Acute Motor Sensory Axonal Neuropathy Research Articles

Guillain-Barré syndrome: History, pathogenesis, treatment, and future directions.

Since its description by Guillain, Barré, and Strohl in 1916, Guillain-Barré syndrome (GBS) has attracted a large literature. The author reviews the history of research into its pathogenesis and treatment to highlight promising avenues for future research. This is a nonsystematic personal review. Since the early 1900s, the clinical picture of GBS has been illustrated in multiple series culminating in the ongoing International Guillain-Barré Syndrome study of 2000 patients. In the 1950s and 1960s, the inflammatory nature of the commonest form, acute inflammatory demyelinating polyradiculoneuropathy (AIDP), was described. In the 1990s, two axonal forms, acute motor-sensory axonal neuropathy and acute motor axonal neuropathy, were recognized. In the 1990s and early 2000s, these forms were shown to be due to antibodies against Campylobacter jejuni glycans cross-reacting with glycolipids on axonal membranes. The pathogenesis of AIDP remains unknown, but T-cell responses to the compact myelin proteins, P2 and P0, which cause experimental autoimmune neuritis, suggest that T cells are important. Randomized controlled trials in the 1970s and 1980s showed no benefit from corticosteroids. Trials in the 1980s showed benefit from plasma exchange and in the 1990s from intravenous immunoglobulin. Future research should seek biomarkers to identify subgroups with different treatment responses, define the true natural history of the disease with population-based epidemiological studies, study the pathology in autopsies early in the disease, seek causative antibodies and confirm autoimmune T-cell responses in AIDP, and expand treatment trials to include anti-T-cell agents.

Pattern of Clinical Profile and Outcome of Gillian Barre Syndrome in Sudanese Patients

Introduction: Guillian Barre'-Syndrome (GBS) is an acute, autoimmune disease affecting spinal roots and peripheral nerves, characterized by a rapidly progressive, ascending weakness of the extremity, trunk and even respiratory and facial muscles and with minor sensory and autonomic dysfunction. As in Sudan there is no enough data regarding the pattern of presentation and outcome of GBS, our research aimed to study clinical profile, electrophysiological variant, and outcome of Sudanese patients diagnosed with (GBS). Methods: A multi centers cross-sectional hospital-based study conducted in Khartoum from March to September 2020, 62 patients enrolled in the study, structured questionnaire consists of personal data, clinical history, examination findings, electrophysiological study result, management and outcome is used to assess the clinical profile and outcome of the disease. Results: The study revealed that males and females are equally represented, 70% patients are between 18 years and 50 years. The onset of the weakness was less than or equal 3 days in 33.9% of patients, from 4 to 7 days in 41.9%, from 8 to 14 days in 11.3% and above 14 days in 12.9%.40.3% patients had Evidence of autonomic dysfunction. The majority of patients presented with weakness that started in the lower limbs (93.5%), in (4.8%) the weakness started in the upper limbs and then descend. In (1.6%) the weakness started in the pharyngeal and respiratory muscles from the start. Sensory complains were observed in 54 patients (87.1%), 3 patients (4.8%) developed urinary bladder incontinence and 36 patients (58.1%) had back pain at the onset of the disease. On clinical examination; (98.4%) of patients had absent reflexes, (35.5%) had bilateral facial nerve palsy and (4.8%) had unilateral facial nerve pals. Based on the electrophysiological studies, (37.09 %) were recorded as acute motor axonal neuropathy (AMAN), (29.03%) as acute inflammatory demyelinating poly neuropathy (AIDP) and (29.03%) as acute motor-sensory axonal neuropathy (AMSAN). Regarding the outcome, 30 patients (48.4%) fully recovered, 25 patients (40.3%) recovered with motor deficit, and 7 patients (11.3%) unfortunately died. Conclusion: This study concluded that Guillian Barre'-Syndrome (GBS) in Sudanese population mainly affects age group from 18 to 50 years with variable maximum onset of weakness, presentation and outcome. Lower limb weakness and absent reflexes were the most presenting features. NCS showed axonal type of GBS found to be in nearly 66% of patients and 40% showed clinical evidence of dysautonomia.

Dengue-induced Guillain–Barre syndrome: a case series

BackgroundDengue virus is an RNA virus that is associated with a myriad of neurological manifestations. Less than 5% of patients develop neuromuscular complications. Guillain–Barre syndrome (GBS) is an uncommon neurological sequelae of dengue fever. Studies have shown acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) to be the most common variants associated with dengue fever in Asian countries. Very few cases have been reported from Pakistan to date.Case presentationWe present four adult patients with dengue-associated GBS. The time interval between onset of symptoms of dengue fever and development of lower limb weakness in all patients was between 6 and 20 days. Dengue was diagnosed based on either serology or dengue NS1 antigen testing. Acute inflammatory demyelinating polyneuropathy (AIDP) (n = 1), AMAN (n = 1), and AMSAN (n = 2) variants were identified. One patient had a coexisting myopathy which has not been previously reported. All patients showed good recovery after treatment with plasmapheresis or intravenous immunoglobulins.ConclusionsOur case series is valuable in contributing to the limited pool of reported cases of dengue fever complicated by GBS. Early detection of new onset symmetrical limb weakness during or after a dengue infection is imperative for early treatment and to limit disability.

Guillain-Barre Syndrome, Clinical Spectrum and Electrophysiological Sub Types: A Local Experience

Background: Guillain-Barre syndrome is uncommon neurological disease that occurs in acute phase as generalized polyradiculo-neuropathy. It has multiple variants some of them being uncommon and rarely encountered. Objective: The objective of the study was to determine the frequency and clinical characteristics of Guillain-Barre syndrome variants. Methods: A cross-sectional study was carried out in neurology department, Mayo hospital from July to December 2022. Patients clinically diagnosed as Guillain-Barre syndrome by using Brighton criteria were included. All the patients underwent electro diagnostic procedures and they were categorized into five basic variants i.e., Acute motor axonal neuropathy, Acute motor sensory axonal neuropathy, acute inflammatory demyelinating polyradiculo-neuropathy, acute sensory axonal neuropathy and mixed variety acute polyradiculo-neuropathy. Results: Out of total 85 patients, males were 56 (65.9%) and 29 (34.1%) were females. Adults were more affected with a mean age of 36±15.3. Frequency of acute motor axonal neuropathy (35.3%) was high followed by acute motor sensory axonal neuropathy (31.8%), mixed variety acute polyradiculo-neuropathy (16.5%), acute inflammatory demyelinating polyradiculo-neuropathy (12.9%) and acute sensory axonal neuropathy (3.5%) respectively. Conclusion: Acute motor axonal neuropathy is frequently encountered Guillain-Barre syndrome variant in our setup. Also, the frequency of acute sensory axonal neuropathy and mixed variety acute polyradiculo-neuropathy has increased with a declining trend of acute inflammatory demyelinating polyradiculo-neuropathy.

Pattern of Autonomic Involvement in Adult Patients with Guillain Barre Syndrome in a Tertiary Hospital

Background: Guillain Barre Syndrome (GBS) is an acute post infectious immune mediated peripheral neuropathy with a marked variation in pathology, clinical presentation and prognosis. Autonomic dysfunction is one of the important manifestations of GBS which may lead to significant morbidity and mortality. Objective: The aim of the study is to assess the autonomic involvement, to determine its frequency and pattern of involvement in adult patients with GBS Methods: An observational, descriptive, cross sectional study was carried out in the Department of Neurology, BSMMU, Dhaka from March, 2015 to September, 2017. Total 43 patients of GBS and 35 apparently healthy controls were recruited as the study population. On the basis of nerve conduction study patients were classified into different groups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) and other variants. Disability status at the time of autonomic testing was measured by Hughes functional grading scale. The following tests of autonomic nervous system were performed in both patients and controls 1) resting heart rate and heart rate on changing posture (30: 15 ratio) 2) supine blood pressure and blood pressure on changing posture 3) heart rate response to valsalva maneuver 4) heart rate response to deep breathing and E: I ratio 5) sphincter disturbance by symptoms questionnaire. Results: The mean age of patients was 35±12 years (range18 to 65 years) and 58.1% were male. Around 88.4 % of patients showed some sort of autonomic dysfunction. Variation of heart rate by different maneuver like posture change, deep breathing and valsalva maneuver was found commonly. Among them 30:15 ratio was abnormal in majority of the patients (82.4%) followed by abnormal max-min HR/min (58.1%) and abnormal valsalva ratio (37.2%). Other abnormalities were postural hypotension (38.2%), sinus tachycardia (25.6%), hypertension (16.3%), hypotension (2.7%), sinus arrhythmia (4.7%), constipation (30%), urinary retention (7%) and urinary incontinence (4.7%). Conclusion: In this study different patterns of autonomic dysfunction was found in 88.4% of patients with GBS involving both sympathetic and parasympathetic components. The present study found no significant association between autonomic dysfunction and motor disability scores. Thus autonomic function assessment is essential in every patient with GBS in addition to motor & sensory function. Bangladesh Medical Res Counc Bull 2022; 48(3): 174-179

Guillain-Barre Syndrome linked to SARS-CoV-2 infection – meta-analysis and literature review

Background
 The novel coronavirus disease-2019 (COVID-19), which is caused by Severe Acute Respiratory Distress Syndrome coronavirus 2 (SARS-CoV2), was originally detected in Wuhan, China in December 2019. In this meta-analysis and literature review, we compared and summarized the clinical presentation, cerebrospinal fluid (CSF) and electromyography (EMG) findings and outcomes in SARS-CoV-2 patients with Guillain-Barre Syndrome (GBS) and its variants.
 Methods
 We conducted a literature review in February 2023 searching for terms “Guillain-Barre Syndrome and COVID-19", “SARS neurology”, “COVID-19 complications”. We used PubMed and Google Scholar databases inquiring case reports or series of cases published between April 1, 2020, and September 14, 2023.
 Results
 Of the 52 GBS cases 61,5% (n=32) were male and 39,5% (n=20) were female. The mean age was 57 years old. A total of 75% (n=33) patients presented acute inflammatory demyelinating polyneuropathy (AIDP) variant, 6,8% (n=3) presented acute motor axonal neuropathy (AMAN) variant, 15,9% (n=7) presented acute motor-sensory axonal neuropathy (AMSAN) variant. A total of 85,7% (n=42) of patients were diagnosed with albuminocytological dissociation. During the hospitalization, a total of 30,8% (n=16) required mechanical ventilation. A total of 61,5% (n=32) of patients were treated with a 5-day regimen of intravenous immunoglobulin (IVIG) in dose 0.4 g/kg/day. There were 46,1% (n=24) complete recoveries from GBS, 32,7% (n=17) partial recoveries and 9,6% (n=5) of patients did not respond to treatment. A total of 11,5% (n=6) of patients died.
 Conclusion
 It is crucial to follow patients with COVID-19 and GBS over time to estimate properly the efficacy of treatment and evaluate the real percentage of recovery and complications.

A Case of Acute Motor Sensory Axonal Neuropathy, a Rare Form of Guillain-Barre Syndrome, Developing After Multiple COVID-19 Infections (P13-8.009)

<h3>Objective:</h3> Guillain-Barre syndrome (GBS), an autoimmune polyneuropathy characterized by ascending weakness and characteristic cerebrospinal fluid (CSF) findings, has been identified in patients previously diagnosed with COVID-19, treated for COVID-19 infection, or vaccinated against the same. GBS is divided into axonal and demyelinating subtypes, and confirmatory testing such as electromyography and nerve conduction studies can help differentiate between subtypes. Here we present a case with recurrent COVID-19 infections, most recently treated with bebtelovimab, who developed acute motor sensory axonal neuropathy (AMSAN), a rare variant of axonal GBS. <h3>Background:</h3> A 33-year-old female presented with progressive ascending weakness and sensory loss that started two weeks after COVID-19 infection. She was fully vaccinated, however, had four episodes of COVID-19 infection within the past year and a half. Two weeks after the most recent infection, which was treated with bebtelovimab, she developed rapidly progressive weakness and sensory impairment in bilateral lower extremities quickly involving upper extremities. She received two rounds of intravenous immunoglobulin (IVIG) three and five weeks after symptoms onset. Her symptoms continued to progress requiring hospitalization. Exam showed severe weakness in all limbs with distal muscle atrophy in upper extremities. Imaging was negative. CSF demostrated albuminocytological dissociation. Nerve conduction studies concluded severe motor and sensory axonal neuropathy with active denervation which led to the suspicion of Acute Motor and Sensory Axonal Neuropathy (AMSAN), a rare variant of GBS. She underwent plasmapheresis with only minimal improvement. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> N/A <h3>Conclusions:</h3> Many cases of GBS have been reported after COVID-19 infection but only a few of them are about AMSAN. This case presents unique features including multiple COVID-19 infections despite prior vaccination, and the development of this rare variant of GBS. It raises the concern of multiple COVID-19 infections having a cumulative impact on peripheral nervous system leading to more severe forms of GBS with poor response to treatment. <b>Disclosure:</b> Dr. Narvaez Caicedo has nothing to disclose. Mr. Rao has nothing to disclose. Dr. Patel has nothing to disclose. Dr. Wu has nothing to disclose.

Guillain-Barré Syndrome Mimicking Brainstem Death Following Traumatic Intracerebral Hemorrhage (P12-7.006)

<h3>Objective:</h3> NA <h3>Background:</h3> NA <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 73-year-old man attended the ED following an assault where he sustained multiple traumatic blows to the head. He was alert, complained of headache but provided a detailed account. There were facial injuries but no clinical signs of cranial or skull base fractures. There was injury to the right eye, in which he had pre-existing visual impairment from previous injury. Vision was normal in the left. Right pupillary light reflex was absent but left was normal. The remainder of the neurological examination was unremarkable. CT-Head identified a small traumatic subarachnoid hemorrhage over the left cerebral convexity and a small contusion in the left temporo-parietal region. Vitreous hemorrhage was evident in the right eye. Clinically stable, follow-up CT on day 3 showed small intra-cerebral haemorrhage within the contusion, with minimal mass effect. However, on day 8, his GCS dropped and he was admitted to the ITU for airway management. Both pupils were non-responsive to light. Expansion of haemorrhage was suspected, however repeat CT was stable. EEG showed slowing over the left hemisphere without epileptiform activity. MRI excluded alternative causes for his neurological status such as brainstem ischaemia. With no clinical improvement, formal brainstem death testing was initiated. No potential confounders were recognised. The pupillary, corneal, oculocephalic, cough and gag reflexes were absent. Unexpectedly, deep tendon reflexes were absent in all limbs. A peripheral nerve stimulator using a train-of-four test protocol found no response. Post-traumatic Guillain-Barré syndrome (GBS) was suspected. Intravenous immunoglobulin was commenced. Nerve conduction studies found absent motor and sensory responses in all limbs. EMG showed fibrillation potentials and sharp waves. These findings were consistent with acute motor-sensory axonal neuropathy (AMSAN) variant of GBS. Serum Anti-GM1 antibodies were identified. After multiple rounds of plasmapheresis the patient was weaned from the ventilator. Nevertheless, severe neurological disability remained. <h3>Conclusions:</h3> NA <b>Disclosure:</b> Dr. Finegan has nothing to disclose. Dr. Ryan has nothing to disclose. Michael J. Hennessy has nothing to disclose. Kevin Murphy has nothing to disclose. Dr. Crowley has nothing to disclose. Dr. Elamin has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche .

Is Guillain–Barre syndrome following chickenpox a parainfectious disease? A case report and literature review

BackgroundPolyradiculoneuropathy following infection with varicella zoster virus (VZV) is rare and most of the time, happens in the context of reactivation of latent VZV. We report a case of acute polyradiculoneuropathy following primary infection with VZV marked by atypical clinical features raising the hypothesis of a para-infectious disease.Case presentationWe describe a 43-years-old male who developed ataxia, dysphagia, dysphonia, and oculomotor disorders (vertical binocular diplopia and bilateral ptosis) followed by quadriplegia with areflexia which occurred 4 days later. The patient had a history of varicella that occurred 10 days before the onset of these symptoms. Nerve conduction study revealed features consistent with an acute motor-sensory axonal neuropathy (AMSAN). Anti-ganglioside antibodies were negative.Based on clinical presentation and ancillary examination, we retain the Miller Fisher/Guillain-Barré overlap syndrome diagnosis.The patient was treated with high doses of methylprednisolone but the evolution of the disease was nevertheless marked by a complete recovery six weeks after onset of symptoms.ConclusionGBS following varicella is a rare but severe disease occurring most often in adults and marked by greater involvement of the cranial nerves. Its clinical features suggest that it is a para-infectious disease. Antiviral therapy has no effect on the course of the disease but its administration within the first 24 h after the onset of chickenpox in adults can prevent its occurrence.

Miller Fisher Syndrome Induced by Chemotherapy in Known Case of Acute Lymphocytic Leukaemia: A Case Report

Introduction: Guillain-Barre Syndrome (GBS) is an acute-onset autoimmune-mediated neuropathy. Guillain-Barre Syndrome can be divided into three subtypes: acute inflammatory demyelinating poly-radiculo-neuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). About 20% of patients with GBS develop respiratory failure and require mechanical ventilation. We are presenting a variant of GBS (Miller Fisher Syndrome, or MFS), which has been confirmed by nerve conduction studies along with the triad of ophthalmoplegia, ataxia, and areflexia. The objective of this study is to present a rare case of chemotherapy-induced GBS. Important clinic findings: A 25-year-old gentleman with acute lymphocytic leukemia on active chemotherapy treatment presented with lower limb weakness. This weakness started after his fifth chemotherapy session. After the sixth chemotherapy, he developed complete paralysis of the left lower limb. Later, he developed right lower limb paralysis. He was also complaining of eye dryness and incomplete closure of both eyes. While inpatient, he developed upper-limb weakness. His chemotherapy consisted of MESNA, cyclophosphamide, doxorubicin, vincristine, cyorabine, and methotrexate. He had ptosis and ophthalmoplegia in the left abducent and right oculomotor regions. He had bilateral facial nerve palsy. He was hypotonic with power grade 3 in the upper limbs and grade 0 in the lower limbs with areflexia. His sensation was intact in the upper limbs but lost in the lower limbs. His planter reflexes were mute. Diagnoses and Management: Intravenous immunoglobulins were given for 5 days. A nerve conduction study showed severe demyelinating sensorimotor polyradoculoneuropathy with secondary axonal loss. The triad of ataxia, ophthalmoplegia, and areflexia was consistent with MFS. The patient improved over the course of the hospital stay but did not reach full recovery. Conclusion: Although GBS is uncommon, it must be taken into account when making a differential diagnosis for any patient presenting with progressive weakness. Drug history is important in all GBS cases.

Guillain-Barré Syndrome in Children: A Narrative Review

Among the most typical reason of sudden, acquired weakness is Guillain-Barré Syndrome characterized by symmetrical ascending paralysis which is frequently sparked by an earlier illness. “Acute Inflammatory Demyelinating Polyneuropathy,” one of the prevailing kinds of Guillian-Barre Syndrome, is divided into four subtypes: acute inflammatory demyelinating polyradiculoneuropathy, Miller Fisher syndrome, acute motor sensory axonal neuropathy, and acute motor axonal neuropathy. Guillain-Barré syndrome can happen at any age, but it is frequently seen in persons over 40 years. More often, males are impacted. This syndrome lasts for 4 weeks, but if it is prolonged than that, it can be categorized as subacute, and if it lasts longer than 8 weeks, it can be classified as chronic. An antecedent infection is present in 70% of patients. The most frequent trigger for Guillain-Barré Syndrome is Campylobacter jejuni, along with the Cytomegalovirus, Zika virus, mycoplasma, hepatitis A and B, and human immunodeficiency virus. The antecedent infection generally occurs 1 to 3 weeks before. The pathogenesis of the disease solely based on the molecular mimicry. In some circumstances, collapse of respiratory system and autonomic dysfunction might make Guillain-Barré Syndrome more difficult to treat.

Clinical subtypes, seasonality, and short-term prognosis of Guillain-Barré syndrome in an Eastern city of Turkey

Background &amp; Objective: This study aimed to analyze the frequency Guillain-Barré syndrome (GBS) subtypes and their relationship with clinical characteristics, seasonal variations and early prognosis in Van City, Turkey. Methods: Patients with GBS who were admitted between January 2007 and December 2017 and diagnosed with acute inflammatory demyelinating neuropathy (AIDP), acute motor axonal neuropathy (AMAN) or acute motor sensory axonal neuropathy (AMSAN) were reviewed. Demographics, season of clinical onset, history and type of preceding infection, the Hughes Disability Score (HDS) at admission and discharge were recorded. Results: Of a total 100 patients, 51% was diagnosed with AIDP, 25% with AMAN and the remaining 24% with AMSAN subtype. The most common seasonal onset was during the spring (34%), followed by the fall (30%). The history of gastroenteritis (GE) was present in 26% of the patients and these patients were more likely to have AMAN and AMSAN subtypes. HDS on admission and at discharge were significantly higher in patients with AMAN and AMSAN compared to those with AIDP (p=0.003 and p&lt;0.001, respectively). The most important predictor of poor outcome at discharge was HDS on admission explaining between 50% and 80% of the total variance. Conclusion: There is a high prevalence of AMAN and AMSAN subtypes in Eastern region of Turkey. The history of GE, which is also commonly found in patients with GBS in this region, is more likely detected in patients with AMAN and AMSAN subtypes. Finally, clinical severity on admission is the most important contributor to clinical outcome at discharge.

Disease profile of Guillain-Barré syndrome among Indian patients receiving intravenous immunoglobulin: a real-world observational study

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Objectives of the study were to evaluate the real-world usage pattern of intravenous immunoglobulin (IVIg) for the treatment of patients with Guillain‐Barré syndrome (GBS), and to understand the disease characteristics and demographic patterns of these patients.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; This real-world, retrospective, analysis included data of patients with GBS who received IVIg treatment at various centers across India. The study data was collected between April 2021 and March 2022. &lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; A total of 3064 patients with GBS who received IVIg treatment were included. The mean (SD) age of the patients was 48.97 (14.97) years, and majority of the patients were men (68.8%). Acute inflammatory demyelinating polyneuropathy (AIDP) was the most common subtype of GBS (60.5%), followed by acute motor axonal neuropathy (AMAN; 11.6%), and acute motor sensory axonal neuropathy (AMSAN; 3%). The majority (&amp;gt;94%) of patients received IVIg therapy as first-line treatment. A large proportion of the patients (n=2402, 78.4%) were given the standard dose of 2 g/kg bodyweight (given over 5 days) and 97.1% (n=2974) of the total study population received IVIg regimen over the standard protocol of 5 days.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; In the clinical spectrum of GBS, AIDP was the most common subtype. IVIg was used at generally recommended dose and duration in patients with GBS.&lt;/p&gt;

Nationwide survey of childhood Guillain-Barré syndrome, Fisher syndrome, and Bickerstaff brainstem encephalitis in Japan

ObjectiveGuillain-Barré syndrome (GBS), Fisher syndrome (FS), and Bickerstaff brainstem encephalitis (BBE) are immune-mediated neuropathies presenting with symptoms such as weakness, ophthalmoplegia, ataxia, and consciousness disturbances. Although the epidemiology of GBS and BBE in patients of all ages has been reported, childhood data have not been well-investigated. We aimed to determine the clinical features, therapeutics, and prognoses of childhood GBS, FS, and BBE in Japan. MethodsWe sent questionnaires to 1068 pediatric neurologists in Japan from 2014 to 2016 to determine the number of children less than 15 years old with GBS, FS, or BBE and their age and sex. We subsequently performed a secondary survey to investigate the clinical features, laboratory data, treatment, and prognosis. ResultsFive-hundred thirty-eight pediatric neurology specialists (50.4%) responded to the first survey. The total number of children with GBS, FS, and BBE in Japan from 2014 to 2016 were 87, 10, and 6, respectively. GBS was classified as acute inflammatory demyelinating neuropathy (35.6%), acute motor axonal neuropathy (20.7%), or acute motor-sensory axonal neuropathy (10.3%), with a male-to-female ratio of 1.29:1.0 and a wide distribution of onset ages. The disease severities of GBS, FS, and BBE were variable, but all children could walk within one year. ConclusionThe prognoses of childhood GBS, FS, and BBE were generally favorable, as long as the patient was promptly treated with either intravenous immunoglobulin or plasma exchange.

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.

Clinical and pathological findings in neurolymphomatosis: Preliminary association with gene expression profiles in sural nerves.

Although inflammation appears to play a role in neurolymphomatosis (NL), the mechanisms leading to degeneration in the peripheral nervous system are poorly understood. The purpose of this exploratory study was to identify molecular pathways underlying NL pathogenesis, combining clinical and neuropathological investigation with gene expression (GE) studies. We characterized the clinical and pathological features of eight patients with NL. We further analysed GE changes in sural nerve biopsies obtained from a subgroup of NL patients (n=3) and thirteen patients with inflammatory neuropathies as neuropathic controls. Based on the neuropathic symptoms and signs, NL patients were classified into three forms of neuropathy: chronic symmetrical sensorimotor polyneuropathy (SMPN, n=3), multiple mononeuropathy (MN, n=4) and acute motor-sensory axonal neuropathy (AMSAN, n=1). Predominantly diffuse malignant cells infiltration of epineurium was present in chronic SMPN, whereas endoneurial perivascular cells invasion was observed in MN. In contrast, diffuse endoneurium malignant cells localization occurred in AMSAN. We identified alterations in the expression of 1266 genes, with 115 up-regulated and 1151 down-regulated genes, which were mainly associated with ribosomal proteins (RP) and olfactory receptors (OR) signaling pathways, respectively. Among the top up-regulated genes were actin alpha 1 skeletal muscle (ACTA1) and desmin (DES). Similarly, in NL nerves ACTA1, DES and several RPs were highly expressed, associated with endothelial cells and pericytes abnormalities. Peripheral nerve involvement may be due to conversion towards a more aggressive phenotype, potentially explaining the poor prognosis. The candidate genes reported in this study may be a source of clinical biomarkers for NL.

Assessment of neurological sequelae and new-onset symptoms in the long-term follow-up of paediatric Guillain-Barre syndrome: A longitudinal study from India.

Guillain-Barre syndrome (GBS) is the commonest cause of acute flaccid paralysis in children. There is a paucity of studies that assess the long-term outcome of paediatric GBS. To assess the frequency of neurological sequelae and the new-onset symptoms in the long-term follow-up of paediatric GBS and to identify the risk factors associated with them. This longitudinal study involved 78 children with GBS treated between January 2015 and 2021. The parents of those children were contacted to visit the hospital for a detailed neurological examination and to look for new-onset symptoms after the initial treatment for GBS. Of the 78 children, acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, and acute motor-sensory axonal neuropathy variants were observed in 30 (38.5%), 27 (34.6%) and 11 (14.1%) children, respectively. The median (interquartile range (IQR)) duration of follow-up was 3 (2, 4.5) years. The median (IQR) time to independent ambulation was 30 (13.5, 105) days. The neurological sequelae were found in 22 (28.2%) children. GBS disability score at admission (odds ratio (OR)=4.6; 95% confidence interval (CI): 1.1-19.8; P=0.04) and axonal variant of GBS (OR=4.1; 95% CI: 1.5-20.8; P=0.04) were found to be independent predictors of neurologic sequelae. A total of 28 children experienced new-onset symptoms after GBS, with frequent falls while running and fatigue being the predominant symptoms. Those children with demyelinating variant achieved independent ambulation earlier than the axonal group on survival analysis (log-rank P value=0.04). The presence of neurological sequelae and new-onset symptoms were found in 28.2 and 35% of the GBS children, respectively. High GBS disability score at admission and axonal variant of GBS were independent predictors of neurological sequelae. Knowledge about these would help in devising a plan for rehabilitation.

Prognostic Implications of Early Albuminocytological Dissociation in Guillain-Barré Syndrome.

Half of Guillain-Barré syndrome (GBS) present elevated cerebrospinal fluid (CSF) protein levels within 1 week since symptom onset and 80% within 2 weeks. Our objective was to determine the clinical and prognostic implication of albuminocytological dissociation in early GBS. An ambispective cohort study was conducted. Good outcome was considered if the patient was able to walk unaided (Guillain-Barré disability score [GDS] ≤ 2 points) at 3-month follow-up. Patients were classified into two groups: with and without albuminocytological dissociation; we compared clinical and paraclinic characteristics between the groups. We analyzed clinical and electrophysiological factors related to presenting early dissociation through a multivariate model. We included 240 patients who fulfilled Asbury criteria for GBS. On further selection, only 94 patients fulfilled inclusion. Mean age was 45.94 ± 17.1 years and 67% were male. Median time from symptom onset to admission was 5 days (IQR 3-6). Regarding albuminocytological dissociation and electrophysiological variants, we found a significant difference: acute inflammatory demyelinating polyneuropathy (AIDP) [60.6% vs 26.2%, p = 0.002], acute motor axonal neuropathy (AMAN) [21.2% vs 49.1%, p = 0.009] and acute motor sensory axonal neuropathy (AMSAN) [12.1% vs 1.6%, p = 0.05]. We did not observe significant differences in recovery of independent walking in short term between both groups. The presence of conduction block in any variant (OR 3.21, 95% CI 1.12-9.16, p = 0.02) and absence of sural registration (OR 5.69, 95% CI 1.48-21.83, p = 0.011) were independent factors related to early dissociation. Early dissociation (<7 days) is not associated with any particular clinical feature or unfavorable outcome. It is more common to see in AIDP rather than axonal variants.

Outcomes in Guillain-Barré Syndrome following a second therapeutic cycle – A single-centre retrospective observational study

IntroductionThe treatment of Guillain-Barré Syndrome (GBS) with intravenous immunoglobulin (IVIg) or plasma exchange (PE) reduces time to clinical recovery. Although sometimes used in clinical practice, the benefit of a second treatment cycle is of unproven benefit. AimsOur aim was to compare GBS prognosis in patients treated with one or two cycles of IVIg or PE. MethodsWe selected patients with electrophysiological studies compatible with acute inflammatory demyelinating polyneuropathy or acute motor-sensory axonal neuropathy, from January 2018 to December 2020 in our hospital. Our primary outcome was any improvement in the Guillain-Barré Syndrome Disability Score (GBS-DS) at a mean of twelve weeks. We compared patients treated with one or two treatment cycles with a binary regression. ResultsWe included twenty-six patients, 65.4% with the classical presentation and 30.8% were treated with two cycles. Patients treated with two cycles presented a higher basal GBS-DS (median 4; IQR 1–5) compared with the group of patients treated with one cycle (median 3; IQR 1–5), p = 0.01. The remaining basal characteristics were similar between groups. The two-cycle treatment regimen did not associate with an improvement in GBS-DS (OR 0.28, 95% CI 0.03–2.35, p = 0.24). Likewise there was no benefit in the need for intensive care unit (OR 2.0, 95% CI 0.37–10.92, p = 0.42) or mechanical invasive ventilation (OR 10.2, 95% CI 0.86–120.96, p = 0.66). DiscussionOur analysis reinforces the recent literature data regarding the absence of benefit of two treatment cycles in patients with GBS.

Unusual phenotype of acute motor sensory axonal neuropathy with ophthalmoplegia, and anti‐GD1a , ‐ GD1b and ‐ GM1 antibodies

Background Acute motor sensory axonal neuropathy (AMSAN) is a severe form of Guillain–Barré syndrome, which typically is not related to ocular palsy, and is pathologically associated with antibodies against GM1 and GD1a gangliosides. Case presentation We present a patient with a severe, drug-resistant form of AMSAN with ophthalmoplegia related to antiganglioside (anti-GD1a, -GD1b, -GM1) antibodies in the serum and cerebrospinal fluid, and with a poor long-term outcome. Conclusions Rare cases of AMSAN with ophthalmoplegia are described in the literature. The present patient is a case of an unusual clinical phenotype of AMSAN, the characteristics of which warn of a serious clinical course that requires timely aggressive treatment.