clinical characteristics, subtyping and prognosis in Guillain-Barre syndrome (GBS). METHOD The study was based on consecutive GBS patients admitted between 2009 and 2017. Disability was serially assessed using the GBS disability scale. RESULTS Fifty-six GBS patients were identified with an average age of 55 years (range, 5-86 years) and a male/female ratio of 2.1. The interval to nadir was

Guillain-Barré syndrome (GBS) is an acute monophasic immune-mediated polyradiculoneuropathy. Here, we report a case of a young man with acute motor axonal neuropathy (AMAN) subtype of GBS having hepatitis A virus (HAV) infection. A 30-year-old man with icterus was referred to emergency center of Razi Hospital. He complained of flu-like symptoms 10 days before the onset of icterus. Also, he suffered from gradual fatigue and weakness with dark urine. He experienced neurological symptoms of muscle paralysis (ascending from the legs to hands). Neurological consultant suspected GBS at the first step based on clinical examinations. He was candidate for five sessions of plasmapheresis. The ultrasonography revealed liver span 166 mm, which was greater than the normal range, with normal parenchymal echo. The gallbladder wall was thicker than normal and gallstone with lesion was not seen in different conditions. He was discharged after total improvement of neurological symptoms and muscular power. In addition, the results of International normalized ratio (INR), partial thromboplastin time (PTT), prothrombin time (PT), alkaline phosphatase (ALK), alanine aminotransferase (ALT) , aspartate aminotransferase (AST), bilirubin total and direct (Bil T, D) tests were normal after 2-month follow-up. Although, acute viral infections such as hepatitis E virus (HEV) is common in patients with GBS; the possibility of HAV infection in patients with its risk factor should not be neglected.

To compare the clinical profile and short-term outcome of children with axonal and demyelinating subtypes of childhood Guillain Barre syndrome (GBS). This is a prospective observational study conducted in a tertiary care teaching hospital in North India. Consecutive children with Guillain Barre 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.

Guillain–Barré syndrome (GBS) is an acute fatal progressive disease caused by autoimmune mechanism mainly affecting peripheral nervous system. Although the syndrome is clinically sub-classified into several variants, specific biomarker and exact pathomechanism of each subtypes are not well elucidated yet. In current study, integrative metabolomic and lipidomic profiles were acquisitioned from cerebrospinal fluid samples of 86 GBS from three variants and 20 disease controls. And the data were systematically compared to our previous result on inflammatory demyelination disorders of central nervous system (IDDs) and healthy controls. Primary metabolite profiles revealed unique metabolic traits in which 9 and 7 compounds were specifically changed in GBS and IDD, respectively. Next, the biomarker panel with 10 primary metabolites showed a fairly good discrimination power among 3 GBS subtypes, healthy controls, and disease controls (AUCs ranged 0.849–0.999). The robustness of the biomarker panel was vigorously validated by multi-step statistical evaluation. Subsequent lipidomics revealed GBS variant-specific alteration where the significant elevations of lyso-phosphatidylcholines and sphingomyelins were unique to AIDP (acute inflammatory demyelinating polyneuropathy) and AMAN (acute motor axonal neuropathy), respectively. And metabolome-wide multivariate correlation analysis identified potential clinical association between GBS disability scale (Hughes score) and CSF lipids (monoacylglycerols, and sphingomyelins). Finally, Bayesian network analysis of covarianced structures of primary metabolites and lipids proposed metabolic hub and potential biochemical linkage associated with the pathology.

textabstractObjective: TLR4 plays an important role in the pathogenesis of Guillain-Barre syndrome (GBS). The relationships between TLR4 polymorphisms and susceptibility to GBS are poorly understood. We investigated the frequency and assessed the association of two single nucleotide polymorphisms (SNPs) in the extracellular domain of TLR4 (Asp299Gly and Thr399Ile) with disease susceptibility and the clinical features of GBS in a Bangladeshi cohort. Methods: A total of 290 subjects were included in this study: 141 patients with GBS and 149 unrelated healthy controls. The TLR4 polymorphisms Asp299Gly and Thr399Ile were genotyped using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) assay. Results: The minor 299Gly allele was significantly associated with GBS susceptibility (P = 0.0137, OR = 1.97, 95% CI = 1.17–3.31), and was present at a significantly higher frequency in patients with the acute motor axonal neuropathy (AMAN) subtype of GBS (P = 0.0120, OR = 2.37, 95% CI = 1.26–4.47) than acute inflammatory demyelinating polyneuropathy (AIDP) subtype (P = 0.961, OR = 1.15, 95% CI = 0.38–3.48); when compared to healthy controls. The genotype frequency of the Asp299Gly polymorphism was not significantly different between patients with GBS and healthy controls. The Asp299-Thr399 haplotype was associated with a significantly lower risk of developing GBS (P = 0.0451, OR = 0.63, 95% CI = 0.40– 0.99). No association was observed between the Thr399Ile polymorphism and GBS disease susceptibility. Interpretation: The TLR4 minor 299Gly allele was associated with increased susceptibility to GBS and the axonal GBS subtype in the Bangladeshi population. However, no associations were observed between the genotypes of the Asp299Gly and Thr399Ile SNPs and antecedent C. jejuni infection or disease severity in Bangladeshi patients with GBS.

Our aim was to evaluate sensitivity and specificity of conduction studies parameters for prognosis and differential diag nosis in children with acute motor axonal neuropathy (AMAN) & acute inflammatory demyelinating polyneuropathy (AIDP). Methods. 40 children were included: 20 healthy controls (7-14 years) and 20 patients (8-15 years) with AIDP or AMAN. All underwent conduction studies on 3-7 day since the clinical symptoms onset. We registered and evaluated motor conduction velocity, compound muscle action potential (CMAP) amplitude of nn. tibialis, peroneus, medianus, ulnaris; sensory conduction velocity & sensory nerve action potential (SNAP) amplitude for nn. medianus, suralis, peroneus superficialis, ulnaris, H-reflex threshold & latency, reactivity of neural conductivity (RNC) in short-term hand ischemia in acute phase (3-14 day since the disease onset) and in early recovery period (15-30 day since the symptoms onset). ROC-analysis was performed. Results. In 95% of the patients with Guillain-Barré syndrome H-reflex was absent. In first 10 days SNAP amplitude of median nerve >8.9 µV, peroneal nerve >3.6 µV, CMAP of peroneal nerve ≤0,4 µV with normal motor conduction velocity indicates AMAN presence. Motor axons of peripheral nerves in children in acute and recovery phase of AIDP are resistant towards ischemia. Prognostic criteria for long period of walk recovery (more than 30 days) in these patients are RNC on 10th minute of local ischemia ≤2.5%, ulnar nerve CMAP amplitude ≤1,1 mV and distal CMAP amplitude from median nerve ≤1.6 mV. Conclusions. Conduction studies may be implemented on all phases of Guillain-Barré syndrome in children for prognosis and differential diagnosis between its axonal and demyelinating forms. H-reflex absence in children in the first 5 days of acute polyneuropathy may serve as additional diagnostic criteria for Guillain-Barré syndrome. RNC parameters may be implemented for the prognosis of the walk period recovery duration.

Myelin phagocytosis by macrophages has been an essential feature of demyelinating diseases in the central and peripheral nervous systems, including Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and multiple sclerosis (MS). The discovery of autoantibodies, including anti-ganglioside GM1 antibodies in the axonal form of GBS, anti-neurofascin 155 and anti-contactin 1 antibodies in typical and distal forms of CIDP, and anti-aquaporin 4 antibodies in neuromyelitis optica, contributed to the understanding of the disease process in a subpopulation of patients conventionally diagnosed with demyelinating diseases. However, patients with these antibodies are now considered to have independent disease entities, including acute motor axonal neuropathy, nodopathy or paranodopathy, and neuromyelitis optica spectrum disorder, because primary lesions in these diseases are distinct from those in conventional demyelinating diseases. Therefore, the mechanisms underlying demyelination caused by macrophages remain unclear. Electron microscopy studies revealed that macrophages destroy myelin as if they are the principal players in the demyelination process. Recent studies suggest that macrophages seem to select specific sites of myelinated fibers, including the nodes of Ranvier, paranodes, and internodes, for the initiation of demyelination in individual cases, indicating that specific components localized to these sites play an important role in the behavior of macrophages that initiate myelin phagocytosis. Along with the search for autoantibodies, the ultrastructural characterization of myelin phagocytosis by macrophages is a crucial step in understanding the pathophysiology of demyelinating diseases and for the future development of targeted therapies.

IntroductionAlthough acute transverse myelitis (ATM) is a rare neurological condition (1.34-4.6 cases per million/year) COVID-19-associated ATM cases have occurred during the pandemic.Case-finding methodsWe report a patient from Panama with SARS-CoV-2 infection complicated by ATM and present a comprehensive clinical review of 43 patients with COVID-19-associated ATM from 21 countries published from March 2020 to January 2021. In addition, 3 cases of ATM were reported as serious adverse events during the clinical trials of the COVID-19 vaccine ChAdOx1 nCoV-19 (AZD1222).ResultsAll patients had typical features of ATM with acute onset of paralysis, sensory level and sphincter deficits due to spinal cord lesions demonstrated by imaging. There were 23 males (53%) and 20 females (47%) ranging from ages 21- to 73- years-old (mean age, 49 years), with two peaks at 29 and 58 years, excluding 3 pediatric cases. The main clinical manifestations were quadriplegia (58%) and paraplegia (42%). MRI reports were available in 40 patients; localized ATM lesions affected ≤3 cord segments (12 cases, 30%) at cervical (5 cases) and thoracic cord levels (7 cases); 28 cases (70%) had longitudinally-extensive ATM (LEATM) involving ≥4 spinal cord segments (cervicothoracic in 18 cases and thoracolumbar-sacral in 10 patients). Acute disseminated encephalomyelitis (ADEM) occurred in 8 patients, mainly women (67%) ranging from 27- to 64-years-old. Three ATM patients also had blindness from myeloneuritis optica (MNO) and two more also had acute motor axonal neuropathy (AMAN).ConclusionsWe found ATM to be an unexpectedly frequent neurological complication of COVID-19. Most cases (68%) had a latency of 10 days to 6 weeks that may indicate post-infectious neurological complications mediated by the host’s response to the virus. In 32% a brief latency (15 hours to 5 days) suggested a direct neurotropic effect of SARS-CoV-2. The occurrence of 3 reported ATM adverse effects among 11,636 participants in the AZD1222 vaccine trials is extremely high considering a worldwide incidence of 0.5/million COVID-19-associated ATM cases found in this report. The pathogenesis of ATM remains unknown, but it is conceivable that SARS-CoV-2 antigens –perhaps also present in the AZD1222 COVID-19 vaccine or its chimpanzee adenovirus adjuvant– may induce immune mechanisms leading to the myelitis.

Aims and Objectives: To study clinical profile and outcome in Pediatric Guillain Barre Syndrome (GBS). Materials and Methods: Retrospective study of 30 patients (age 1 month to 12 years) admitted with GBS enrolled over a period of 2 years (August 2016-July 2018) from Department of Pediatrics of tertiary centre. Results: Mean age was 5.4 years (21 males; 9 females). Most common presenting complaints- symmetrical lower limb weakness (26 cases; 86.67% cases), respiratory complaints (6 cases; 20% cases), quadriparesis (4 cases; 13.33% cases) and facial palsy (2 cases; 6.67%). Antecedent illnesses- gastrointestinal (6 cases) and respiratory (3 cases). Two patients had varicella (in preceding one week) and one had mumps (one month prior to presentation). MRI-spine done in 12 patients; of whom 9 had features of GBS (thickening and contrast enhancement of the intrathecal and cauda equina nerve roots on T1 weighted MRI). Nerve conduction studies done in 16 patients, of which Acute Motor Axonal Neuropathy was seen in 10 cases. Intravenous immunoglobulin was given to 27 patients while 3 received methylprednisolone in addition. 90% patients receiving IVIG showed improvement. Sixteen patients were admitted to the intensive care unit and 7 required mechanical ventilation. Average hospital stay was 13 days. Two patients had recurrent episodes. Common complications included- pneumonia (6 cases; 2 aspiration and 4 ventilator associated) and autonomic disturbances (6 cases). Two patients died due to autonomic disturbances and presence of autonomic disturbances predicted higher mortality (P = 0.034). Conclusions: Gastrointestinal illness was common antecedent illness for GBS. Symmetrical lower limb weakness was commonest complaint. Pneumonia and autonomic disturbances were commonest complications. Presence of autonomic disturbances predicted higher mortality.

COVID-19-associated acute motor axonal neuropathy, a variant of Guillain-Barre Syndrome: Expanding the neurological manifestations of SARS-CoV-2

Background Guillain–Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy characterized by rapidly evolving weakness and areflexia, reaching nadir within 4 weeks. Data on the characteristic of GBS in Saudi Arabia are limited. This study aimed to describe the clinical, electrophysiological, and laboratory characteristics and outcome of a multicenter cohort of patients with GBS. Methods This is a retrospective multicenter nationwide study. Patients who had GBS, identified through Brighton Criteria, between January 2015 and December 2019 were included. Data collected included demographics, clinical features, cerebrospinal fluid profile, reported electrophysiological patterns, treatment, and outcome. Reported GBS subtypes were compared using chi-square, Fisher's exact, or Mann–Whitney U tests, as appropriate. Results A total of 156 patients with GBS were included (men, 61.5%), with a median age of 38 (interquartile range, 26.25–53.5) years. The most commonly reported antecedent illnesses were upper respiratory tract infection (39.1%) and diarrhea (27.8%). All but two patients (98.7%) had weakness, 64.1% had sensory symptoms, 43.1% had facial diplegia, 33.8% had oropharyngeal weakness, 12.4% had ophthalmoplegia, and 26.3% needed mechanical ventilation. Cytoalbuminological dissociation was observed in 69.1% of the patients. GBS-specific therapy was administered in 96.8% of the patients, of whom 88.1% had intravenous immunoglobulin, and 11.9% had plasmapheresis. Approximately half of the patients were able to walk independently within 9 months after discharge, and a third regained the ability to walk independently thereafter. Death of one patient was caused by septicemia. Acute inflammatory demyelinating polyradiculoneuropathy was the most commonly reported GBS subtype (37.7%), followed by acute motor axonal neuropathy (29.5%), and acute motor-sensory axonal neuropathy (19.2%). Conclusion The clinical and laboratory characteristics and outcome of GBS in the Arab population of Saudi Arabia are similar to the international cohorts. The overall prognosis is favorable.

Electrophysiological classification of Guillain-Barre syndrome (GBS) is important for predicting its clinical course; however, few reports discuss GBS patients who do not conform to the acute inflammatory demyelinating polyneuropathy (AIDP) or acute motor axonal neuropathy (AMAN) classifications. Therefore, the present study assessed the features of unclassified types of GBS and compared them to those of AIDP and AMAN. We compared clinical symptoms, nerve conduction, and laboratory data among patients with AIDP, AMAN, and unclassified subtypes of GBS, according to criteria developed by Rajabally, Hadden, and Ho. According to the Rajabally criteria, the F wave frequency in the upper and lower extremities was higher in the unclassified subgroup than in the AIDP and AMAN subgroups; however, according to the Hadden and Ho criteria, the F wave frequency in only the lower extremities was higher in the unclassified subgroup than in the other subgroups. The unclassified subgroup showed better prognosis using the Rajabally criteria. Classification with the Rajabally criteria is useful for predicting prognosis and determining treatment in patients with GBS. Moreover, unclassified patients exhibit the quickest recovery.

COVID-19 has become a pandemic. It affects multiple systems of the body including the nervous system. It invades the nervous systems through multiple routes - either olfactory tract, bloodstream (by binding to endothelial receptors) or via ACE-2 receptors in the brain. We report a case of Guillain-Barre syndrome (GBS) variant (acute motor axonal neuropathy (AMAN type)) associated with COVID-19 infection with positive polymerase chain reaction (PCR) test for COVID-19 and positive contact history with infected family member. GBS and its variants like AMAN can occur due to COVID-19 infection through its immune-mediated effects. Diagnosis of GBS should depend on the clinical and supportive criteria. The treatment should be started early to prevent progression and disease co-morbidities.