Acute Inflammatory Demyelinating Neuropathy Research Articles

Guillain–Barré syndrome after COVID‐19 vaccination with suspected concurrent cytomegalovirus reactivation

AbstractBackgroundGuillain–Barré syndrome (GBS) is an immune‐mediated polyneuropathy most commonly associated with antecedent infections, such as Campylobacter jejuni or viral infections, but vaccination is also known as an antecedent event. In recent years, due to the worldwide pandemic, vaccination against coronavirus disease 2019 (COVID‐19) has widely spread throughout the world. Although surveillance of vaccination‐associated adverse effects is still ongoing, multiple cases of GBS after COVID‐19 vaccination have been reported. However, the exact pathophysiology of COVID‐19 vaccination causing GBS has not been clarified. Not only new‐onset infection, but also reactivation of dormant viral agents, is known to trigger GBS. This reactivation might occur after COVID‐19 vaccination; however, there are no reports of this phenomenon in association with post‐vaccination GBS.Case PresentationA 44‐year‐old immunocompetent woman presented with acute onset weakness in all four limbs 6 days after COVID‐19 vaccination. Neurological examination showed dysphagia, symmetrical weakness, absent tendon reflexes and distal dominant sensory disturbance in all four extremities. Nerve conduction studies were compatible with demyelinating neuropathy, and serum was positive for anti‐GD1a‐IgG antibody. A diagnosis of acute inflammatory demyelinating neuropathy was made. Serological examination on admission suggested concomitant cytomegalovirus reactivation. Plasma exchange rapidly improved her symptoms, including weakness and sensory disturbance, and she was discharged without residual symptoms.ConclusionsThe present case suggests that cytomegalovirus reactivation might play a role in the pathogenesis of GBS associated with COVID‐19 vaccination. Accounting for cytomegalovirus reactivation might reveal unidentified pathophysiology of GBS after COVID‐19 vaccination.

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

Background & 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<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.

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.

Bilateral Facial Weakness with Distal Paresthesia Following COVID-19 Vaccination: A Scoping Review for an Atypical Variant of Guillain-Barré Syndrome.

Background and Purpose: Recent population-based studies from the US and UK have identified an increase in the occurrence of Guillain–Barré syndrome (GBS) following coronavirus disease 2019 (COVID-19) vaccination. However, the localized variant of GBS might be underestimated due to its rarity and atypical features. We aimed to identify and characterize bilateral facial weakness with distal paresthesia (BFWdp) as a GBS variant following COVID-19 vaccination. Materials and Methods: Relevant studies published during the COVID-19 pandemic were searched and identified in the MEDLINE, Embase, and other databases. Results: This review found that 18 BFWdp cases presented characteristics similar to previous BFWdp cases as defined in the literature: male dominance, frequent albuminocytological dissociation, and acute inflammatory demyelinating neuropathy pattern. In contrast, facial nerve enhancement on brain MRI and antiganglioside antibody positivity were often observed in BFWdp following COVID-19 vaccination. Conclusions: The mechanism of BFWdp following COVID-19 vaccination appears to be somewhat different from that of sporadic BFWdp. Neurological syndromes with rare incidence and difficulty in diagnosis should be considered adverse events of COVID-19 vaccination.

Review Article on Guillain Barre Syndrome

Guillain-Barré syndrome (GBS) is an autoimmune disease caused by a viral or viral infection and can be reduced. The most common HIV virus is Campylobacter June, which is a major cause of bacterial gastroenteritis worldwide. Immune-mediated immune responses may play a role in the development of GBS by interacting with brain tissue. Because the infected organism contains homologous epitopes, it initiates molecular and cell immune responses that interact with the ganglioside surface areas of the peripheral nerve (cell imitation). The immune response to epitopes in the upper layer of Schwann cells acts as a target for acute inflammatory demyelinating neuropathy (85%) caused by a reaction to epitopes found in the Schwann-cell surface or myelin. A strong axonal variation of GBS is caused by a reaction to epitopes found in the axonal membrane (15 percent of cases). Although caring for these people may be difficult, complete predictions are good. The basis of treatment is to provide the best supportive care and expectation and avoidance of complications. Patients in need of intubation and respiratory assistance should be admitted to the intensive care unit by 33% of cases. Immunomodilution with IgG infusions or plasma exchange therapy can accelerate the progression of this condition. Conclusion: High-quality intensive care is the necessary aspect of management of cases of GBS. Clinical studies show plasma exchange is one of the most promising treatments. Intravenous immunoglobulin is also effective but has side effects. Corticosteroid with IMG is insufficient and the evidence of this working is less.

Electrophysiological features of Guillain-Barré syndrome in Mongolian adult patients

The Guillain-Barré syndrome (GBS) is a rare but severe disorder that affects peripheral nerves and is classified into several subtypes based on clinical presentation and electrophysiological abnormalities. Incidence and predominant subtypes of GBS differ geographically. The diagnosis is made upon clinical features and confirmed by nerve conduction studies (NCSs) which can differentiate subtypes such as demyelinated and axonal. Demyelinating subtypes in Europe and North America and axonal subtypes in Asia are predominant. Electrophysiological subtypes have not been determined in Mongolia. Therefore, we aimed to determine common electrophysiological subtypes of Guillain-Barré syndrome in Mongolia. In our study, 75 patients referred to Reflex Neurological Clinic between 2016 and 2019 were retrospectively reviewed. After excluding the cases by the criteria, patients were classified as demyelinating, axonal, normal and equivocal subtypes based on Hadden criteria. We compared the electrophysiological features of axonal and demyelinating subtypes. SPSS-23 was used for statistical analysis. The results are expressed in averages (standard deviations) and percentages (numbers). The difference between the mean and the group of variables was calculated using the T-test and the Chi-square test. Patients were 44% male (33), and the mean age was 46.4±15.1. There was no significant association between seasonal factors and electrophysiological subtypes. Among 75 patients with Guillain-Barré syndrome, a nerve conduction study was performed in the first week on 36% of patients, 33.3% in the 2nd week, and others in the 3rd-4th week after symptom onset. Electrophysiology subtypes were classified as demyelinating (65.3%), axonal (20%), within the normal range (5.4%) and equivocal (9.3%) based on Hadden criteria. The absence of F-waves on electrophysiological examination was higher in patients within 14 days of the disease onset, whereas non-sensory changes were higher in patients after 14 days. We conclude that acute inflammatory demyelinating neuropathy (AIDP) was the most frequent subtype of GBS in Mongolia.

Nationwide Survey of Childhood Guillain-Barré Syndrome, Fisher Syndrome, and Bickerstaff Brainstem Encephalitis in Japan

Background: Guillain-Barre syndrome (GBS), Fisher syndrome (FS), and Bickerstaff brainstem encephalitis (BBE) are immune-mediated neuropathies presenting with symptoms such as muscle 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. Methods: We 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. This study was approved by the local ethics committee of the Graduate School of Medicine, Chiba University. Findings: Five-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, suggesting annual incidence rates of was 0·19, 0·023, and 0·0071 per 100,000 children, respectively, in the pediatric population (0-14 years). GBS was classified as acute inflammatory demyelinating neuropathy (39·7%), acute motor axonal neuropathy (24·4%), or acute motor-sensory axonal neuropathy (11·5%), 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 were able to walk within one year, indicating an excellent childhood prognosis. No in-hospital deaths occurred in this study. Interpretation: The 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. Clinical Trial Registration Details: The research scheme was registered at the UMIN Clinical Trials Registry (UMIN-CTR) as UMI18739. Funding Information: This work was supported by the institutional budget of the Chiba University Graduate School of Medicine (grant reference J09KF00284). Declaration of Interests: The authors declare no conflict of interest. Ethics Approval Statement: The study protocol was approved by the institutional ethics committee of the Graduate School of Medicine, Chiba University (No. 319), and the Collaborative Research Committee of the Japanese Society of Child Neurology (No. 13-04).

Polyneuropathy in Young Siberian Huskies Caused by Degenerative and Inflammatory Diseases.

Polyneuropathy is defined as the simultaneous dysfunction of several peripheral nerves. In dogs, a number of breeds are predisposed to a variety of immune-mediated and/or degenerative inherited forms of polyneuropathy, with laryngeal paralysis and/or megaesophagus as important clinical features of many of these conditions. This case series describes degenerative and inflammatory polyneuropathies in 7 young Siberian huskies that were categorized based on clinicopathological characteristics as follows: (1) slowly progressive laryngeal paralysis and megaesophagus caused by primary axonal degeneration with large fiber loss (n = 2); (2) slowly progressive polyneuropathy without megaesophagus or laryngeal paralysis caused by primary axonal degeneration with large fiber loss (n = 2); (3) acute inflammatory demyelinating neuropathy causing sensory, motor and autonomic nerve deficits (n = 2); and (4) ganglioradiculitis (sensory neuronopathy; n = 1). Based on the predominantly young age at onset, slow progression, relatedness of affected dogs, and clinical and pathological similarities with inherited neuropathies reported in other dog breeds, a hereditary basis for the degenerative polyneuropathies in Siberian huskies is suspected. However, 5 different mutations in 3 genes known to cause polyneuropathy in other dog breeds (NDRG1, ARHGEF10, or RAB3GAP1) were not detected in the affected Siberian huskies suggesting that more genetic variants remain to be identified. This study highlights the varied underlying lesions of polyneuropathies in young Siberian huskies.

IVIG efficacy in CIDP patients is not associated with terminal complement inhibition

Patients with acute and chronic inflammatory demyelinating neuropathies exhibit elevated serum and cerebrospinal fluid (CSF) levels of terminal complement activation products and therapeutic inhibition of complement activation is currently tested for its safety and efficacy in patients with Guillain-Barré syndrome (GBS). Here, we determined serum levels of the complement activation products C3a, C5a and the soluble terminal complement complex (sTCC) in 39 individuals with chronic inflammatory demyelinating polyneuropathy (CIDP) who participated in one of the largest ever conducted clinical trial in patients with CIDP (ICE trial) and received Intravenous Immunoglobulin (IVIG) or placebo (albumin) in 3 week intervals for up to 24 weeks. In placebo-treated patients with spontaneous disease remission, serum sTCC levels moderately decreased over time. Levels of complement activation products were, however, not modulated by IVIG and remained unchanged in patients with a beneficial response to IVIG therapy as compared to those with steady or worsened disease. These results suggest that the therapeutic efficacy of IVIG in CIDP is based on immunomodulatory mechanisms different from complement inhibition. Terminal complement activation merits further investigation as a surrogate marker for disease progression and therapeutic target in patients with CIDP.

Severe Muscle Weakness Needing Intubation in AIDS Patient

SESSION TITLE: Critical Care 3 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Flaccid paralysis has myriad of differential diagnoses ranging from different types of neuropathies, myopathies and neuromuscular disorders. HIV is now a chronic disease with the advent of highly active anti retroviral therapy (HAART) such that several HIV related complications are now evident. HIV myopathy is a rare condition that can manifest at any stage of HIV irrespective of the CD4 count. We present a case of HIV associated myopathy in a patient who presented with about 2-3 day history of generalized weakness. CASE PRESENTATION: A 41-year-old woman with HIV/AIDS who was non compliant with HAART presented with flaccid paralysis which progressed to respiratory failure requiring intubation and mechanical ventilation. Her CD4 count was 6/mcL with 75000 HIV RNA copies/ml. Cerebrospinal fluid analysis was negative for albumin-cytological dissociation. MRI brain and spine were negative for progressive multifocal leukoencephalopathy. MRI of bilateral lower extremities showed intramuscular edema. EMG/NCS was inconclusive in view of obesity and severe edema. Creatine kinase and aldolase were mildly elevated. Left quadriceps femoris biopsy was suggestive of occlusive small arteriopathy consistent with vasculitis, and severe myofiber atrophy. ANA, ANCA, CMV PCR, HTLV I/II were negative. She had similar presentation a year ago where she responded to steroids which were tapered over 8 months ago. She was diagnosed with AIDS at that time and started on HAART, however, patient was non compliant with treatment. This time however, she had minimal response to steroids and antiretroviral therapy. She had ATN and subsequent renal failure from multiple medications. Given her poor prognosis, comfort measures were initiated and patient passed away shortly after extubation. DISCUSSION: Neuromuscular disorders associated with HIV include neuropathies such as distal symmetric polyneuropathy, acute and chronic inflammatory demyelinating neuropathy, mononeuropathy, radiculopathy, etc., and myopathies.1 Muscle disorders in HIV could be from the virus itself resulting in HIV myopathy, which is similar in picture to autoimmune polymyositis; drug induced myopathy from HAART such as zidovudine which is a mitochondrial disorder; HIV wasting syndrome; myopathy from opportunistic infections such as CMV, etc. The biggest case series with HIV induced polymyositis had remission in 80% of patients at 9 month follow up.2 However this result has not been replicated and HIV myopathy is thought to have variable steroid response. CONCLUSIONS: HIV myopathy is a rare manifestation with variable prognosis and response to steroids. Immunosuppressive therapy is used with caution due to toxicity. Reference #1: Robinson-Papp J, Simpson DM. Neuromuscular diseases associated with HIV-1 infection. Muscle Nerve. 2009;40:1043-1053. Reference #2: Johnson RW, Williams FM, Kazi S, Dimachkie MM, Reveille JD. Human immunodeficiency virus-associated polymyositis: a longitudinal study of outcome. Arthritis Rheum. 2003;49:172-178. DISCLOSURE: The following authors have nothing to disclose: Pooja Gurram, Vikas Koppurapu, Krishna Siva Sai Kakkera, Kevin Davis, Swathi Subramany, Rajani Jagana No Product/Research Disclosure Information

Acute axonal neuropathy subtype of Guillain Barré syndrome in a French pediatric series: Adequate follow-up may require repetitive electrophysiological studies.

Different subtypes of Guillain Barré Syndromes (GBSs) are defined by their electrophysiological characteristics, acute inflammatory demyelinating neuropathy (AIDP), and acute motor/motor-sensory axonal forms (AMAN/AMSAN) with either reversible nerve conduction failure (RCF) or axonal degeneration. Our aim was to describe initial clinical and electrophysiological characteristics of axonal forms of GBS in a pediatric population and their short- and long-term evolution. Electroneuromyogram (ENMG) results were collected at diagnosis and at two months of evolution and interpreted using the recently proposed pattern of RCF vs axonal degeneration. Clinical evaluation was standardized using the GBS disability scale ("GBSds") at diagnosis, and then at 3, 6, and 12 months of evolution. Outcome was compared to those of patients with AIDP diagnosed within the same period. Eleven patients were included, among whom eight patients presenting with AMAN and three with AMSAN. Two subgroups were identified according to severity. Three patients had a severe form (GBSds ≥2 at 12 months), two of them presenting an axonal degeneration on ENMG studies. Seven patients had a less severe form (GBSds ≤1 at 12 months), five of them with RCF on ENMG studies. Axonal forms had a more severe evolution than demyelinating forms (n=17) at 3 months (median GBSds 3 and 2, respectively), 6 months (2 and 0), and 12 months (1 and 0), (p<0,05). Axonal forms of GBS in children have a more severe global outcome than demyelinating forms. Axonal degeneration in two successive early ENMGs may be a prognostic factor of poor outcome.

Case Report: Subacute onset of the motor-sensory axonal neuropathy variant of Gullain-Barré syndrome after epidural anesthesia

Guillain-Barré syndrome (GBS) is an acute ascending peripheral neuropathy, caused by autoimmune damage of the peripheral nerves. GBS can be divided into three subtypes: acute inflammatory demyelinating neuropathy, acute motor axonal neuropathy, and the more rare type, acute motor and sensory axonal neuropathy (AMSAN). Reports of AMSAN with onset after epidural anesthesia and spinal surgery are extremely rare, and the linkage between development of GBS and neuroaxial anesthesia remains conclusively unconfirmed. We present a case in which the patient developed subacute motor and predominantly sensory neuropathy following epidural blockade. The case emphasizes the need of including AMSAN in differential diagnostic considerations to changes in motor and sensory function following epidural anesthesia, allowing accelerated rehabilitation and relevant alleviating therapy.

Charcot-Marie-Tooth Phenotype with a 13-Year-Old Migrant: DD of Immune Neuropathy

Background: A subacute, flaccid paresis in a child or youth from Central Europe, starting at the distal lower extremities and increasing within days, initially suggested the differential diagnosis of a primary immune neuropathy in the sense of Guillen-Barré syndrome (GBS), here in particular an acute inflammatory demyelinating neuropathy (AIDP). However, this attribution is questionable considering a different country of origin, particularly from the Asian or Southern American region, since there is a prevalence of GBS subtypes divergent from Central Europe, with considerably different prognosis. Apart from this connection the resulting central importance of the electrophysiological findings for an early accurate diagnosis shall be demonstrated.

Diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating neuropathy (CIDP) is generally considered to be an immune-mediated neuropathy with potential target antigens, although its precise pathogenesis is unclear. Unlike its more acute counterpart, acute inflammatory demyelinating neuropathy, the diagnosis is necessarily delayed as a result of clinical definitions but also the fluctuating clinical manifestations which can evolve over months and years. In addition, as a result of its relative rarity it is not usually definitively diagnosed and treated until patients come into contact with specialist neurology services. However, even within specialist settings the diagnosis is often elusive as a result of heterogeneous clinical manifestations, the patchy nature of the demyelinating pathology and limitations of available diagnostic tests. Despite the limited understanding of disease aetiology, recent studies have underlined the important role of both cellular and humoral components of the immune system in pathogenesis which in turn has informed treatment approaches. Although there remains no overall consensus on the optimum management strategy for CIDP, it is becoming clear that individualising therapeutic interventions depending on accurate identification of the CIDP variant is important. These include the typical symmetrical motor and sensory polyneuropathy, multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), pure motor, pure sensory and distal acquired demyelinating symmetric (DADs) neuropathy, all of which may demonstrate variation in response to intravenous immunoglobulins, plasma exchange (PE) and other immunosuppressive therapies possibly as a result of different underlying pathological mechanisms. In this month’s Journal Club, we review three papers focusing on different aspects of diagnosis and treatment in CIDP. The first paper explores patterns of magnetic resonance neurography and the distribution of nerve hypertrophy in CIDP variants. The second paper reviews different electrophysiological profiles and treatment response in typical and atypical forms, whilst the third investigates predictors of intravenous immunoglobulin (IVIG) responsiveness in patients with CIDP.

P485: Event-related potentials in patients with primary Sjögren’s syndrome

s of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S179 Abstract P484 – Figure 2P484 – Figure 2 neuropathy with a high variable clinical course and outcome. Despite immunotherapy up to 20% of patients remain severely disabled and mortality in the first year is approximately 4%. Aim of this study is to perform an epidemiological analysis of clinical and electrophysiological features of patients admitted in our ward for GBS in last ten years; to test the incidence of different GBS subtypes and their clinical and electrophysiological evolution; to test on our sample the prognostic value of variables known in literature and we suggested integration with new prognostic markers. We collected data prospectively from 89 patients regarding: demographic features, previous infections, clinical manifestations, kind of therapy received, motor deficit and disability (evaluated respectively with MRC sumscore and GBS disability score (GBSDS)). We measured dosage of protein in the cerebrospinal fluid (CSF). Serial nerve conduction studies (NCS) were performed. Differences between groups were compared by Fisher’s exact test. Potential prognostic factors were tested in uni and multivariable logistic analysis and was estimated by regression analysis. 19% of GBS were acute motor axonal neuropathy (AMAN) and showed more severe prognosis. High age was predictive of poor prognosis in acute inflammatory demyelinating neuropathy (AIDP) but not in AMAN. High GBSDS at nadir and cranial nerves involvement were predictive of being enable to run at 1 year. Conduction block did not have a significant distribution in GBS subtypes and showed no correlation with clinical outcome. In NCS performed between 30 and 90 days from the clinical onset, a pathological distal cMAP duration (DcMAPD) represented a distinctive marker of primary demyelinating pathophysiological mechanism, in our sample of GBS. The endurance of pathological DcMAPD showed a predictive value on long term disability in AIDP. This study confirms the key-role of serial NCS for a proper diagnosis of GBS subtypes. Further clinical-electrophysiological studies are necessary to define more inclusive work-up in GBS enabling forecasting of the patients with poorest long term outcome.

English

The central nervous system (CNS) is among the most frequent and main target of HIV infection in severely immunocompromised patients. Neurological manifestations occur due to either primary pathologic process of HIV or secondary to opportunistic infection. The present study was conducted to ascertain the prevalence of various neurological manifestations in HIV positive patients, correlation of CD4&nbsp;levels in CNS opportunistic infection and their outcome. This was a prospective observational study of 105 HIV infected patients with clinical evidence of CNS involvement. A detailed clinical history and CNS examination was carried out. CD4counts was measured by flow cytometry method and other investigations like&nbsp;magnetic resonance imaging&nbsp;(MRI), brain/electromyography, nerve conduction studies and cerebrospinal fluid (CSF) examination were done as required for diagnosis. HIV induced primary illness was present in about 30% cases while 70% associated with secondary CNS manifestations were mainly due to opportunistic infection. The most common primary illness was distal symmetrical polyneuropathy (20.9%), followed by AIDS dementia complex (3.8%), acute inflammatory demyelinating neuropathy (3.8%). On the other hand, the most common secondary CNS infection was tuberculous bacterial (TBM; 32.3%), followed by cryptococcal meningitis (13.3%), progressive multifocal leukoencephalopathy (PML; 11.4%), and cerebral toxoplasmosis (9.5%). The commonest presenting symptoms of TBM were fever (72.38%), while headache and vomiting was 27.62 and 28.57%, respectively. Mean CD4 count was 172 &plusmn; 81.2 in distal symmetrical polyneuropathy (DSPN), 282&nbsp;&plusmn;&nbsp;75.3 in acute inflammatory demyelinating neuropathy (AIDP) and 95&nbsp;&plusmn;&nbsp;6.5 for AIDS dementia complex. &nbsp; Key words:&nbsp;HIV positive patients, CD4&nbsp;count neurological manifestation.

Antiganglioside antibodies in Guillain–Barré syndrome and its related conditions

Guillain–Barré syndrome (GBS) is typically classified into two major subtypes: acute inflammatory demyelinating neuropathy and acute motor axonal neuropathy. Its most recognizable variant is Fisher syndrome. The last two decades have seen considerable advances in our understanding of GBS. Of note, various autoantibodies against ganglioside antigens have been identified and found to have significant associations with the axonal forms of GBS and Fisher syndrome. In this article, we discuss the different clinical presentations in GBS and the role of antiganglioside antibodies in their underlying pathogenesis. We also discuss the impact that antiganglioside antibodies have had in the development of experimental models and treatment modalities in GBS.

CIDP – the relevance of recent advances in Schwann cell/axonal neurobiology

Early pathological studies in patients with acute and chronic inflammatory demyelinating neuropathies, and the animal model experimental autoimmune neuritis (EAN) showed similarities in the process of demyelination. These studies focused on compact myelin proteins and peptides as targets of immune attack in Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and EAN. However, serological studies in patients with subsets of GBS highlighted the importance of gangliosides - glycolipids enriched in non-compact Schwann cell regions and the node, paranodal, and internodal axolemma. In the acute motor axonal neuropathy (AMAN) rabbit model, antibodies to the ganglioside GM1 bind in the nodal region, impair Na channel clustering and disturb Schwann cell/axon organisation. Schwann cell neurobiological studies now highlight the importance of adhesion molecules, including neurofascins, gliomedin, contactins, and NrCAM to Schwann cell/axon integrity. Changes to nodal fine structure by immune responses against such molecules may provide a mechanism for reversible conduction failure or block. Recovery of patients with CIDP or multifocal motor neuropathy (MMN) following treatment may sometimes be better explained by reversal of conduction failure than remyelination or regeneration. This review considers the importance of the intricate molecular arrangements at the nodal and paranodal regions in inflammatory neuropathies such as CIDP. Early images of compact myelin stripping and phagocytosis, may have diverted the research focus away from these vital non-compact myelin Schwann cell areas.

Electrocardiographic Abnormalities in Patients with Guillain-Barre Syndrome

Introduction: Guillain-Barre syndrome (GBS) is an acute inflammatory demyelinating neuropathy accompanied with autonomic dysfunction. Although the mortality cause in patients hospitalized with GBS relates to cardiac arrhythmias, the electrocardiographic abnormalities in acute phase of GBS remains unclear. Methods: We retrospectively reviewed 11 patients hospitalized with GBS (7 men, mean age: 63 years) to evaluate the characteristics of 12-lead electrocardiogram (ECG) in acute phase. Results: 12-lead ECG showed sinus rhythm in all patients. Complete right bundle branch block were seen in 2 patients and first-degree atrioventricular block in 2 patients. The PQ, QRS, and QTc intervals (mean±SD) were 160±24 ms, 100±20 ms, and 446±22 ms, respectively. J wave with the amplitude of 0.16±0.03 mV was observed in 8 (73%) patients. No patients showed lethal arrhythmias such as sinus arrest or ventricular tachycardia during hospitalization. Conclusion: J wave was frequently found among patients with GBS in acute phase, which may be related to autonomic dysfunction.

PATTERNS OF GUILLAIN-BARRÉ SYNDROME IN CHILDREN: RESULTS FROM A MEXICAN POPULATION

We read with interest the article by Nachamkin et al., who reported that acute motor axonal neuropathy (AMAN) was more common (59%) than AIDP (41%).1 This distribution of Guillain-Barre syndrome (GBS) is similar in China, Japan, and South America, whereas the acute inflammatory demyelinating neuropathy (AIDP) form of GBS is more common in Western Europe, America, and Australia, where it constitutes 80–90% of GBS in children and adults.2 We are interested in the …