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autoimmune polyneuropathy

S Pereira, C M Cruz, M Soares, J Gandara, S Ferreira, V Lopes, R Vizcaíno, J Daniel, H P Miranda
INTRODUCTION: In liver transplantation, late graft dysfunction can have several causes, particularly rejection, infection, vascular, biliary complications, and others, usually suspected by abnormal liver tests. However, normal liver tests do not confirm a normal graft and liver biopsy could identify unexpected features with repercussions in immunosuppressive therapy. The aim of this study was to determinate the histological abnormalities in patients 10 years after liver allograft transplantation with sustainably normal liver tests and evaluate the changes in immunosuppressive therapy triggered by histological data...
September 2016: Transplantation Proceedings
Vikram A Londhey
SLE (systemic lupus erythematosus) is a multisystem autoimmune disorder of unknown aetiology which can present with myriad clinical presentation. The neurological manifestations of SLE consist of central nervous system (CNS) and peripheral nervous system manifestations (PNS). The CNS manifestations are aseptic meningitis, cerebrovascular accidents (stroke), demyelinating disorders, headache, involuntary movements like chorea, myelopathy, acute confusional states, cognitive dysfunction, mood disorder, seizures, psychosis and cranial nerve palsies...
December 2015: Journal of the Association of Physicians of India
Susanna Esposito, Maria Roberta Longo
The term Guillain-Barré syndrome (GBS), the most frequent cause of acute paralytic neuropathy, covers a number of recognisably distinct variants. The exact cause of GBS is unknown, but 50-70% of cases appear 1-2weeks after a respiratory or gastrointestinal infection, or another immune stimulus that induces an aberrant autoimmune response targeting peripheral nerves and their spinal roots. The interplay between the microbial and host factors that dictate whether and how the immune response shifts towards autoreactivity is still unclear, and nothing is known about the genetic and environmental factors that affect an individual's susceptibility to the disease...
September 23, 2016: Autoimmunity Reviews
Juan M Racosta, Luciano A Sposato, Kurt Kimpinski
INTRODUCTION: High-dose intravenous immunoglobulin (IVIg) is an evidence-based treatment for multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyneuropathy (CIDP). Recently, subcutaneous immunoglobulin (SC-Ig) has received increasing attention. METHODS: We performed a meta-analysis of reports of efficacy and safety of SC-Ig versus IVIg for inflammatory demyelinating polyneuropathies. RESULTS: A total of 8 studies comprising 138 patients (50 with MMN and 88 with chronic CIDP) were included in the meta-analysis...
September 20, 2016: Muscle & Nerve
Isaak Quast, Christian W Keller, Falk Hiepe, Björn Tackenberg, Jan D Lünemann
Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune neuropathy. While both cell-mediated and humoral mechanisms contribute to its pathogenesis, the rapid clinical response to plasmapheresis implicates a circulating factor responsible for peripheral nerve injury. We report that treatment-naïve patients with CIDP show increased serum and CSF levels of the anaphylatoxin C5a and the soluble terminal complement complex (sTCC). Systemic terminal complement activation correlates with clinical disease severity as determined by the Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale...
September 2016: Annals of Clinical and Translational Neurology
Rekha Ramaswamy Iyer, Pragnesh Hasmukhlal Shah, Sher Sankar K Roy, Sushil Kumar Kundanlal Suri
BACKGROUND: Guillain-Barre syndrome (GBS) is an autoimmune acute inflammatory demyelinating polyneuropathy affecting the peripheral nervous system treated with high-dose immunoglobulin, physical therapy, or plasmapheresis. Immunoglobulins are expensive and even plasmapheresis might not be affordable to patients visiting government set-ups. AIMS: This study was undertaken to emphasize the efficacy of plasmapheresis in treatment of adult GBS patients and to narrate methods of reducing the economic burden in the treatment of these patients using modified plasmapheresis...
July 2016: Asian Journal of Transfusion Science
Rajat Lahoria, Sean J Pittock, Avi Gadoth, JaNean K Engelstad, Vanda A Lennon, Christopher J Klein
INTRODUCTION: Voltage-gated Kv1 potassium channel-complex (VGKC) autoantibodies subtyped for leucine-rich-glioma-inactivated-1 (LGI1), contactin-associated-protein-like-2 (CASPR2), and Kv-IgGs have a spectrum of neurological presentations. Painful polyneuropathy is seen in some patients, but nerve pathology descriptions are lacking. METHODS: Clinicopathologic features were studied in subtyped VGKC-autoantibody seropositive patients who had undergone nerve biopsies...
August 11, 2016: Muscle & Nerve
Livia Dézsi, Zoltán Horváth, László Vécsei
INTRODUCTION: Intravenous immunoglobulin (IVIg) is increasingly used for the treatment of autoimmune and systemic inflammatory diseases with both licensed and off-label indications. The mechanism of action is complex and not fully understood, involving the neutralization of pathological antibodies, Fc receptor blockade, complement inhibition, immunoregulation of dendritic cells, B cells and T cells and the modulation of apoptosis. AREAS COVERED: First, this review describes the pharmacological properties of IVIg, including the composition, mechanism of action, and adverse events...
August 22, 2016: Expert Opinion on Drug Metabolism & Toxicology
Valeria Katchan, Paula David, Yehuda Shoenfeld
Concurrent association of idiopathic thrombocytopenic purpura (ITP) and peripheral neuropathy is a rare condition. There are only few case reports published concerning peripheral neuropathy with ITP. One of the etiopathogenetic mechanisms proposed is intraneural hemorrhage, but the pathogenesis is not fully understood. Autoimmune nature with common antibodies to the platelets and the nerve´s myelin sheath should also be considered. Here we describe a 47-year-old woman, with a family history of autoimmune diseases...
July 18, 2016: Immunologic Research
Zheng Li, Jianxiong Shen, Jinqian Liang, Fan Feng
Guillain-Barré syndrome (GBS) is an acute autoimmune inflammatory demyelinating polyneuropathy that mostly affects the peripheral nervous system. Little is reported about spinal deformity associated with GBS. This study aims to present a case of scoliosis occurring in the setting of GBS.Case report and literature review.The patient was a 14-year-old male with scoliosis. His spinal plain radiographs showed that the Cobb angle of thoracic scoliosis was 114°. History review revealed that he developed profound lower extremity pain, weakness, and numbness after catching a cold 5 years ago...
June 2016: Medicine (Baltimore)
Z Ren, D Y Hsu, J Brieva, N B Silverberg, S M Langan, J I Silverberg
BACKGROUND: Bullous pemphigoid (BP) is associated with significant disability and comorbid health disorders that may lead to or result from hospitalization. However, little is known about the inpatient burden and comorbidities of BP. METHODS: We analyzed data from the 2002-2012 National Inpatient Sample, including a representative 20% sample of all hospitalizations in the US (n=72,108,077 adults). RESULTS: The prevalences of hospitalization for BP increased from 25...
June 25, 2016: British Journal of Dermatology
Margherita Nosadini, Shekeeb S Mohammad, Agnese Suppiej, Stefano Sartori, Russell C Dale
AIM: Intravenous immunoglobulin (IVIG) is an expensive therapy used in immunodeficiency and autoimmune disorders. Increasing demands and consequent shortages result in a need for usage to conform to guidelines. METHOD: We retrospectively evaluated IVIG use for neuroimmunological indications and adherence to existing guidelines in a major Australian paediatric hospital between 2000 and 2014. RESULTS: One-hundred and ninety-six children (96 male, 100 female; mean age at disease onset 6y 5mo [range 3mo-15y 10mo], mean age at first IVIG dose 7y 2mo [range 3mo-16y 5mo]) received IVIG for neuroimmunological indications during the study period (28...
May 31, 2016: Developmental Medicine and Child Neurology
Rustam H Ziganshin, Olga M Ivanova, Yakov A Lomakin, Alexey A Belogurov, Sergey I Kovalchuk, Igor V Azarkin, Georgij P Arapidi, Nikolay A Anikanov, Victoria O Shender, Mikhail A Piradov, Natalia A Suponeva, Anna A Vorobyeva, Alexander G Gabibov, Vadim T Ivanov, Vadim M Govorun
Acute inflammatory demyelinating polyneuropathy (AIDP) - the main form of Guillain-Barre syndrome-is a rare and severe disorder of the peripheral nervous system with an unknown etiology. One of the hallmarks of the AIDP pathogenesis is a significantly elevated cerebrospinal fluid (CSF) protein level. In this paper CSF peptidome and proteome in AIDP were analyzed and compared with multiple sclerosis and control patients. A total protein concentration increase was shown to be because of even changes in all proteins rather than some specific response, supporting the hypothesis of protein leakage from blood through the blood-nerve barrier...
July 2016: Molecular & Cellular Proteomics: MCP
Nikolai Nikolov, Jürgen Reisinger, Hans P Schwarz
Intravenous immunoglobulins have been used to treat autoimmune disorders (ADs) for over 50 years. The etiologies of various ADs are not fully understood and although intravenous immunoglobulin treatment has proved its immunomodulatory properties, the roles of proposed mechanisms of action also remain a matter of speculation. A systemic search of the literature regarding KIOVIG(®) (Baxalta US, Inc., MA, USA) use in clinical trials on patients with ADs and a detailed review of retrieved articles revealed eight relevant publications...
July 2016: Immunotherapy
Jung-Ick Byun, Soon-Tae Lee, Keun-Hwa Jung, Jun-Sang Sunwoo, Jangsup Moon, Tae-Joon Kim, Jung-Ah Lim, Soyun Kim, Do-Yong Kim, Su-Hyun Han, Hyemin Jang, Hong Il Suh, A-Hyun Cho, Dong Wook Kim, Jung-Won Shin, Yong Seo Koo, Woo Chan Choi, Woong-Woo Lee, Nari Choi, Seongheon Kim, Hyunwoo Nam, Dae Lim Koo, Minah Kim, Byung Chan Lim, Jong-Hee Chae, Ki Joong Kim, Daejong Jeon, Kyung-Il Park, Ki-Young Jung, Manho Kim, Kon Chu, Sang Kun Lee
We aimed to evaluate the prevalence of antineuronal antibodies in a nationwide cohort of patients with encephalopathy of unknown etiology. We screened 1699 patients with idiopathic encephalopathy who were referred from 70 hospitals across Korea for autoimmune synaptic and classic paraneoplastic antibodies. Those with cerebellar degeneration, sensory polyneuropathy or other paraneoplastic syndromes without encephalopathy were not included in this study. One-hundred and four patients (6.12%) had antibody-associated autoimmune encephalopathy...
April 15, 2016: Journal of Neuroimmunology
N K Kharwar, K N Prasad, V K Paliwal, D R Modi
BACKGROUND: Nucleotide oligomerization domain (NOD) proteins are cytosolic pattern recognition receptors that respond to bacterial substrate and induce NF-κB activation in host. Association of NOD polymorphisms have been studied in many autoimmune disorders, however its role in Guillain-Barré syndrome (GBS) remains unknown. We have investigated NOD1 Glu266Lys and NOD2 (Arg702Trp and Gly908Arg) gene polymorphisms among patients with GBS. MATERIALS AND METHOD: Polymorphisms in NOD-1 (Glu266Lys) and NOD-2 (Arg702Trp and Gly908Arg) genes were studied using polymerase chain reaction-restriction fragment length polymorphism in 105 patients with GBS and 100 healthy controls...
April 15, 2016: Journal of the Neurological Sciences
Federico A Filippín, Alejandra Alfonso, Héctor López-Presas
INTRODUCTION: The celiac disease is an autoimmune bowel disease trigger by gliadine proteins, that can has systemic involvement with varied neurological manifestation since polineuropathy, ataxia, epilepsy, migraine to encephalitis. The aim of the current work is to report a case of possible refractory encephalitis due to celiac disease. CASE REPORT: A 45 years-old man with histopathologic diagnosis of celiac disease but negative celiac antibodies developed encephalic, brainstem and periphery nervous system manifestations: cognitive declaim and seizures, diplopia and ataxia, polyneuropathy and polyradiculopathy respectively; inflammatory cebrospinal fluid and cortico-subcortical and multiple brainstem lesions with mild contrast enhancement; the neurologic deficits progress in spite of gluten a free diet and immuno-suppressive treatment...
April 1, 2016: Revista de Neurologia
Panagiotis Zis, Ptolemaios Georgios Sarrigiannis, Dasappaiah Ganesh Rao, Channa Hewamadduma, Marios Hadjivassiliou
Chronic idiopathic axonal polyneuropathy (CIAP) is a term describing neuropathies with both sensory and motor involvement in a length dependant distribution where neurophysiology reveals axonal damage, neuropathy onset is insidious and shows slow or no progression of the disease over at least 6 months with no aetiology being identified despite appropriate investigations. This entity merits further consideration given how common it is, the absence of clarity regarding aetiopathogenesis, natural history and therapies...
October 2016: Journal of Neurology
G Marrali, P Salamone, F Casale, G Fuda, P Cugnasco, C Caorsi, A Amoroso, A Calvo, L Lopiano, D Cocito, A Chiò
BACKGROUND AND PURPOSE: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immunomediated condition affecting the peripheral nervous system where probably macrophages are the primary effector cells for demyelination. Reactive oxygen species (ROS), catalyzed by the NOX family of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase enzymes, can induce peroxidation and are potentially injurious to myelin. Our aim was to assess the activity of NOX2, an isoform of NOX, in a series of CIDP patients and to analyze the effect of intravenous immunoglobulin (IVIg) on NOX2...
May 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Ursula Vogl, Gerda Leitner, Assunta Dal-Bianco, Marija Bojic, Margit Mitterbauer, Werner Rabitsch, Peter Kalhs, Axel Schulenburg
UNLABELLED: Neurologic complications after allogeneic hematopoietic stem cell transplantation (HSCT) are rare but poorly understood. We present a case report of a 57-year-old-male patient who was diagnosed in 2009 with acute myeloid leukemia (AML). He received two standard induction chemotherapies, as well as a following consolidation. Six months later, an allogeneic HSCT was performed. Shortly after HSCT the patient developed progressive polyneuropathy of the lower legs and hypoesthesia...
May 2016: Wiener Klinische Wochenschrift
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