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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/29761297/severe-neurologic-complications-of-immune-checkpoint-inhibitors-a-single-center-review
#3
Sarah Mancone, Thomas Lycan, Tamjeed Ahmed, Umit Topaloglu, Andrew Dothard, William J Petty, Roy E Strowd
BACKGROUND: Immune checkpoint inhibitors (ICIs) are a promising class of anticancer drugs associated with immune-related adverse events (IRAEs). In registration studies of selected cancer populations, neurologic IRAEs were rare. Post-marketing experience describing their prevalence in clinical practice continues to be reported. METHODS: A retrospective cohort of patients treated at our institution with ICIs from 2005 to 2017 was identified. Patients with new neurologic ICD codes documented during or after ICI treatment were enrolled...
May 14, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29759074/dengue-fever-complicated-with-guillain-barr%C3%A3-syndrome-a-case-report-and-review-of-the-literature
#4
Chamara Dalugama, John Shelton, Mahendra Ekanayake, Indika Bandara Gawarammana
BACKGROUND: Dengue is an arboviral infection classically presenting with fever, arthralgia, headache, and rashes. It is hyperendemic in Sri Lanka and has a major impact on health. Neurological complications of dengue fever are rare but have been reported in the literature. CASE PRESENTATION: A 60-year-old Sri Lankan man presented with a history of fever, arthralgia, and generalized malaise of 2 days duration. A diagnosis of dengue was confirmed with leukopenia, thrombocytopenia, and positive NS1 antigen done on day 2 without evidence of hemoconcentration...
May 15, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29752233/electro-physiology-of-coupling-model-and-its-impact-on-naja-kaouthia-venom-treated-sciatic-nerves-of-toad
#5
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
#6
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
#7
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/29681203/the-immunotherapy-of-guillain-barr%C3%A3-syndrome
#8
Paula Restrepo-Jiménez, Yhojan Rodríguez, Paulina González, Christopher Chang, M Eric Gershwin, Juan-Manuel Anaya
Guillain-Barré syndrome is the most common cause of acute flaccid paralysis worldwide. Microorganisms such as Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, Haemophilus influenzae and Zika virus have been linked to the disease. The most common clinical variants are acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy. Plasma exchange and intravenous immunoglobulins are the standard therapy for the disease. Areas covered: research to elucidate the pathophysiology of Guillain-Barré syndrome has led to the development of drugs directed towards new potential therapeutic targets...
May 8, 2018: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/29670818/fingolimod-promotes-blood-nerve-barrier-properties-in-vitro
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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/29604982/hiv-neuropathology
#15
Susan Morgello
Primary human immunodeficiency virus (HIV) neuropathologies can affect all levels of the neuraxis and occur in all stages of natural history disease. Some, like HIV encephalitis, HIV myelitis, and diffuse infiltrative lymphocytosis of peripheral nerve, reflect productive infection of the nervous system; others, like vacuolar myelopathy, distal symmetric polyneuropathy, and central and peripheral nervous system demyelination, are not clearly related to regional viral replication, and reflect more complex cascades of dysregulated host immunity and metabolic dysfunction...
2018: Handbook of Clinical Neurology
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
#16
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
#17
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/29594985/zika-virus-outbreak-in-new-caledonia-and-guillain-barr%C3%A3-syndrome-a-case-control-study
#18
Olivier Simon, Blandine Acket, Carole Forfait, Dominique Girault, Ann-Claire Gourinat, Pauline Millon, Maguy Daures, Jessica Vanhomwegen, Segolene Billot, Antoine Biron, Damien Hoinard, Elodie Descloux, David Guyon, Jean Claude Manuguerra, Sylvie Laumond, Nicolas Molko, Myrielle Dupont-Rouzeyrol
Zika virus (ZIKV) infection has been associated with neurologic disorders including Guillain-Barré syndrome (GBS). In New Caledonia during the ZIKV outbreak (2014-2015), case-control and retrospective studies have been performed to assess the link between ZIKV and GBS. Among the 15 cases included, 33% had evidence of a recent ZIKV infection compared to only 3.3% in the 30 controls involved. All patients were Melanesian, had facial diplegia and similar neurophysiological pattern consistent with acute inflammatory demyelinating polyneuropathy, and recovered well...
March 28, 2018: Journal of Neurovirology
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
#19
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/29582519/peripheral-neuropathy-in-tangier-disease-a-literature-review-and-assessment
#20
Metin Mercan, Vildan Yayla, Serdar Altinay, Serhat Seyhan
Tangier disease (OMIM#205400) is a rare cause of inherited metabolic neuropathies characterized by marked deficiency of high-density lipoproteins and accumulation of cholesterol esters in various tissue resulting from reverse cholesterol transport deficiency. We report a case of a patient with Tangier disease with multifocal demyelinating neuropathy with conduction block who presents with winging scapula, tongue and asymmetric extremity weakness. We also present a review of all studies published from 1960 to 2017 regarding peripheral neuropathy in Tangier disease...
March 26, 2018: Journal of the Peripheral Nervous System: JPNS
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