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chronic inflammatory demyelinating polyneuropathy

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https://www.readbyqxmd.com/read/29780925/ocular-involvement-in-neurolymphomatosis
#1
Katy C Liu, Meridith A Hennessey, Chad M McCall, Alan D Proia
Purpose: To describe the ophthalmic symptoms and histopathological findings in a case of primary neurolymphomatosis (NL). Observations: A man in his 60s with a prior diagnosis of chronic inflammatory demyelinating polyneuropathy developed facial numbness, diplopia, drooling, and difficulty swallowing. Over a 3-month period, he developed total ptosis and ophthalmoplegia of the right eye with a dilated, non-reactive pupil considered secondary to cranial nerve III and VI palsies...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29764262/igpro20-the-polyneuropathy-and-treatment-with-hizentra-%C3%A2-study-path-and-the-treatment-of-chronic-inflammatory-demyelinating-polyradiculoneuropathy-with-subcutaneous-igg
#2
Melvin Berger, Thomas Harbo, David R Cornblath, Orell Mielke
Subcutaneous IgG (SCIG) administration may be preferred over the intravenous route (IVIG) in chronic inflammatory demyelinating polyneuropathy (CIDP) because it minimizes 'end of cycle' treatment-related fluctuations, reduces systemic adverse effects, improves convenience/quality of life and potentially lowers overall costs. Early reports of the use of highly concentrated SCIG preparations suggested they were effective and well-tolerated in chronic inflammatory demyelinating polyneuropathy. This was confirmed in the Polyneuropathy and Treatment with Hizentra® study of 172 subjects randomized to receive maintenance therapy with placebo or one of two doses of IgPro20 (20% IgG stabilized with L-Proline) for 6 months...
May 16, 2018: Immunotherapy
https://www.readbyqxmd.com/read/29752233/electro-physiology-of-coupling-model-and-its-impact-on-naja-kaouthia-venom-treated-sciatic-nerves-of-toad
#3
H K Das, P P Sahu
Demyelination in peripheral nerves causes dysfunction of slowing down and stoppage of nerve impulses causing many neurological diseases, such as chronic inflammatory demyelinating polyneuropathy, Guillain-Barre syndrome, etc. This paper aims to develop a recovery model having interaction of a demyelinated nerve with a normal myelinated nerve. We validated the model by coupling between peripheral nerve of toad (demyelinated with Naja kaouthia venom) and a normal nerve of toad. An increase in both nerve conduction velocity as well as compound action potential amplitude is observed in the repetition of the experiments indicating gradual recovery of the patients...
May 2018: IEEE Transactions on Neural Systems and Rehabilitation Engineering
https://www.readbyqxmd.com/read/29742800/reductions-in-muscle-quality-and-quantity-in-cidp-patients-assessed-by-magnetic-resonance-imaging
#4
Kevin J Gilmore, Timothy J Doherty, Kurt Kimpinski, Charles L Rice
INTRODUCTION: Weakness in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) may be caused by decreases in muscle quantity and quality, but these have not been explored. METHODS: Twelve patients with CIDP (mean 61 years) and ten age- matched (mean 59 years) control subjects were assessed for ankle dorsiflexion strength, and two different MRI scans (T1 and T2) of leg musculature. RESULTS: Isometric strength was lower in CIDP patients by 36% compared with controls...
May 9, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29700616/chronic-inflammatory-demyelinating-polyneuropathy-evaluation-of-the-vestibular-system-with-cervical-and-ocular-vestibular-evoked-myogenic-potentials
#5
Giuseppe Magliulo, Giannicola Iannella, Alessandra Manno, Laura Libonati, Emanuela Onesti, Annarita Vestri, Danilo Alunni Fegatelli, Diletta Angeletti, Annalisa Pace, Giampiero Gulotta, Silvia Gagliardi, Maurizio Inghilleri
PURPOSE: To investigate the possibility of vestibular damage in a group of patients suffering from chronic inflammatory demyelinating polyneuropathy (CIDP) using a diagnostic protocol including the caloric test, C-VEMPs and O-VEMPs. METHODS: Twenty patients suffering from CIDP (mean age 58.5 years, range 33-80 years; 4 women and 16 men) were investigated. To assess any eventual audio-vestibular involvement, all patients of the study underwent pure tone audiometry, Fitzgerald-Hallpike caloric vestibular test, C-VEMPs and O-VEMPs...
April 27, 2018: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/29670818/fingolimod-promotes-blood-nerve-barrier-properties-in-vitro
#6
Hideaki Nishihara, Toshihiko Maeda, Yasuteru Sano, Maho Ueno, Nana Okamoto, Yukio Takeshita, Fumitaka Shimizu, Michiaki Koga, Takashi Kanda
Objective: The main effect of fingolimod is thought to be functional antagonism of lymphocytic S1P1 receptors and the prevention of lymphocyte egress from lymphoid tissues, thereby reducing lymphocyte infiltration into the nervous system. However, a growing number of reports suggest that fingolimod also has a direct effect on several cell types in the nervous system. Although we previously reported that fingolimod enhances blood-brain barrier (BBB) functions, there have been no investigations regarding the blood-nerve barrier (BNB)...
April 2018: Brain and Behavior
https://www.readbyqxmd.com/read/29627036/nocebo-in-chronic-inflammatory-demyelinating-polyneuropathy-a-systematic-review-and-meta-analysis-of-placebo-controlled-clinical-trials
#7
Panagiotis Zis, Marios Hadjivassiliou, Ptolemaios G Sarrigiannis, Thomas M Jenkins, Dimos-Dimitrios Mitsikostas
INTRODUCTION: Nocebo is very prevalent among neurological disorders, resulting in low adherence and treatment outcome. We sought to examine the adverse events (AE) following placebo administration in placebo-controlled randomized clinical trials (RCTs) for chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: After a systematic literature search for RCTs for CIDP pharmacotherapy treatments, we assessed the number of AE in the placebo groups and the number discontinuations because of placebo intolerance...
May 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29618748/fingolimod-therapy-is-not-effective-in-a-mouse-model-of-spontaneous-autoimmune-peripheral-polyneuropathy
#8
Petra Huehnchen, Wolfgang Boehmerle, Matthias Endres
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder, which causes progressive sensory and motor deficits and often results in severe disability. Knockout of the co-stimulatory protein CD86 in mice of the non-obese diabetic background (NoD.129S4-Cd86tm1Shr /JbsJ) results in the development of a spontaneous autoimmune peripheral polyneuropathy (SAPP). We used this previously described transgenic model to study the effects of the sphingosine-1-phosphate receptor agonist fingolimod on SAPP symptoms, functional and electrophysiological characteristics...
April 4, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29615965/neurofascin-and-compact-myelin-antigen-specific-t-cell-response-pattern-in-chronic-inflammatory-demyelinating-polyneuropathy-subtypes
#9
Jan-Markus Diederich, Maximilian Staudt, Christian Meisel, Katrin Hahn, Edgar Meinl, Andreas Meisel, Juliane Klehmet
Objective: The objective of this study is to investigate whether chronic inflammatory demyelinating polyneuropathy (CIDP) and its subtypes differ in their type 1 T-helper (TH1) cell response against nodal/paranodal neurofascin (NF186, NF155) as well as myelin protein zero (P0 180-199) and myelin basic protein (MBP 82-100). Methods: Interferon-gamma (IFN-γ) enzyme-linked immunospot assay was used to detect antigen-specific T cell responses in 48 patients suffering typical CIDP ( n  = 18), distal acquired demyelinating polyneuropathy ( n  = 8), multifocal acquired demyelinating sensory and motor polyneuropathy (MADSAM; n  = 9), and sensory CIDP ( n  = 13) compared to other non-immune polyneuropathy (ON; n  = 19) and healthy controls ( n  = 9)...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29615658/systemic-igf-1-gene-delivery-by-raav9-improves-spontaneous-autoimmune-peripheral-polyneuropathy-sapp
#10
Tong Gao, Nataliia Bogdanova, Sameera Ghauri, Gang Zhang, Jianxin Lin, Kazim Sheikh
Spontaneous autoimmune peripheral polyneuropathy (SAPP) is a mouse model of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in non-obese diabetic (NOD) mice null for costimulatory molecule, B7-2 gene (B7-2-/- ). SAPP is a chronic progressive and multifocal inflammatory and demyelinating polyneuropathy of spontaneous onset with secondary axonal degeneration. Insulin-like growth factor 1(IGF-1) is a pleiotropic factor with neuroprotective, regenerative, and anti-inflammatory effects with extensive experience in its preclinical and clinical use...
April 3, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29610592/predominant-upper-limb-chronic-demyelinating-polyneuropathy-associated-with-hbv-infection
#11
Ioan-Cristian Lupescu, Adriana Octaviana Dulamea
Chronic inflammatory demyelinating polyneuropathy is an acquired, presumably immune-mediated peripheral neuropathy, characterized by symmetric sensory-motor involvement. Although most often idiopathic, it has been described in association with several disorders, sometimes improving under treatment. We present the case of a 57-year-old male who was admitted to hospital for paresthesias and muscle weakness affecting both upper limbs, initially only the hands, but with worsening and ascending progression during the last three years...
December 2017: Mædica
https://www.readbyqxmd.com/read/29603827/differences-between-acute-onset-chronic-inflammatory-demyelinating-polyneuropathy-a-cidp-and-acute-inflammatory-demyelinating-polyneuropathy-aidp-in-adult-patients
#12
Lucas Alessandro, José M Pastor Rueda, Miguel Wilken, Luis A Querol Gutiérrez, Mariano Marrodán, Julián N Acosta, Alberto Rivero, Fabio Barroso, Mauricio F Farez
Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and Acute-onset Chronic Inflammatory Demyelinating Polyneuropathy (A-CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A-CIDP diagnosed at our institution between January-2006 and July-2017 were retrospectively reviewed...
March 30, 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29603302/does-grip-strength-reflect-isokinetic-muscle-strength-in-lower-limbs-in-patients-with-chronic-inflammatory-demyelinating-polyneuropathy
#13
Kirsten L Knak, Linda K Andersen, Ingelise Christiansen, Lars K Markvardsen
INTRODUCTION: Grip strength (GS) is a common measure of general muscle strength in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). However, it is important to investigate the correlation and responsiveness of GS compared with isokinetic muscle strength (IKS) and function of the lower limbs. METHODS: Seventy patients with CIDP were evaluated with GS, IKS, and functional measures of the lower limbs. Reevaluation was performed after 2 and 10/12 weeks...
March 30, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29582526/whole-exome-sequencing-reveals-a-novel-missense-mutation-in-the-mars-gene-related-to-a-rare-charcot-marie-tooth-neuropathy-type-2u
#14
Lena Sagi-Dain, Lilach Shemer, Nathanel Zelnik, Yusri Zoabi, Orit Sadeh, Vardit Adir, Aharon Schif, Amir Peleg
BACKGROUND: Charcot-Marie-Tooth (CMT) is a heterogeneous group of progressive disorders, characterized by chronic motor and sensory polyneuropathy. This hereditary disorder is related to numerous genes and varying inheritance patterns. Thus, many patients do not reach a final genetic diagnosis. PATIENT: We describe a 13-years old girl presenting with progressive bilateral leg weakness and gait instability. Extensive laboratory studies and spinal Magnetic resonance imaging scan were normal...
March 26, 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29573033/individualized-immunoglobulin-therapy-in-chronic-immune-mediated-peripheral-neuropathies
#15
REVIEW
Jeffrey A Allen, Melvin Berger, Luis Querol, Krista Kuitwaard, Robert D Hadden
Despite the well-recognized importance of immunoglobulin therapy individualization during the treatment of chronic inflammatory demyelinating polyneuropathy (CIDP), the pathway to best achieve optimization is unknown. There are many pharmacokinetic and immunobiologic variables that can potentially influence the appropriateness of any individual therapy. Although identification of specific autoantibodies and their targets has only been accomplished in a minority of patients with CIDP, already the diagnostic and treatment implications of specific autoantibody detection are being realized...
March 23, 2018: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29568686/transthyretin-familial-amyloid-polyneuropathy-ttr-fap-parameters-for-early-diagnosis
#16
Fabiola Escolano-Lozano, Ana Paula Barreiros, Frank Birklein, Christian Geber
Background: Familial transthyretin amyloidosis is a life-threatening disease presenting with sensorimotor and autonomic polyneuropathy. Delayed diagnosis has a detrimental effect on treatment and prognosis. To facilitate diagnosis, we analyzed data patterns of patients with transthyretin familial amyloid polyneuropathy (TTR-FAP) and compared them to polyneuropathies of different etiology for clinical and electrophysiological discriminators. Methods: Twenty-four patients with TTR-FAP and 48 patients with diabetic polyneuropathy (dPNP) were investigated (neurological impairment score NIS; neurological disability score NDS) in a cross-sectional design...
January 2018: Brain and Behavior
https://www.readbyqxmd.com/read/29552093/high-resolution-nerve-ultrasound-and-magnetic-resonance-neurography-as-complementary-neuroimaging-tools-for-chronic-inflammatory-demyelinating-polyneuropathy
#17
Kalliopi Pitarokoili, Moritz Kronlage, Philip Bäumer, Daniel Schwarz, Ralf Gold, Martin Bendszus, Min-Suk Yoon
Background: We present a clinical, electrophysiological, sonographical and magnetic resonance neurography (MRN) study examining the complementary role of two neuroimaging methods of the peripheral nervous system for patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Furthermore, we explore the significance of cross-sectional area (CSA) increase through correlations with MRN markers of nerve integrity. Methods: A total of 108 nerve segments on the median, ulnar, radial, tibial and fibular nerve, as well as the lumbar and cervical plexus of 18 CIDP patients were examined with high-resonance nerve ultrasound (HRUS) and MRN additionally to the nerve conduction studies...
2018: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/29544239/-transthyretin-familial-amyloid-polyneuropathy-disease-profile-of-a-multisystem-disorder
#18
Christoph Niemietz, Christoph Röcken, Matthias Schilling, Jörg Stypmann, Constantin E Uhlig, Hartmut H-J Schmidt
Transthyretin-related Familial Amyloid Polyneuropathy (ATTR Amyloidosis, former FAP, here called TTR-FAP) is a rare, progressive autosomal dominant inherited amyloid disease ending fatal within 5 - 15 years after final diagnosis. TTR-FAP is caused by mutations of transthyretin (TTR), which forms amyloid fibrils affecting peripheral and autonomic nerves, the heart and other organs. Due to the phenotypic heterogeneity and partly not specific enough clinical symptoms, diagnosis of TTR-FAP can be complicated...
March 2018: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29542024/guillain-barr%C3%A3-syndrome-rare-extra-intestinal-manifestation-of-hepatitis-b
#19
Amey Dilip Sonavane, Sanjiv Saigal, Abhishek Kathuria, Narendra S Choudhary, Neeraj Saraf
Extrahepatic syndromes are uncommon manifestations of acute and chronic hepatitis B. The pathogenesis likely involves an aberrant immunologic response to extrahepatic viral proteins. Antiviral therapy reduces the availability of these viral protein antigens and thus halts immune activation. Approximately 1% of all cases of acute inflammatory demyelinating polyneuropathy are associated with hepatitis B. Guillain-Barre syndrome (GBS) is a remarkably clinically diverse disorder with distinctive variants characterised by an immune-mediated attack to components of the peripheral nervous system...
March 14, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29541543/challenges-in-the-diagnosis-of-chronic-inflammatory-demyelinating-polyneuropathy
#20
Jeffrey A Allen, Kenneth C Gorson, Deborah Gelinas
Introduction: We explored adherence to the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines for the diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) by reviewing data from a specialty pharmacy database. Materials and Methods: Clinical and electrophysiologic data were reviewed for 65 consecutive patients treated with intravenous immunoglobulin (IVIG) for CIDP. Three neuromuscular neurologists independently classified cases according to EFNS/PNS criteria as (1) fulfilling CIDP criteria; (2) non-CIDP (neither clinical nor electrophysiologic criteria met); or (3) unknown (insufficient information)...
March 2018: Brain and Behavior
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