Inflammatory Polyradiculoneuropathy Research Articles

A rare case of paraneoplastic Guillain-Barré syndrome in a patient with endometrial cancer

Introduction: Guillain-Barré syndrome is an acute, inflammatory polyradiculoneuropathy of autoimmune aetiology. It is a rare disease seen in 1 in 100,000 person-years. Up to 20% of those affected develop severe disability; mortality in Guillain-Barré syndrome is 5%. Guillain-Barré, associated with many malignancies as a paraneoplastic phenomenon, has been reported – especially in haematological malignancies such as lymphoma and leukaemia. Solid tumours associated with paraneoplastic Guillain-Barré syndrome are breast and lung cancers. The association between paraneoplastic Guillain-Barré syndrome and gynaecological malignancies are rare, and only a handful of cases have been previously reported in gynaecological cancers. Case description: We discuss a 65-year-old Sri Lankan female patient diagnosed with metastatic endometrial carcinoma who presented with paraneoplastic Guillain-Barré syndrome. The patient was treated appropriately and eventually recovered from her condition. Conclusion: Paraneoplastic Guillain-Barré syndrome is a rare phenomenon that clinicians can easily miss, and it has rarely been described in gynaecological cancers. Our patient was diagnosed with this rare phenomenon. The timely recognition and prompt treatment of this potentially life-threatening condition with multiple complications is essential in managing patients with malignancies and neuropathy. Further studies on paraneoplastic Guillain-Barré syndrome are needed as cases may be underreported.

Guillain-Barré syndrome associated with COVID-19 infection: A case report

Coronavirus 2 or SARS-CoV-2 is predominantly manifests as a respiratory infection; however, it has been linked to a spectrum of neurological disorders such as vertigo, cephalalgia, cognitive impairment, myalgia, and anosmia. Emerging reports indicate several neurological illnesses that coincide with COVID-19, such as encephalitis and, notably, stroke. In this report, We present an instance of a patient infected with COVID-19 with Guillain-Barré Syndrome in this case report. Guillain-Barre Syndrome (GBS) is an inflammatory polyradiculoneuropathy linked to various viral infections. We present the case of a 76-year-old female patient hospitalized with acute progressive symmetric ascending quadriparesis. Seven days before hospitalization, the patient experienced fever and cough with RT-PCR testing confirmed a positive COVID-19 infection. Cerebrospinal fluid analysis revealed dissociation of cytoalbumin. Coronavirus stimulates inflammatory cells and produces various cytokines, leading to immune-mediated processes. GBS represents an immune-mediated disorder, with its pathogenesis intricately influenced by the pivotal role of molecular mimicry in its onset. Plasmapheresis was performed, resulting in an improved clinical outcome for the patient.

Cytomegalovirus-related Guillain-Barre syndrome in an adult liver transplant recipient: a case report

Guillain-Barré syndrome (GBS) is an immune-mediated condition affecting both the motor and sensory spinal roots and peripheric nerves. GBS is a rare entity, with its global incidence being as low as 0.81 to 1.89 cases per 100.000 person-year. Despite its low incidence, GBS have an important morbidity and lethal potential. This syndrome is usually triggered by infections. Due to its clinical presentation heterogeneity and its disease natural history, there is scarse availability of international clinical practice guidelines for the management of this entity. In this report, we present the case of a 67 years-old man on his fifth month after liver transplant (LT), who developed acute non-inflammatory diarrhea followed by sudden ascending symmetric paresia and soles paresthesias. Electrophysiologic studies reported a demyelinating inflammatory polyradiculoneuropathy compatible with GBS, acute severe inflammatory demyelinating polyneuropathy (AIDP) subtype. His neurolo¬gy deficit was associated with a simultaneous primary Cytomegalovirus (CMV) infection, one of the most common GBS-related infectious agents.

Clinical features and maternal and fetal outcomes in women with Guillain-Barré syndrome in pregnancy.

Guillain-Barre syndrome (GBS) is an acute inflammatory polyradiculoneuropathy rarely observed during pregnancy. In this retrospective study, we analyzed the characteristics of pregnant women with GBS (pGBS) diagnosed in French University Hospitals in the 2002-2022 period and compared them with a reference group of same-age non-pregnant women with GBS (npGBS) identified in the same institutions & timeframe. We identified 16 pGBS cases. Median age was 31years (28-36), and GBS developed in the 1st, 2nd, and 3rd trimester in 31%, 31% and 38% of cases respectively. A previous infection was identified in six cases (37%), GBS was demyelinating in nine cases (56%), and four patients (25%) needed respiratory assistance. Fifteen patients (94%) were treated with intravenous immunoglobulins, and neurological recovery was complete in all cases (100%). Unscheduled caesarean section was needed in five cases (31%), and two fetuses (12.5%) died because of cytomegalovirus (CMV) infection (1 case) and HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome (1 case). In comparison with a reference group of 18 npGBS women with a median age of 30years (27-33), pGBS patients more frequently had CMV infection (31% vs 11%), had a prolonged delay between GBS onset and hospital admission (delay > 7days: 57% vs 12%), more often needed ICU admission (56% vs 33%) and respiratory assistance (25% vs 11%), and more often presented with treatment-related fluctuations (37% vs 0%). This study shows GBS during pregnancy is a severe maternal condition with significant fetal mortality.

Current status of Guillain-Barré syndrome (GBS) in China: a 10-year comprehensive overview.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy; a disease involving the peripheral nervous system which is the most common cause of acute flaccid paralysis worldwide. So far, it is still lack of a comprehensive overview and understanding of the national epidemiological, clinical characteristics, and the risk factors of GBS in China, as well as differences between China and other countries and regions in these respects. With the global outbreak of the coronavirus disease 2019 (COVID-19), an epidemiological or phenotypic association between severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection and GBS has attracted great attention. In this review, we outlined the current clinical data of GBS in China by retrieving literature, extracting and synthesizing the data of GBS in China from 2010 to 2021. Besides, we compared the characteristics of epidemiology, preceding events and clinical profiles of GBS between China and other countries and regions. Furthermore, in addition to conventional intravenous immunoglobulin (IVIG) and plasma exchange (PE) therapy, the potential therapeutic effects with novel medications in GBS, such as complement inhibitors, etc., have become the research focus in treatments. We found that epidemiological and clinical findings of GBS inChina are approximately consistent with those in the International GBS Outcome Study (IGOS) cohort. We provided an overall picture of the present clinical status of GBS in China and summarized the global research progress of GBS, aiming to further understand the characteristics of GBS and improve the future work of GBS worldwide, especially in countries with the middle and low incomes.

Evidence of Lumbosacral Plexus Involvement on MR Neurography in Early Guillain-Barré Syndrome (P14-8.015)

<h3>Objective:</h3> To describe a case of early Guillain-Barré Syndrome (GBS) with novel findings of lumbosacral plexus involvement on MR neurography. <h3>Background:</h3> GBS is an acute inflammatory polyradiculoneuropathy characterized by progressive sensory loss, weakness, areflexia, CSF albumino-cytologic dissociation and cauda equina enhancement on MRI. Diagnosis of early GBS can be challenging, as CSF and electrodiagnostic studies can be normal/non-diagnostic in the first 1–2 weeks. We describe a case of early GBS with cauda equina enhancement and lumbosacral plexus abnormalities on MR neurography, seen only three days after symptoms onset and before any diagnostic abnormalities in CSF or electrodiagnostic testing were detected. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 67-year-old man presented with a 3-day history of rapidly progressive gait instability and leg weakness, 14 days after an uncomplicated L5-S1 fusion. Diffuse arm and leg weakness, distal leg sensory loss, hypoactive arm reflexes and absent leg reflexes were detected on exam. CSF analysis showed normal cells and protein (55mg/dL, reference&lt;60mg/dl). Electrodiagnostic testing showed mildly prolonged F-response latencies of the median and ulnar nerves, absent peroneal F-response, normal tibial F-response latency and absent tibial H-responses, without other findings suggestive of demyelination, and normal needle EMG. Lumbar spine MRI showed enhancement of ventrally positioned nerve roots of the cauda equina and no central or foraminal stenosis. Lumbosacral plexus MR neurography showed prominent enlargement, hyperintensity and enhancement of bilateral lower lumbosacral nerve roots and the proximal sciatic nerves. Plasma exchange was initiated with subsequent improvement in his symptoms and exam findings. <h3>Conclusions:</h3> Diagnosis of early GBS can be challenging, as CSF and electrodiagnostic testing can be normal/non-diagnostic. MRI and MR neurography are useful surrogate tests which may have greater sensitivity in detecting early GBS compared to CSF and electrodiagnostic testing. Involvement of the lumbosacral plexus by imaging is a novel finding, which can further assist in diagnosing early GBS cases. <b>Disclosure:</b> Dr. Weng has nothing to disclose. The institution of Darryl Sneag, 12603 has received research support from GE Healthcare. Dr. Pavlakis has nothing to disclose.

Immune-Mediated Neuropathies: Pathophysiology and Management.

Dysfunction of the immune system can result in damage of the peripheral nervous system. The immunological mechanisms, which include macrophage infiltration, inflammation and proliferation of Schwann cells, result in variable degrees of demyelination and axonal degeneration. Aetiology is diverse and, in some cases, may be precipitated by infection. Various animal models have contributed and helped to elucidate the pathophysiological mechanisms in acute and chronic inflammatory polyradiculoneuropathies (Guillain-Barre Syndrome and chronic inflammatory demyelinating polyradiculoneuropathy, respectively). The presence of specific anti-glycoconjugate antibodies indicates an underlying process of molecular mimicry and sometimes assists in the classification of these disorders, which often merely supports the clinical diagnosis. Now, the electrophysiological presence of conduction blocks is another important factor in characterizing another subgroup of treatable motor neuropathies (multifocal motor neuropathy with conduction block), which is distinct from Lewis-Sumner syndrome (multifocal acquired demyelinating sensory and motor neuropathy) in its response to treatment modalities as well as electrophysiological features. Furthermore, paraneoplastic neuropathies are also immune-mediated and are the result of an immune reaction to tumour cells that express onconeural antigens and mimic molecules expressed on the surface of neurons. The detection of specific paraneoplastic antibodies often assists the clinician in the investigation of an underlying, sometimes specific, malignancy. This review aims to discuss the immunological and pathophysiological mechanisms that are thought to be crucial in the aetiology of dysimmune neuropathies as well as their individual electrophysiological characteristics, their laboratory features and existing treatment options. Here, we aim to present a balance of discussion from these diverse angles that may be helpful in categorizing disease and establishing prognosis.

Anti-Lactosylceramide antibody positive combined central peripheral demyelination emerging from long-standing juvenile-onset chronic inflammatory polyradiculoneuropathy; a report of two cases

Anti-Lactosylceramide (LacCer) antibodies are associated with neurological inflammation involving both the peripheral and central nervous system (PNS, CNS respectively), however, the documented number of cases is small. Uncertainty remains whether its positivity can identify a unique clinical entity. Here, we describe two anti-LacCer antibody positive cases, both with long histories (> 30 years) of teenage-diagnosed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). CNS lesions including the medulla oblongata were observed for the first time in adulthood. We suggest that this secondary progression of CNS lesions in juvenile-onset CIDP can be one of the characteristic features of anti-LacCer antibody associated neurological disorder.

Clinical genetic counselling and rehabilitation treatment of a patient with Guillain–Barré syndrome after COVID-19

The authors present a clinical case of rehabilitation after the development of GuillainBarr syndrome, an acute autoimmune inflammatory polyradiculoneuropathy, in a patient who underwent a SARS CoV-2 infection. The patient previously manifested with severe comorbidities (arterial hypertension, hypercholesterolemia, type 2 diabetes mellitus, stenosing atherosclerosis of brachiocephalic arteries). A diagnostic panel of single nucleotide gene polymorphisms associated with risk factors of cardiovascular diseases, metabolic disorders, immunopathology, pharmacogenetics was applied using PCR-RT Genetic Passport test system, and the results were interpreted in order to predict potential complications, adverse drug reactions and the choice of biomarkers for preventive measures. We have compared clinical manifestations, comorbid background and the identified genotype features, as follows: minor homo- and heterozygous variants of ACE, AGT, CYP1A2, NOS3, PPARD, EDN, PALLD, SNX19 genes associated with predisposition to cardiovascular diseases, increasing the risk of dysregulation of blood pressure, development of endothelial dysfunction. The following gene variants were revealed: FXII, ITGA2, ITGB3, MTHFR, MTRR, MTR, PAI-1 that increase the risk of venous and arterial thrombosis, along with gene variants of ADRB3, FTO, INSIG2, KCNG11, LEP, PPARD, TCF7L2, ApoC3, PON1 associated with carbohydrate and lipid metabolism disorders; polymorphisms in the genes determining the immune response, i.e., IL4, IL6, IL8, IL10, CDH1, BDNF1, CRP, CCR5 (with del32 allele considered a risk factor of severe SARS-CoV-2), homozygous polymorphism of a gene of FCGR2 associated with risk of antigen-antibody-complement-mediated cytotoxicity, circulation arrest, deposition of immune complexes in endothelium of microvessels, decreased antithrombotic effects and increased procoagulant activity. Pharmacogenetic study revealed a variant of the CYP4F2 gene, a CYP2C19 gene polymorphism associated with delayed metabolism of a number of pharmaceuticals which requires higher drug dosage, or choosing a drug with a different mechanism of action; gene variants of CYP1A2, GSTP1, GSTT1 reducing efficiency of the cellular detoxification system; NAT2*5 and NAT2*6 variants determining a decrease in appropriate enzyme activities when administering a standard dose of drugs with slowdown of their detoxification, accumulation of toxic metabolites causing clinical adverse effects (hepatotoxicity, dyspepsia, lupus-like syndrome, polyneuritis). Based on the genotype that determines pathogenesis of the multifactorial pathology (including immune-mediated complications of COVID-19), a personalized approach is recommended to the patient, in terms of treatment and prevention of complications. On the basis of testing the biochemical, immunological and blood coagulation biomarkers, an adequate choice of pharmaceuticals is recommended for the patient.

Biopsy Characteristics, Subtypes, and Prognostic Features in 107 Cases of Feline Presumed Immune-Mediated Polyneuropathy.

Inflammatory polyradiculoneuropathy (IMPN) is one of the causes of sudden onset of neuromuscular signs such as para-/tetraparesis in young cats. Even though most cases have a favorable outcome, persistent deficits, relapses, and progressive courses are occasionally seen. As clinical presentation does not always appear to predict outcome and risk of recurrence, this study was initiated to screen for prognostic biopsy findings in a large cohort of histologically confirmed IMPN cases with clinical follow-up. In total, nerve and muscle specimens of 107 cats with biopsy diagnosis of presumed autoreactive inflammatory polyneuropathy and 22 control cases were reviewed by two blinded raters for a set of 36 histological parameters. To identify patterns and subtypes of IMPN, hierarchical k-means clustering of 33 histologic variables was performed. Then, the impact of histological parameters on IMPN outcome was evaluated via an univariate analysis to identify variables for the final multivariate model. The data on immediate outcome and follow-up were collected from submitting neurologists using a purpose-designed questionnaire. Hierarchical k-means clustering sorted the tissues into 4 main categories: cluster 1 (44/129) represents a purely inflammatory IMPN picture, whereas cluster 2 (47/129) was accompanied by demyelinating features and cluster 3 (16/129) by Wallerian degeneration. Cluster 4 (22/129) reflects normal tissues from non-neuropathic control cats. Returned questionnaires provided detailed information on outcome in 63 animals. They were categorized into recovered and non-recovered. Thereby, fiber-invasive infiltrates by mononuclear cells and mild fiber loss in intramuscular nerve branches correlated with higher probabilities of recovery. Remyelination in semithin sections, on the other hand, is correlated with a less favorable outcome. Animals grouping in cluster 1 had a tendency to a higher probability of recovery compared to other clusters. In conclusion, diagnosis of feline IMPN from nerve and muscle biopsies allowed for the identification of histologic features that were positively or negatively correlated with outcome.

CLINICAL CASE OF COVID-ASSOCIATED GUILLAIN-BARRE SYNDROME

The aim of our article is to shed light on the problem of the development of Guillain-Barre syndrome after COVID-19 infection&#x0D; Materials and methods&#x0D; The main research methods we used were scientific research and the actual description of our own clinical case. Laboratory research methods, magnetic resonance imaging, and electromyography were used to confirm the diagnosis and perform differential diagnostics.&#x0D; Results.&#x0D; In 2021, the Center for Acute Neurology and Pain Treatment treated 12 patients with Guillain-Barre syndrome. Guillain-Barre syndrome (GBS) is an acute autoimmune inflammatory polyradiculoneuropathy. The clinical picture of GBS includes progressive motor and sensory disorders in the extremities and pain.&#x0D; We bring to your attention a clinical case of GBS in patient B., who was treated at the clinical hospital "Feofania". Patient B., 32 years old, was hospitalized in the Feofania Clinical Hospital with complaints of weakness in her arms and legs, inability to walk, foreign body sensation in the throat, difficulty swallowing, chest discomfort, and difficulty breathing. A few weeks before the disease, the patient was treated for a mild SARS-CoV-2 infection. The patient's condition gradually deteriorated by day 25 of the disease, despite plasmapheresis, intravenous immunoglobulin and glucocorticoid therapy. However, from day 29, the condition gradually improved and on re-examination after 2 months, the patient remained mild distal paresis of the leg muscles (up to 4 points), decreased tendon reflexes from the lower extremities. Disorders of sensitivity and function of the pelvic organs were not observed.&#x0D; Conclusion.&#x0D; In our article, we present a case of COVID-19-associated GBS with a classic clinical picture, increased peripheral paresis, stabilization, and gradual recovery of all functions (respiration, pelvic function, motor, sensory, and reflex) through pathogenetic treatment and follow-up rehabilitation measures.

Guillain barre syndrome and its association with covid-19 infection – A clinical case series

A novel coronavirus (COVID-19) pandemic is caused by severe acute respiratory syndrome coronavirus 2 (SARs-CoV-2). This pandemic has been globally alarming in the current period. Several neurological manifestations are reported occurring with the infection. Guillain barre syndrome (GBS) or acute onset inflammatory polyradiculoneuropathy has been among the frequent manifestations observed among them. To know the pattern and outcome of GBS in COVID-19 affected individuals. We have taken six individuals admitted with flaccid quadriparesis in the last two months. All were affected recently by COVID 19 infection, which RT PCR of the nasopharyngeal swab confirmed. The study participants have undergone nerve conduction studies and have been diagnosed with Guillain Barre syndrome using Brighton criteria. We did cerebrospinal fluid (CSF) analysis after admission. We initiated all patients on Intravenous immunoglobulin according to body weight (2g/kg divided over five days). We used the Barthel index score to assess the outcome of the individuals. We observed a mean duration of 18.25 days between the COVID-19 infection and the onset of symptoms. Apart from motor quadriparesis and sensory symptoms being in common, we also noticed cranial nerves and autonomic involvement. We made the diagnosis using the nerve conduction studies and Brighton criteria. After initiating intravenous immunoglobulin, all patients had a good outcome, and quality of life was better after two months of follow up. Guillain Barre syndrome is one of the neurological manifestations of COVID-19 and has a dramatic response with intravenous immunoglobulin and better outcome with treatment.

COVID-19-Associated Guillain-Barré Syndrome: A Case Report

Guillain-Barré syndrome (GBS) is an inflammatory polyradiculoneuropathy associated with numerous viral infections. Recently, there have been many case reports describing the association between coronavirus disease-2019 (COVID-19) and GBS [1]. In this report, we describe the symptoms of Guillain Barre Syndrome (GBS) in one infected patient with COVID-19, for the first time. We reported a 67-years- old male patient with no pathological history, complaints of acute progressive symmetric ascending quadriparesis. Two weeks prior to hospitalization, the patient suffered from fever and cough. After hospitalization a thoracic CT scan showed 10-25% of consolidation and ground glass opacities. RT-PCR was reported positive for COVID-19 infection. Electrodiagnostic tests five days after neurological symptom onset showed a demyelinating pattern in accordance with Guillain–Barré syndrome (GBS) criteria.

A Case of Guillain-Barré Syndrome as the First Presentation of Systemic Lupus Erythematosus

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with different clinical manifestations. Acute [resembling Guillain-Barré syndrome (GBS)] or chronic (chronic inflammatory demyelinating polyradiculoneuropathy) inflammatory polyradiculoneuropathy has been reported in rare SLE cases. Case Presentation: We reported a 39-year-old woman that presented with acute peripheral neuropathy, and she was eventually diagnosed with SLE. She developed distal numbness and paraesthesia followed by progressive upper and lower extremity weakness and difficulty in swallowing and speaking. She had a history of flu-like illness three weeks before to symptoms. Conclusions: Progressive upper and lower extremity weakness along with areflexia and electrodiagnostic findings suggested the diagnosis of Guillain-Barré syndrome. Over a month, significant neurological recovery occurred, and the patient's function continued to recover.

Hepatitis E Virus Quasispecies in Cerebrospinal Fluid with Neurological Manifestations.

Hepatitis E virus (HEV) infection can lead to a variety of neurological disorders. While HEV RNA is known to be present in the central nervous system, HEV quasispecies in serum and cerebrospinal fluid (CSF) have rarely been explored. We studied the virus’ quasispecies in the blood and the CSF of five patients at the onset of their neurological symptoms. The samples of three patients suffering from meningitis, neuralgic amyotrophy and acute inflammatory polyradiculoneuropathy were taken at the acute phase of the HEV infection. The samples from the other two patients were taken during the chronic phase (5 years after HEV diagnosis) when they presented with clinical signs of encephalitis. We sequenced at least 20 randomly polyproline regions of the selected virus clones. Phylogenetic analysis of the virus variants in the blood and the CSF revealed no virus compartmentalization for the three acute-phase patients but there was clear evidence of HEV quasispecies compartmentalization in the CSF of the two patients during chronic infection. In conclusion, prolonged infection in the immunocompromised condition can lead to independent virus replication in the liver and the tissues, producing viruses in CSF.

Neurofascin-155 Immunoglobulin Subtypes: Clinicopathologic Associations and Neurologic Outcomes.

Background and ObjectiveMultiple studies highlighting the diagnostic utility of neurofascin-155 (NF155)–immunoglobulin G4 (IgG4) in chronic demyelinating inflammatory polyradiculoneuropathy (CIDP) have been published. However, few studies comprehensively address the long-term outcomes or clinical utility of NF155–immunoglobulin M (IgM) or NF155–immunoglobulin G (IgG) in the absence of NF155-IgG4. We evaluated phenotypic and histopathologic specificity and differences in outcomes between these NF155 antibody isotypes or IgG subclasses. We also compare NF155-IgG4-seropositive cases to other seropositive demyelinating neuropathies.MethodsNeuropathy patient sera at Mayo Clinic were tested for NF155-IgG4, NF155-IgG, and NF155-IgM autoantibodies. Demographic and clinical data of all seropositive cases were reviewed.ResultsWe identified 32 NF155 cases (25 NF155-IgG-positive [20 NF155-IgG4-positive], 7 NF155-IgM-seropositive). NF155-IgG4-seropositive patients clinically presented with distal more than proximal muscle weakness, positive sensory symptoms (prickling, asymmetric paresthesia, neuropathic pain), and gait ataxia. Cranial nerve involvement (11/20 [55%]) and papilledema (4/12 [33%]) occurred in many. Electrodiagnostic testing (EDX) demonstrated demyelinating polyradiculoneuropathy (19/20 [95%]). Autonomic involvement occurred in 45% (n = 9, median composite autonomic scoring scale score 3.5, range 1–7). Nerve biopsies from the NF155-IgG4 patients (n = 11) demonstrated grouped segmental demyelination (50%), myelin reduplication (45%), and paranodal swellings (50%). Most patients needed second- and third-line immunosuppression but had favorable long-term outcomes (n = 18). Among 14 patients with serial EDX over 2 years, all except one demonstrated improvement after treatment. NF155-IgG-positive, NF155-IgG4-negative (NF155-IgG-positive) and NF155-IgM-positive patients were phenotypically different from NF155-IgG4-seropositive patients. Sensory ataxia, neuropathic pain, cerebellar dysfunction, and root/plexus MRI abnormalities were significantly more common in NF155-IgG4-positive compared to myelin-associated glycoprotein (MAG)–IgM neuropathy. Chronic immune sensory polyradiculopathy (CISP)/CISP-plus phenotype was more common among contactin-1 neuropathies compared to NF155-IgG4-positive cases. NF155-IgG4-positive cases responded favorably to immunotherapy compared to MAG-IgM-seropositive cases with distal acquired demyelinating symmetric neuropathy (p < 0.001) and had better long-term clinical outcomes compared to contactin-1 IgG (p = 0.04).DiscussionWe report long-term follow-up and clinical outcome of NF155-IgG4 cases. NF155-IgG4 but not IgM or IgG cases have unique clinical–electrodiagnostic signature. We demonstrate NF155-IgG4-positive patients, unlike classical CIDP with neuropathic pain and dysautonomia common at presentation. Long-term outcomes were favorable.Classification of EvidenceThis study provides Class III evidence that NF155-IgG4-seropositive patients, compared to patients with typical CIDP, present with distal more than proximal muscle weakness, positive sensory symptoms, and gait ataxia.

A post COVID-19 Guillain-Barre syndrome

Guillain-Barré syndrome consists group of clinical syndromes that manifests as an acute or subacute autoimmune inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes. It is also part of so-called Yuki syndrome. Its annual incidence is 1–2/100000. The exact cause of Guillain-Barre syndrome is unknown. But two-thirds of patients report symptoms of an infection in the six weeks preceding. These include respiratory or a gastrointestinal infection (EBV, C. Jejuni) or Zika virus.

Relationship between cerebrospinal fluid protein level and electrophysiologic abnormalities in the acute inflammatory demyelinating polyradiculoneuropathy variant of Guillain-Barré syndrome.

Objective: Guillain-Barré syndrome (GBS) is an autoimmune disease characterized by weakness in limbs or cranial nerve innervated muscles. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common variant. Electrophysiologic abnormalities and elevated cerebrospinal fluid (CSF) protein are frequently present in AIDP, but the relationship between these two parameters is not well known. We aimed to fill this gap by studying this relationship.Methods: This was a prospective cross-sectional study conducted for two years in the Department of Neurology, Dr. Ruth K. M. Pfau Civil Hospital, Karachi, Pakistan. All 90 adult patients with the AIDP variant of GBS were selected. Nerve conduction studies were performed to determine the degree of demyelination through the four electrophysiologic demyelination criteria. The CSF sample was sent to lab immediately after lumbar puncture. SPSS version 20.0 was used. The CSF protein level was measured with mean ±SD. Demyelination criteria were measured in frequency and percentages. Chi-square test was applied to a number of demyelination criteria and T-test/ANOVA was applied on mean CSF protein level.Results: We found a mean CSF protein of 37.41 mg/dl (±3.69) with one demyelination criterion, 81.87 mg/dl (±17.39) with two demyelination criteria, 119.75 mg/dl (±31.42) with three demyelination criteria, and 134.00 mg/dl (±42.87) with four demyelination criteria (P-value <0.001). Conclusion: This study demonstrates a significant relationship between CSF protein levels and degree of demyelination in the AIDP variant of GBS. This is an under-researched area in GBS and this study adds favorably to limited data in this regard.

Comparative Study between COVID Associated Guillan Barre Syndrome and Non-COVID Associated Guillan-Barré Syndrome

More than 12 million people have been infected with COVID-19 worldwide, with more than 500,000 deaths to date.1 Although COVID-19 research is rapidly evolving, new findings must be thoroughly scrutinised before any conclusions or treatment protocols are established or amended.2 Although COVID-19 is most usually associated with respiratory symptoms such as cough and dyspnea; it has recently been associated with a neurotropic presentation.3 Guillain-Barré Syndrome (GBS) is best described as an acute inflammatory polyradiculoneuropathy clinically characterized by areflexia and progressive weakness of arms and legs. Though, many rare variants of GBS have been described, the commonly observed subtypes such as Acute Motor Axonal Neuropathy (AMAN), Acute Motor Sensory Axonal Neuropathy (AMSAN) and Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) tend to fulfil the above-mentioned criteria.4 Recently, several case reports have suggested a relationship between the occurrence of Guillain-Barré syndrome (GBS) and a previous SARS-CoV-2 infection, which preceded the GBS onset by up to 4 weeks. Therefore, a post-infectious dysregulation of the immune system, triggered by SARS- CoV2, appears to be the most probable cause COVID-19 is a systemic disorder presenting typically with fever and respiratory symptoms but neurological manifestations such as acute cerebrovascular diseases, seizures, ageusia, anosmia meningitis, encephalitis and skeletal muscle involvement were soon reported.5 More recently, an increase in case reports of Guillain-Barré syndrome (GBS) in people infected with SARS-CoV-2 has prompted concerns about a possible link.

Characterization and management of neurological adverse events during immune-checkpoint inhibitors treatment: an Italian multicentric experience.

Neurological immune-related adverse events (nirAEs) are rare toxicities of immune-checkpoint inhibitors (ICI). With the increase of ICI oncological indications, their incidence is growing. Their recognition and management remain nevertheless challenging. A national, web-based database was built to collect cases of neurological symptoms in patients receiving ICI and not attributable to other causes after an adequate workup. We identified 27 patients who developed nirAEs (20 males, median age 69years). Patients received anti-PD1/PDL1 (78%), anti-CTLA4 (4%), or both (19%). Most common cancers were melanoma (30%) and non-small cell lung cancer (26%). Peripheral nervous system was mostly affected (78%). Median time to onset was 43.5days and was shorter for peripheral versus central nervous system toxicities (36 versus 144.5days, p = 0.045). Common manifestations were myositis (33%), inflammatory polyradiculoneuropathies (33%), and myasthenia gravis (19%), alone or in combination, but the spectrum of diagnoses was broad. Most patients received first-line glucocorticoids (85%) or IVIg (15%). Seven patients (26%) needed second-line treatments. At last follow-up, four (15%) patients were deceased (encephalitis, 1; myositis/myasthenia with concomitant myocarditis, 2; acute polyradiculoneuropathy, 1), while seven (26%) had a complete remission, eight (30%) partial improvement, and six (22%) stable/progressing symptoms. ICI treatment was discontinued in most patients (78%). Neurological irAEs are rare but potentially fatal. They primarily affect neuromuscular structures but encompass a broad range of presentations. A prompt recognition is mandatory to timely withheld immunotherapy and administrate glucocorticoids. In corticoresistant or severely affected patients, second-line treatments with IVIg or plasmapheresis may result in additional benefit.