scholarly journals The Role of Plasma Exchange in the Treatment of Refractory Autoimmune Neurological Diseases: a Narrative Review

2021 ◽  
Author(s):  
Saiju Jacob ◽  
Gordon Mazibrada ◽  
Sarosh R Irani ◽  
Anu Jacob ◽  
Anna Yudina
Keyword(s):  
Plasma Exchange ◽  
Neurological Disorders ◽  
Neurological Diseases ◽  
Autoimmune Encephalitis ◽  
Complete Recovery ◽  
Success Rates ◽  
Society Meeting ◽  
Relapsing Remitting ◽  
Inflammatory Neuropathies

AbstractAutoimmune neurological disorders are commonly treated with immunosuppressive therapy. In patients with refractory conditions, standard immunosuppression is often insufficient for complete recovery or to prevent relapses. These patients rely on other treatments to manage their disease. While treatment of refractory cases differs between diseases, intravenous immunoglobulin, plasma exchange (PLEX), and immune-modulating treatments are commonly used. In this review, we focus on five autoimmune neurological disorders that were the themes of the 2018 Midlands Neurological Society meeting on PLEX in refractory neurology: Autoimmune Encephalitis (AE), Multiple Sclerosis (MS), Neuromyelitis Optica Spectrum disorders (NMOSD), Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) and Myasthenia Gravis (MG). The diagnosis of inflammatory neuropathies is often challenging, and while PLEX can be very effective in refractory autoimmune diseases, its ineffectiveness can be confounded by misdiagnosis. One example is POEMS syndrome (characterized by Polyneuropathy Organomegaly, Endocrinopathy, Myeloma protein, Skin changes), which is often wrongly diagnosed as CIDP; and while CIDP responds well to PLEX, POEMS does not. Accurate diagnosis is therefore essential. Success rates can also differ within ‘one’ disease: e.g. response rates to PLEX are considerably higher in refractory relapsing remitting MS compared to primary or secondary progressive MS. When sufficient efforts are made to correctly pinpoint the diagnosis along with the type and subtype of refractory autoimmune disease, PLEX and other immunotherapies can play a valuable role in the patient management. Graphical abstract

Microbiota-Immune System Interactions in Human Neurological Disorders

2020 ◽  
Vol 19 (7) ◽  
pp. 509-526
Author(s):  
Qin Huang ◽  
Fang Yu ◽  
Di Liao ◽  
Jian Xia
Keyword(s):  
Immune System ◽  
Gut Microbiota ◽  
Neurological Disorders ◽  
Brain Function ◽  
Neurological Diseases ◽  
Host Immune System ◽  
Gut Dysbiosis ◽  
Characteristic Features ◽  
Novel Therapeutic Strategies

: Recent studies implicate microbiota-brain communication as an essential factor for physiology and pathophysiology in brain function and neurodevelopment. One of the pivotal mechanisms about gut to brain communication is through the regulation and interaction of gut microbiota on the host immune system. In this review, we will discuss the role of microbiota-immune systeminteractions in human neurological disorders. The characteristic features in the development of neurological diseases include gut dysbiosis, the disturbed intestinal/Blood-Brain Barrier (BBB) permeability, the activated inflammatory response, and the changed microbial metabolites. Neurological disorders contribute to gut dysbiosis and some relevant metabolites in a top-down way. In turn, the activated immune system induced by the change of gut microbiota may deteriorate the development of neurological diseases through the disturbed gut/BBB barrier in a down-top way. Understanding the characterization and identification of microbiome-immune- brain signaling pathways will help us to yield novel therapeutic strategies by targeting the gut microbiome in neurological disease.

scholarly journals Therapeutic Plasma Exchange as a Treatment for Autoimmune Neurological Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Ivana Nieto-Aristizábal ◽  
Álvaro J. Vivas ◽  
Pablo Ruiz-Montaño ◽  
Cristian C. Aragón ◽  
Iván Posso-Osorio ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Medical Records ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Neurological Diseases ◽  
Statistical Significance ◽  
Autoimmune Encephalitis ◽  
Therapeutic Plasma Exchange ◽  
Replacement Solution ◽  
Spectrum Disorders ◽  
Number Of Cycles

Introduction. Therapeutic plasma exchange (TPE) is commonly used as treatment of certain autoimmune neurological diseases (ANDs), and its main objective is the removal of pathogenic autoantibodies. Our aim was to describe the clinical profile and the experience with the usage of TPE in patients with ANDs at our institution. Methods. This is an observational retrospective study, including medical records of patients with diagnosis of ANDs who received TPE, between 2011 and 2018. Characteristics of TPE, such as number of cycles, type of replacement solution, and adverse effects, were evaluated. The modified Rankin Scale (mRS) was applied to measure the clinical response after the therapy. Results. 187 patients were included with the following diagnoses: myasthenia gravis (MG), n = 70 (37%); Guillain–Barré syndrome (GBS), n = 53 (28.3%), neuromyelitis optica spectrum disorders (NMOSD), n = 35 (18.7%); chronic inflammatory demyelinating polyneuropathy (CIDP), n = 23 (12.2%); and autoimmune encephalitis (AE), n = 6 (3.2%). The most used types of replacement solution were albumin (n = 131, 70%) and succinylated gelatin (n = 45, 24%). All patients received a median of five cycles (IQR 5-5). Hypotension and hydroelectrolytic disorders were the main complications. After TPE, 99 patients (52.9%) showed improvement in the mRS scores and a statistical significance (p<0.05) was seen between the admission score and after TPE for every diagnosis except for CIDP. Conclusion. TPE has an adequate safety profile, and improvement in functionality in treated patients reflects its effectiveness.

scholarly journals Role of Enzymes in Causing Neurological Disorders

2021 ◽  
Vol 12 (1) ◽  
pp. 466-476
Author(s):  
Vysakh Visweswaran ◽  
Roshni PR
Keyword(s):  
Neurological Disorders ◽  
Drug Targets ◽  
Molecular Mechanisms ◽  
Neurological Diseases ◽  
Treatment Options ◽  
Direct Result ◽  
Permanent Disability ◽  
Genetic Abnormalities ◽  
Underlying Pathology

Diseases of the nervous system are always associated with poor prognosis and limited treatment options. The fragile nature of the neurons and their inability to replicate means that neurological disorders are associated with a permanent disability. Pharmacotherapy of neurological diseases requires understanding the molecular mechanisms involved in the disease pathology. In most of the cases a faulty cellular biochemical pathway is involved, resulting from a defective enzyme. This article focusses on role of enzymes in various neurological disorders. To review pertinent literature and summarise the role of enzymes in the underlying pathology of various neurological disorders. A comprehensive literature search was conducted using PubMed, SCOPUS, J-GATE and Google Scholar and relevant papers were collected using the keywords enzymes, Alzheimer's disease, redox, thiamine, depression, neurotransmitters, epileptogenesis. The literature review highlighted the role of enzymes in major neurological disorders and their potential to be used as drug targets and biomarkers. Identifying defective enzymes gives us new molecular targets to focus on for developing more effective pharmacotherapeutic options. They can be also considered as potential biomarkers. An abnormal enzyme is most often a direct result of an underlying genetic abnormality. Identifying and screening for these genetic abnormalities can be used in early identification and prevention of disease in individuals who have a genetic predisposition. The modern advances in genetic engineering shows a lot of promise in correcting these abnormalities and development of revolutionary cures although ethical concerns remain. 

scholarly journals Insights Into the Role of CSF1R in the Central Nervous System and Neurological Disorders

2021 ◽  
Vol 13 ◽  
Author(s):  
Banglian Hu ◽  
Shengshun Duan ◽  
Ziwei Wang ◽  
Xin Li ◽  
Yuhang Zhou ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neurological Disorders ◽  
Neurological Diseases ◽  
Colony Stimulating Factor ◽  
Loss Of Function ◽  
Axonal Spheroids ◽  
The Central Nervous System ◽  
Stimulating Factor

The colony-stimulating factor 1 receptor (CSF1R) is a key tyrosine kinase transmembrane receptor modulating microglial homeostasis, neurogenesis, and neuronal survival in the central nervous system (CNS). CSF1R, which can be proteolytically cleaved into a soluble ectodomain and an intracellular protein fragment, supports the survival of myeloid cells upon activation by two ligands, colony stimulating factor 1 and interleukin 34. CSF1R loss-of-function mutations are the major cause of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) and its dysfunction has also been implicated in other neurodegenerative disorders including Alzheimer’s disease (AD). Here, we review the physiological functions of CSF1R in the CNS and its pathological effects in neurological disorders including ALSP, AD, frontotemporal dementia and multiple sclerosis. Understanding the pathophysiology of CSF1R is critical for developing targeted therapies for related neurological diseases.

scholarly journals Store-Operated Calcium Channels in Physiological and Pathological States of the Nervous System

2020 ◽  
Vol 14 ◽  
Author(s):  
Isis Zhang ◽  
Huijuan Hu
Keyword(s):  
Nervous System ◽  
Calcium Channels ◽  
Neurological Disorders ◽  
Neurological Diseases ◽  
Physiological Role ◽  
The Body ◽  
Store Operated Calcium Entry ◽  
Important Pathway ◽  
Molecular Components

Store-operated calcium channels (SOCs) are widely expressed in excitatory and non-excitatory cells where they mediate significant store-operated calcium entry (SOCE), an important pathway for calcium signaling throughout the body. While the activity of SOCs has been well studied in non-excitable cells, attention has turned to their role in neurons and glia in recent years. In particular, the role of SOCs in the nervous system has been extensively investigated, with links to their dysregulation found in a wide variety of neurological diseases from Alzheimer’s disease (AD) to pain. In this review, we provide an overview of their molecular components, expression, and physiological role in the nervous system and describe how the dysregulation of those roles could potentially lead to various neurological disorders. Although further studies are still needed to understand how SOCs are activated under physiological conditions and how they are linked to pathological states, growing evidence indicates that SOCs are important players in neurological disorders and could be potential new targets for therapies. While the role of SOCE in the nervous system continues to be multifaceted and controversial, the study of SOCs provides a potentially fruitful avenue into better understanding the nervous system and its pathologies.

scholarly journals Therapeutic Plasma Exchange – A 2 Years Experience at a Tertiary Care Centre in Mumbai, Maharashtra, India

2021 ◽  
Vol 10 (15) ◽  
pp. 1069-1073
Author(s):  
Moni Mukesh Udani ◽  
Akanksha Jivrag Neogi ◽  
Shweta Wasudeo Dhote ◽  
Iqbal Singh
Keyword(s):  
Plasma Exchange ◽  
Neurological Diseases ◽  
Tertiary Care ◽  
Complete Recovery ◽  
Therapeutic Plasma Exchange ◽  
Effective Procedure ◽  
Guillain Barre Syndrome ◽  
Tertiary Care Centre ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

BACKGROUND Therapeutic plasma exchange is a process where the blood collected from patient is passed through an apheresis instrument where the plasma is removed and discarded and reinfusion of blood cells done with replacement fluids like plasma or albumin to the patient.1 It is to remove pathogenic autoantibodies, immune complexes, cryoglobulins and toxins present in the plasma. Plasma exchange is considered effective and cheaper immunomodulatory treatment when compared to intravenous immunoglobulin (IVIG). 2 We present our institutional experience with therapeutic plasma exchange (TPE) in treatment of various non-neurological and neurological diseases. Our study was conducted to assess the indications, complications and outcome of TPE in the treatment of patients. METHODS A retrospective study of TPE procedures was carried out in the Department of Immunohaematology and blood transfusion, M.G.M Medical College and Hospital, Navi Mumbai from June 2018 to June 2020. A total of 45 procedures were performed among 13 patients between 4 years of age to 66 years of age. Clinical parameters were checked, and laboratory investigations were done before the procedure. Data was collected from the requisition forms by the clinicians and the apheresis database. RESULTS A total of 47 procedures were carried out among 13 patients. TPE is a safe and effective procedure for treating patients with neurologic and non-neurological diseases. Most common indication was Guillain Barre syndrome followed by myasthenia gravis. Incidence of adverse reactions was 7.6 %. CONCLUSIONS TPE is a safe and effective procedure for treating patients with neurologic and nonneurological diseases. It benefited 10 out of 13 patients, and they showed complete recovery. KEY WORDS Therapeutic Plasma Exchange, Guillain Barre syndrome

scholarly journals Autoimmune Encephalitis and CSF Anti-GluR3 Antibodies in an MS Patient after Alemtuzumab Treatment

2019 ◽  
Vol 9 (11) ◽  
pp. 299 ◽  
Author(s):  
Maria Chiara Buscarinu ◽  
Arianna Fornasiero ◽  
Giulia Pellicciari ◽  
Roberta Reniè ◽  
Anna Chiara Landi ◽  
...  
Keyword(s):  
Cognitive Deficits ◽  
Focal Epilepsy ◽  
Brain Magnetic Resonance Imaging ◽  
Autoimmune Encephalitis ◽  
Complete Recovery ◽  
Neurological Status ◽  
Relapsing Remitting ◽  
The Central Nervous System ◽  
Relapsing Remitting Multiple Sclerosis ◽  
Serial Brain

A 45-year-old Italian woman, affected by relapsing–remitting multiple sclerosis (RR-MS) starting from 2011, started treatment with alemtuzumab in July 2016. Nine months after the second infusion, she had an immune thrombocytopenic purpura (ITP) with complete recovery after steroid treatment. Three months after the ITP, the patient presented with transient aphasia, cognitive deficits, and focal epilepsy. Serial brain magnetic resonance imaging showed a pattern compatible with encephalitis. Autoantibodies to glutamate receptor 3 peptide A and B were detected in cerebrospinal fluid and serum, in the absence of any other diagnostic cues. After three courses of intravenous immunoglobulin (0.4 mg/kg/day for 5 days, 1 month apart), followed by boosters (0.4 mg/kg/day) every 4–6 weeks, her neurological status improved and is currently comparable with that preceding the encephalitis. Autoimmune complications of the central nervous system during alemtuzumab therapy are relatively rare: only one previous case of autoimmune encephalitis following alemtuzumab treatment has been reported to date.

Regulating inflammation associated Ferroptosis-a treatment strategy for Parkinson disease

2021 ◽  
Vol 28 ◽  
Author(s):  
Marthandam Asokan Shibu ◽  
B. Mahalakshmi ◽  
V. Bharath Kumar
Keyword(s):  
Clinical Trials ◽  
Cell Death ◽  
Treatment Strategy ◽  
Neurological Disorders ◽  
Neurological Diseases ◽  
Underlying Mechanism ◽  
Acute Injury ◽  
Brain Iron ◽  
Kidney Tumors

: Ferroptosis plays a critical regulatory role for a new kind of cell death initiating and developing an array of disorders like neurological diseases, acute injury of kidney, tumors and ischemia etc. This selective deposition of iron is one of the pathogenic reasons for PD and although it’s underlying mechanism is still unknown. In this review, the role of neuroinflammation in Parkinson’s disease (PD) leading to neurodegeneration has been discussed in detail. The accumulation of brain iron has been found in many chronic neurological disorders including PD. We have also discussed the unique features of Ferroptosis as compared to other cellular death pathways and it links in aggravating the pathology of PD. Further, the concept of targeting Ferroptosis for PD pathology and inducers and inhibitors, pharmacological drugs and clinical trials for PD candidates in phase IV stage in completed status are detailed in the respective sections.

scholarly journals Elevated platelet-bound IgG associated with an episode of thrombotic thrombocytopenic purpura

Blood ◽  
1981 ◽  
Vol 58 (4) ◽  
pp. 682-684 ◽  
Author(s):  
PJ Sims ◽  
EB Boswell
Keyword(s):  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Successful Treatment ◽  
Combined Therapy ◽  
Complete Recovery ◽  
Antiplatelet Drugs ◽  
High Dose ◽  
Normal Range ◽  
Thrombocytopenic Purpura

Abstract The level of platelet-associated IgG (PAIgG) were measured during the successful treatment of a patient with thrombotic thrombocytopenic purpura. Prior to therapy. PAIgG was found to be markedly elevated to 195 fg/cell (normal range 0--3.5 fg/cell). The institution of combined therapy with intensive plasma exchange transfusions, high-dose steroids, and antiplatelet drugs resulted in a complete recovery and a decline in PAIgG to the normal range. The possible role of platelet antibody in the pathogenesis of this disorder is discussed.

scholarly journals Nutraceuticals in Neurological Disorders

2020 ◽  
Vol 21 (12) ◽  
pp. 4424 ◽  
Author(s):  
Rashita Makkar ◽  
Tapan Behl ◽  
Simona Bungau ◽  
Gokhan Zengin ◽  
Vineet Mehta ◽  
...  
Keyword(s):  
Side Effects ◽  
Neurological Disorders ◽  
Functional Foods ◽  
Neurological Diseases ◽  
Financial Burden ◽  
Lifestyle Changes ◽  
Increased Risk ◽  
Medicinal Foods ◽  
The Individual

Neurological diseases are one of the major healthcare issues worldwide. Posed lifestyle changes are associated with drastically increased risk of chronic illness and diseases, posing a substantial healthcare and financial burden to society globally. Researchers aim to provide fine treatment for ailing disorders with minimal exposed side effects. In recent decades, several studies on functional foods have been initiated to obtain foods that have fewer side effects and increased therapeutic activity. Hence, an attempt has been made to unravel several extraction techniques to acquire essential bioactive compounds or phytochemicals from therapeutically active food products. This has led to the conception of the term functional foods being meddled with other similar terms like “pharmafoods,” “medifoods”, “vitafoods”, or “medicinal foods”. With a dire need to adhere towards healthy options, the demand of nutraceuticals is widely increasing to combat neurological interventions. An association between food habits and the individual lifestyle with neurodegeneration has been manifested, thereby proposing the role of nutraceuticals as prophylactic treatment for neurological interventions. The current review covers some of the major neurological disorders and nutraceutical therapy in the prevention of disease.

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