Immune-mediated Neurological Disorders Research Articles

Fifty Years of Handedness Research: A Neurological and Methodological Update.

Handedness, a complex human aspect that reflects the functional lateralization of the hemispheres, also interacts with the immune system. This study aimed to expand the knowledge of the lateralization of hand, foot, and eye activities in patients with immune-mediated (IM) or other (noIM) neurological diseases and to clarify the properties of the Edinburgh Handedness Inventory (EHI) in an Italian population. Three hundred thirty-four patients with IM or noIM diseases affecting the brain or spine and peripheral nervous system were interviewed about stressful events preceding the disease, subjective handedness, and familiarity for left-handedness or ambidexterity. The patients and 40 healthy subjects underwent EHI examination. In the whole group of participants, 24 items of the EHI were classified into five factors (Hand Transitive, Hand Refined, Hand Median, Foot, Eye), demonstrating good reliability and validity. Chronological age had a significant influence on hand and foot EHI factors and the laterality quotient (LQ), particularly on writing and painting. In the patient groups, EHI factors and the LQ were also predicted by age of disease onset, duration of disease, and family history of left-handedness or ambidexterity. No differences were found between patients and healthy subjects, but pencil use scored significantly lower in patients with IM diseases than in those with noIM brain diseases. These results demonstrate that the lateralization of hand and foot activities is not a fixed human aspect, but that it can change throughout life, especially for abstract and symbolic activities. Chronic neurological diseases can cause changes in handedness. This may explain why, unlike systemic immunological diseases, IM neurological diseases are not closely associated with left-handedness. In these patients, the long version of the EHI is appropriate for determining the lateralization of body activities to contextualize the neurological picture; therefore, these findings extend the Italian normative data sets.

MOGAD: A novel disease

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an immune-mediated inflammatory neurological disease and a new addition to the demyelinating disorders of the central nervous system(CNS).In MOGAD, specific antibodies (MOG IgG) targeting MOG protein lead to primary demyelination in the CNS, but notably spare astrocytes. The most frequent presentation in children is ADEM and in adult ON. We present a case of 20 year old female who visited our OPD with complaints of diminution of vision with muscular weakness and strain while walking with a history of two similar episodes in the past. The clinical characteristics, laboratory investigation and neuroimaging help in diagnosis. Although the prognosis is generally favorable but severe residual disability can also occur.This underscores the importance of identifying the causes of demyelination on time and prompt treatment of this rather novel disease by judicious use of steroids based on standard protocolresulting in the general well-being of the patient. The present case therefore highlights the need to create awareness of the Clinical-Lab-Imaging characteristics of MOGAD andalso suggests the importance of making a detailed multidisciplinary approach into the cause of optic neuritis for better prognosis.

Clinical features and pathogenesis of Glial fibrillary acidic protein (GFAP) antibody-associated disorders

Glial fibrillary acidic protein (GFAP) antibody-associated disorders (AD) were recently proposed to be immune-mediated neurological disorders. The pathogenesis of GFAP antibody-AD is poorly understood. Pathologically, there is a marked infiltration of large numbers of lymphocytes, including CD8+ and CD4+ T cells, into the meningeal and brain parenchyma, especially around the perivascular areas. GFAP-specific cytotoxic T cells are considered to be the effector cells of GFAP antibody-AD. The common phenotype of GFAP antibody-AD includes meningoencephalitis with or without myelitis. During the clinical disease course, patients present with consciousness disturbances, urinary dysfunction, movement disorders, meningeal irritation, and cognitive dysfunction. The detection of GFAP antibodies in the cerebrospinal fluid (CSF) by cell-based assay is essential for a diagnosis of GFAP antibody-AD. The CSF can be examined for lymphocyte-predominant pleocytosis and elevated protein levels. Brain linear perivascular radial enhancement patterns are observed in about half of GFAP antibody-AD patients. Spinal cord magnetic resonance imaging is used to detect longitudinal extensive spinal cord lesions. Although corticosteroid therapy is generally effective, some patients have a poor prognosis and relapse.

Clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease in Brazil: a single-center experience.

Anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibody-associated disease (MOGAD) is an immune-mediated neurological disorder with a broad spectrum of clinical presentation that is often difficult to distinguish from other demyelinating diseases, such as multiple sclerosis and neuromyelitis optica spectrum disorder. To describe the clinical and paraclinical characteristics of MOGAD in a Brazilian tertiary center. We retrospectively reviewed the records of adult and pediatric patients who tested positive for anti-MOG antibodies and presented with clinical and radiological diseases compatible with MOGAD. Forty-one patients (10 children) were included: 56% female, 58% Caucasian, mean age at onset 31 years (range 6-64), with a mean disease duration of 59.6 months (range 1-264 months). The most frequent onset presentation was optic neuritis (68%), acute disseminated encephalomyelitis (ADEM, 12%), and myelitis (10%). A monophasic disease course was observed in 49%. EDSS median was 2.1 at the last visit. Most patients (83%) were under continuous immunosuppressive treatment. Azathioprine was the first-line treatment in 59%. In all ADEM cases, conus, and root involvement was radiologically observed on MRI. Brazilian MOGAD patients presented with a similar spectrum of previously reported MOGAD phenotypes. Conus and spinal root involvement seems to be frequently present in MOGAD-ADEM and could serve as radiologic characteristics of this clinical entity.

Mapping the Relationship of White Matter Lesions to Depression in Multiple Sclerosis

BackgroundMultiple sclerosis (MS) is an immune-mediated neurological disorder, and up to 50% of patients experience depression. We investigated how white matter network disruption is related to depression in MS. MethodsUsing electronic health records, 380 participants with MS were identified. Depressed individuals (MS+Depression group; n = 232) included persons who had an ICD-10 depression diagnosis, had a prescription for antidepressant medication, or screened positive via Patient Health Questionnaire (PHQ)-2 or PHQ-9. Age- and sex-matched nondepressed individuals with MS (MS−Depression group; n = 148) included persons who had no prior depression diagnosis, had no psychiatric medication prescriptions, and were asymptomatic on PHQ-2 or PHQ-9. Research-quality 3T structural magnetic resonance imaging was obtained as part of routine care. We first evaluated whether lesions were preferentially located within the depression network compared with other brain regions. Next, we examined if MS+Depression patients had greater lesion burden and if this was driven by lesions in the depression network. Primary outcome measures were the burden of lesions (e.g., impacted fascicles) within a network and across the brain. ResultsMS lesions preferentially affected fascicles within versus outside the depression network (β = 0.09, 95% CI = 0.08 to 0.10, p < .001). MS+Depression patients had more lesion burden (β = 0.06, 95% CI = 0.01 to 0.10, p = .015); this was driven by lesions within the depression network (β = 0.02, 95% CI = 0.003 to 0.040, p = .020). ConclusionsWe demonstrated that lesion location and burden may contribute to depression comorbidity in MS. MS lesions disproportionately impacted fascicles in the depression network. MS+Depression patients had more disease than MS−Depression patients, which was driven by disease within the depression network. Future studies relating lesion location to personalized depression interventions are warranted.

The evolution of multiple sclerosis disease-modifying therapies: An update for pharmacists.

Multiple sclerosis (MS) and the evolution of disease-modifying therapies (DMTs) and their indications, mechanisms of action, efficacy, pregnancy class, and cost are discussed. MS is an immune-mediated, demyelinating, and progressive neurological disorder that can cause both motor and cognitive deficits. Onset of MS typically occurs between the ages of 20 and 40 years, and the disease can result in significant disability over time. Since the introduction of the first DMT for the treatment of MS in 1993, significant progress has been made in the development of new classes of DMTs with different mechanisms of action, higher efficacy, and simpler administration schedules, offering patients better alternatives. However, drawbacks with the use of DMTs include their increasing cost and formulary restrictions. The treatment landscape of MS has significantly changed over the past 2 decades, and the introduction of newer classes of DMTs provides an opportunity for pharmacists to play an important role in the management of this patient population.

Neutrophil-to-lymphocyte ratio is an independent predictor for neurological disability in patients with idiopathic transverse myelitis

IntroductionThe neutrophil-to-lymphocyte ratio (NLR) has been found to be useful in the prognostication of immune-mediated neurological disorders because it roughly reflects the systemic innate immune response compared to the adaptive immune response. However, studies on the validity of NLR in demyelinating disorders of the central nervous system have shown conflicting results. Therefore, we aimed to investigate NLR in the idiopathic transverse myelitis (ITM) cohort.MethodsWe retrospectively analyzed the cohort data of patients with ITM between January 2006 and February 2020. The medical data of all patients with myelitis were reviewed to exclude patients with disease-associated myelopathy according to predefined exclusion criteria. The relationship between the natural log-transformed NLR (lnNLR) and the clinical, paraclinical, and imaging data was evaluated. Factors associated with neurological disability were analyzed using a linear mixed-effects model. Predictive factors for moderate-to-severe neurological disability (Expanded Disability Status Scale [EDSS] score ≥ 4) were investigated.ResultsA total of 124 participants were included in the analysis. The lnNLR correlated with EDSS and lesion length. Linear mixed-effects analysis showed that age, lesion length, and lnNLR were independently associated with neurological disabilities. Multivariable logistic regression revealed that lnNLR (odds ratio [OR] = 4.266, 95% confidence interval [CI] = 1.220–14.912, p = 0.023) and lesion length (OR = 1.848, 95% CI = 1.249–2.734, p = 0.002) were independent predictive factors of the worst neurological disability.ConclusionNLR may be used as an independent prognostic factor for predicting poor neurological outcomes in patients with ITM.

Elevated plasma neurofilament light chain in immune-mediated neurological disorders (IMND) detected by immunomagnetic reduction (IMR)

BackgroundIn cases of immune-mediated neurological disorders (IMND), different syndromes are associated with antibodies against neuronal surface antigens, intra-neuronal antigens, astrocytic aquaporin, and gangliosides. These autoantibodies can be pathogenic or connected to neuroinflammation and resulting neuronal injuries. This study aims to identify a blood biomarker that can detect neuronal damage in individuals with IMND. To this end, we use immunomagnetic reduction (IMR) nanobead technology to measure plasma neurofilament light chain (NfL). MethodsThe patients with IMND were enrolled in the Chang Gung Memorial Hospital at Keelung from 2018 to 2023. Seronegative patients were excluded based on the results of antibody tests. The healthy controls (HC) were community-dwelling adults from the Northeastern Taiwan Community Medicine Research Cohort (NTCMRC) conducted by the Community Medicine Research Center of the Keelung CGMH from 2020 to 2022. IMR technique detects magnetic susceptibility via measuring magnetic signal reduction caused by antigen–antibody immunocomplex formation on magnetic nanobeads. The plasma level of NfL was determined by the magnetic susceptibility changes in IMR. ResultsThe study enrolled 57 IMND patients from the hospital and 73 HC participants from the communities. The plasma NfL was significantly higher in the IMND than in the HC (11.022 ± 2.637 vs. 9.664 ± 2.610 pg/mL, p = 0.004), regardless of age effects on plasma NfL in an analysis of covariance (ANCOVA) (F = 0.720, p = 0.950). In the receiver of operation curve analysis, the area under curve for plasma NfL to discriminate IMND and HC was 0.664 (95% CI = 0.549 to 0.739, p = 0.005).The subgroup analysis of plasma NfL in the IMND patients showed no difference between peripheral immune-mediated neuropathy (IMN) and central immune-mediated encephalomyelitis (IMEM) (11.331 ± 2.895 vs. 10.627 ± 2.260 pg/mL, p = 0.322), nor between tumor and non-tumor IMND (10.784 ± 3.446 vs. 11.093 ± 2.391 pg/mL, p = 0.714). Additionally, the antibody class of ganglioside antibodies in IMN did not have an impact on plasma NfL level (p = 0.857). ConclusionPlasma NfL measurement is a reliable indicator of axonal injuries in patients with IMND. It is equally effective in detecting nerve injuries in inflammatory peripheral neuropathies and central neuroinflammation. The IMR nanobead technology offers a feasible method of detecting plasma NfL, which helps identify axonal injuries in IMND.

The immunopathogenesis of narcolepsy type 1.

Narcolepsy type 1 (NT1) is a chronic sleep disorder resulting from the loss of a small population of hypothalamic neurons that produce wake-promoting hypocretin (HCRT; also known as orexin) peptides. An immune-mediated pathology for NT1 has long been suspected given its exceptionally tight association with the MHC class II allele HLA-DQB1*06:02, as well as recent genetic evidence showing associations with polymorphisms of T cell receptor genes and other immune-relevant loci and the increased incidence of NT1 that has been observed after vaccination with the influenza vaccine Pandemrix. The search for both self-antigens and foreign antigens recognized by the pathogenic T cell response in NT1 is ongoing. Increased T cell reactivity against HCRT has been consistently reported in patients with NT1, but data demonstrating a primary role for T cells in neuronal destruction are currently lacking. Animal models are providing clues regarding the roles of autoreactive CD4+ and CD8+ T cells in the disease. Elucidation of the pathogenesis of NT1 will allow for the development of targeted immunotherapies at disease onset and could serve as a model for other immune-mediated neurological diseases.

Autoimmune Autonomic Ganglionopathy: Solving the 10 Assignments

Autoimmune autonomic ganglionopathy (AAG) is a rare acquired immune-mediated neurological disease that causes various autonomic symptoms. AAG is induced by autoantibodies for the α3 and β4 subunits of the ganglionic acetylcholine receptor (gAChR). gAChR antibodies mediate synaptic transmission in all autonomic ganglia, resulting in dysautonomia. Recent clinical and basic research topics in AAG include the following: 1)analysis of clinical features; 2)novel methods for gAChR antibody detection; 3)efficacy of combined immunotherapy; 4)novel experimental AAG; 5)COVID-19 and mRNA COVID-19 vaccination and its association with autonomic dysfunction; and 6)dysautonomia as an immune-related adverse event of immune checkpoint inhibitors in cancer therapy. The author and his collaborators have previously established "10 assignments" to understand the basic research and clinical issues of AAG. In this review, the author describes the current status of research on each of the "10 assignments," incorporating research trends over the last five years.

Best practices in the use of human immunoglobulin preparations for intravenous administration in the treatment of rare neurological diseases

Aim. To describe best practices in using human normal immunoglobulin in patients with immune-mediated neurological disorders according to the data of one clinical center.Materials and methods. From 2016 to 2021, 20 patients with various autoimmune disorders of the peripheral and central nervous system were treated with human normal immunoglobulin at the Neurology Unit No.1 of Pavlov First Saint Petersburg State Medical University. Treatment efficacy was assessed by changes in the neurological examination data according to specialized scales for specific diseases or clinical manifestations (INCAT, QMGS, MoCA, EDSS). Safety of the therapy was assessed considering the instructions to the drug.Results. In the vast majority of patients, treatment allowed to stabilize the course of the disease or was accompanied by pronounced regression.Conclusion. The considered clinical cases of the use of human normal immunoglobulin preparations demonstrate the possibility of their use in the treatment of a number of autoimmune neurological diseases for unregistered indications.

Anti-mGluR1 encephalitis: Case illustration and systematic review.

The literature for immune-mediated neurological disorders is evolving like no other field of neurological illnesses. Many new antibodies or disorders have been described in the last decade. The cerebellum is a brain structure susceptible to these immune-mediated pathologies, and anti-metabotropic glutamate receptor 1 (mGluR1) antibody has a predilection to the cerebellar tissue. Anti-mGluR1 encephalitis is a rare autoimmune disease affecting the central and peripheral nervous systems, triggering an acute or subacute cerebellar syndrome with varying degrees of severity. Anti-mGluR1 encephalitis is a rare autoimmune disease affecting the central nervous system. We aimed to systematically review reported cases of anti-mGluR1 encephalitis and summarize their clinical presentation, management, outcomes, and case reports. A search of the PubMed and Google Scholar databases was conducted and included all cases of anti-mGluR1 encephalitis published in English before October 1, 2022. A comprehensive systematic review was conducted using "metabotropic glutamate receptor type 1," "mGluR1," autoantibodies," "autoantibodies," "autoimmunity," and "antibody" as keywords. The risk of bias assessment of the evidence was performed using appropriate tools. The qualitative variables were presented as frequency and percentage. Including our case, 36 cases of anti-mGluR1 encephalitis (19 males, median age 52.5 years, 11.1% pediatric cases) have been reported. The most common clinical manifestations are ataxia, dysarthria, and nystagmus. Initial imaging was normal in 44.4% of patients; however, 75% of patients showed abnormality later in the disease course. The first-line therapy options include glucocorticoids, intravenous immunoglobulin, and plasma exchange. Rituximab is the most commonly used second-line treatment. Complete remission was achieved in only 22.2% of patients, and 61.8% were disabled by the end of their course. Anti-mGluR1 encephalitis manifests as symptoms of cerebellar pathology. Although the natural history has not been completely elucidated, early diagnosis with prompt initiation of immunotherapy could be imperative. Any patient suspected to have autoimmune cerebellitis should be tested for the presence of anti-mGluR1 antibody in the serum and cerebrospinal fluid. Escalation to an aggressive therapy approach should be applied in cases that do not respond to first-line therapies, and extended follow-up durations are required in all cases.

Therapeutic Plasma Exchange in Certain Immune-Mediated Neurological Disorders: Focus on a Novel Nanomembrane-Based Technology.

Therapeutic plasma exchange (TPE) is an efficient extracorporeal blood purification technique to remove circulating autoantibodies and other pathogenic substances. Its mechanism of action in immune-mediated neurological disorders includes immediate intravascular reduction of autoantibody concentration, pulsed induction of antibody redistribution, and subsequent immunomodulatory changes. Conventional TPE with 1 to 1.5 total plasma volume (TPV) exchange is a well-established treatment in Guillain-Barre Syndrome, Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Neuromyelitis Optica Spectrum Disorder, Myasthenia Gravis and Multiple Sclerosis. There is insufficient evidence for the efficacy of so-called low volume plasma exchange (LVPE) (<1 TPV exchange) implemented either by the conventional or by a novel nanomembrane-based TPE in these neurological conditions, including their impact on conductivity and neuroregenerative recovery. In this narrative review, we focus on the role of nanomembrane-based technology as an alternative LVPE treatment option in these neurological conditions. Nanomembrane-based technology is a promising type of TPE, which seems to share the basic advantages of the conventional one, but probably with fewer adverse effects. It could play a valuable role in patient management by ameliorating neurological symptoms, improving disability, and reducing oxidative stress in a cost-effective way. Further research is needed to identify which patients benefit most from this novel TPE technology.

Low-volume Plasma Exchange In Immune-mediated Neurological Diseases : A tertiary care center study

BACKGROUND The optimal cost benefit of standard plasma exchange (SPE) has not been met where most of the patients do not afford the treatment. As an alternative, low volume plasma exchange (LVPE) is cost-effective than SPE where albumin replacement is eliminated. The aim of this study was to analyse the efficacy and safety of LVPE in various Immune mediated neurological diseases (IMND).&#x0D; METHODS A hospital-based retrospective study was conducted in patients with IMND who were admitted in TUTH between October 15, 2020, and October 14, 2022. Hemodynamically stable patients over the age of 18 who met the plasma exchange criteria were eligible. Outcomes and treatment-related complications were studied separately for the different diseases.&#x0D; RESULTS Of the 29 patients enrolled, 3 had Myasthenia gravis (MG), 6 had Neuromyelitis optica spectrum disorder (NMOSD), 11 had Gullian Barre syndrome (GBS), 6 had Chronic inflammatory demyelinating polyneuropathy (CIDP), and 3 had Autoimmune encephalitis (AE). Patients with MG, GBS, and CIDP all showed statistically significant improvement in mRS scores from 2.3±0.6 to 0 (p value = 0.0198), 3.03±1.8 to 2.2±1.4 (p value=0.0046) and 2.5±1.4 to 1.8±1(p value=0.025) respectively by the time they were discharged following treatment. Two patients developed Deep vein thrombosis (DVT), and two developed anaphylaxis during the course of their treatment, although none deteriorated further while in the hospital.&#x0D; CONCLUSION LVPE was effective in MG, GBS and CIDP. Myasthenia crisis patients who re-quired mechanical ventilation demonstrated complete resolution with LVPE making it a potential life-saving alternative for those who cannot afford standard therapy.

Opsoclonus-myoclonus syndrome associated with pancreatic neuroendocrine tumor: a case report

BackgroundOpsoclonus-myoclonus syndrome (OMS) is a rare, immune-mediated neurological disorder. In adults, the pathogenesis can be idiopathic, post-infectious or paraneoplastic, the latter etiology belonging to the ever-expanding group of defined paraneoplastic neurological syndromes (PNS). In contrast to other phenotypes of PNS, OMS cannot be ascribed to a single pathogenic autoantibody. Here, we report the first detailed case of paraneoplastic, antibody-negative OMS occurring in association with a pancreatic neuroendocrine tumor (pNET).Case presentationA 33-year-old female presented with a two-week history of severe ataxia of stance and gait, dysarthria, head tremor, myoclonus of the extremities and opsoclonus. Her past medical history was notable for a metastatic pancreatic neuroendocrine tumor, and she was subsequently diagnosed with paraneoplastic opsoclonus-myoclonus syndrome. Further workup did not reveal a paraneoplastic autoantibody. She responded well to plasmapheresis, as she was refractory to the first-line therapy with corticosteroids.ConclusionsThis case expands current knowledge on tumors associated with paraneoplastic opsoclonus-myoclonus syndrome and the age group in which it can occur. It further adds evidence to the effectiveness of plasmapheresis in severe cases of opsoclonus-myoclonus syndrome with a lack of response to first-line therapy.

SARS-CoV-2-Related Acute Necrotizing Encephalopathy of Childhood With Good Response to Tocilizumab in an Adolescent

BackgroundAcute necrotizing encephalopathy of childhood (ANEC) is a rare parainfectious neurological disorder. ANEC is associated with a high mortality rate and poor neurological outcomes. ANEC is postulated to arise from immune-mediated or metabolic processes driven by viral infections. Although there have been some case reports of acute necrotizing encephalopathy with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) coinfection in adults, paediatric cases are rare. MethodsA single case report of SARS-CoV-2-related ANEC in an 11-year-old boy is presented through retrospective chart review. Literature search was performed using PubMed, Embase, Cochrane database, and Google Scholar to compare and analyze similar cases of parainfectious immune-mediated encephalopathies related to SARS-CoV-2 in children. ResultsAn 11-year-old boy with acute SARS-CoV-2 infection presented with ophthalmoplegia, ataxia, and aphasia. Neuroimaging findings demonstrated significant swelling and signal changes in bilateral thalami, brainstem, and cerebellar hemispheres, consistent with ANEC. His high ANEC Severity Score indicated poor neurological prognosis. Treatment with a combination of early steroid therapy, intravenous immunoglobulin therapy, and targeted interleukin 6 (IL-6) blockade yielded good neurological improvements. Literature search identified 19 parainfectious immune-mediated neurological disorders related to SARS-CoV-2 in children. The only other pediatric ANEC case identified was postinfectious and thus not included. ConclusionsThis is the first report of a pediatric case of SARS-CoV-2-related ANEC, which responded well to early immunotherapy, including IL-6 blockade. Early immunotherapy with IL-6 blockade can be considered as an adjunct in managing severe ANEC.

Therapeutic plasma exchange for myasthenia gravis, Guillain-Barre syndrome and other immune-mediated neurological diseases, over a 40-year experience

ABSTRACT Background Therapeutic plasma exchange (TPE) was first used in neurology in the 1980s for myasthenia gravis (MG) and Guillain-Barré syndrome (GBS). Indications have since grown. Fear of complications with this treatment modality limit its use. Research design & methods A study of patients undergoing TPE for neurological diseases (1981-2020) in a university hospital in Madrid, Spain. Clinical indications, complications, procedure number, apheresis technique and replacement fluids were prospectively recorded and retrospectively analysed. Historical trends were studied. Results Over 40 years, 159 patients (48.69±18.15 years, 54.3% females) underwent TPE using central-venous catheter and replacement fluid albumin. We performed 1207 procedures over 189 cycles (6.4±3.8 procedures/cycle). Most patients underwent TPE for category I-II indications, mainly GBS and MG (77.7%). Complication rate was low (3.9% procedures), mostly hypotensive/vasovagal reactions (55.3%) and vascular access-related complications (38.3%). Most were mild-moderate (92.9%), permitting TPE completion, and somewhat more frequent during the first procedure (38.3%) and after periods of little TPE use. GBS patients were more prone to complications than MG patients (6.5% vs. 1.2%,p<0.001) mainly hypotensive/vasovagal reactions (3.7% vs. 1.0%,p=0.008). Conclusions TPE is well-tolerated with low complication rate (<4% procedures), mainly hypotensive/vasovagal reactions. Patients with GBS seem more prone to them than MG patients. Acquaintance with this technique seems necessary.

Palliative Care for Patients With Multiple Sclerosis: Recommendations Emerging From a Case Study.

Multiple sclerosis (MS) affects more than 2.8 million people worldwide and is an incurable, heterogeneous, chronic, degenerative, demyelinating, immune-mediated neurological disease of the central nervous system. It affects the physical, mental, psychosocial, financial, and spiritual dimensions of patients and their families. Given this illness trajectory and the multiple complex symptoms associated with MS, palliative care services would improve the quality of life for MS patients. Palliative care is a human right for all patients with a life-limiting, progressive disease. The goal of palliative care is the prevention and relief of suffering by means of assessment and treatment that holistically addresses symptoms and suffering. Thus, this article argues for the early integration of palliative care for persons given a diagnosis of MS. This argument is underscored by the analysis of a case study of a typical patient with MS who would have benefited from conjunctive palliative care.

Utilisation of immunoglobulin in New Zealand.

Immunoglobulin is an expensive and scarce resource and usage is increasing worldwide. Immunoglobulin is used to treat a variety of clinical conditions, particularly primary and acquired immunodeficiencies and immune-mediated neurological disorders. As immunoglobulin usage continues to increase, plasma collection must increase accordingly in order to sustain immunoglobulin production. The New Zealand Blood Service (NZBS) is the provider of immunoglobulin in New Zealand (NZ). Information regarding national immunoglobulin usage warrants analysis given the rise in usage. To review immunoglobulin usage in NZ with a focus on the trend in the amount used, number of patients, clinical indications and compliance with international guidelines. A comparison with international immunoglobulin usage was performed. The impact on national plasma collection was reviewed. Data on immunoglobulin usage, number of patients and plasma collection over the past decade were obtained from the NZBS Tableau database. Data from international literature were reviewed. Immunoglobulin usage in NZ has been increasing over the past decade, with an annual growth rate of 6.4%. The three main indications for immunoglobulin are primary immunodeficiency disorders, chronic inflammatory demyelinating polyneuropathy (CIDP) and acquired hypogammaglobulinaemia secondary to haematological malignancies. Prominent growth in usage is evident for CIDP and acquired hypogammaglobulinaemia. Immunoglobulin usage in NZ is low compared with other countries, such as Australia and the United States. There has been a marked increase in plasma donations in order to keep up with immunoglobulin demand. Immunoglobulin is a strategic resource and appropriate usage is critical to regulate demand.

Autoimmune neuromyotonia.

Autoimmune neuromyotonia encompasses a group of rare immune-mediated neurological disorders frequently associated with anti-contactin-associated protein-like 2 (CASPR2) antibodies and featuring clinical and electrical signs of peripheral nerve hyperexcitability (PNH). We aim to summarize the current knowledge on immune-mediated neuromyotonia, focusing on clinical presentations, pathophysiology, and management. Neuromyotonia is a major feature of several autoimmune neurological syndromes characterized by PNH with or without central neurological system involvement. Experimental and clinical evidence suggest that anti-CASPR2 antibodies are directly pathogenic in autoimmune neuromyotonia patients. Neuromyotonia, a form of PNH, is a major feature in several syndromes associated with anti-CASPR2 antibodies, including cramp-fasciculation syndrome, Isaacs syndrome, Morvan syndrome, and autoimmune limbic encephalitis. Diagnosis relies on the identification of motor, sensory, and autonomic signs of PNH along with other neurological symptoms, anti-CASPR2 antibody-positivity, and of characteristic electroneuromyographic abnormalities. Paraneoplastic associations with thymoma are possible, especially in Morvan syndrome. Patients usually respond to immune-active treatments, including steroids, intravenous immunoglobulins, plasma exchanges, and rituximab.