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Demyelinating polyneuropathy

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https://www.readbyqxmd.com/read/28403522/structure-activity-relationship-studies-spr-affinity-characterization-and-conformational-analysisof-peptides-mimicking-the-hnk-1-carbohydrate-epitope
#1
Paolo Rovero, Matthaia Ieronymaki, Francesca Nuti, Diego Brancaccio, Giada Rossi, Feliciana Real-Fernández, Yihong Cao, Olivier Monasson, Maud Larregola, Elisa Peroni, Jacques Uziel, Giuseppina Sabatino, Ettore Novellino, Alfonso Carotenuto, Anna Maria Papini
The design of molecules mimicking biologically relevant glycans is a significant goal for understanding important biological processesand may lead to new therapeutic and diagnostic agents. We focused our attention on the trisaccharide human natural killer cell-1 (HNK-1), considered the antigenic determinant ofmyelin-associated glycoprotein and the target of clinically relevant auto-antibodies in autoimmune neurological disorders such as IgM monoclonal gammopathy and demyelinating polyneuropathy.We describe a structure-activity relationship study based on surface plasmon resonance binding affinities aiming at the optimization of a peptide mimicking the HNK-1 minimal epitope...
April 12, 2017: ChemMedChem
https://www.readbyqxmd.com/read/28397393/early-discrimination-of-sensorimotor-guillain-barr%C3%A3-syndrome-into-demyelinating-or-axonal-subtype-by-automated-nerve-excitability-testing
#2
So Young Pyun, Mi-Ri Kang, Joo Young Lee, Kim Jong Kuk, Seong-Il Oh, Jong Seok Bae
In the early stage of disease, differentiating acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor sensory axonal neuropathy (AMSAN) using only a conventional nerve conduction studies (NCS) may be difficult. We evaluated the differences in the motor axonal excitability properties of 16 cases of sensorimotor Guillain-Barré syndrome by nerve excitability testing (NET). The antiganglioside antibody assay and follow-up NCS resulted in 12 patients diagnosed as AIDP and 4 patients as AMSAN. Clinical and excitability parameters in each group were compared with those in 30 normal controls...
April 11, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28397326/diagnosis-and-management-of-neuropathies-associated-with-plasma-cell-dyscrasias
#3
REVIEW
Evan Rosenbaum, Douglas Marks, Shahzad Raza
Neuropathies associated with plasma cell dyscrasias are a major cause of morbidity for patients managed by medical oncologists. Because of similarities in clinical presentation and on nerve conduction studies, identifying the underlying disease leading to a paraproteinemic neuropathy can often be difficult. In addition, the degree of neurologic deficit does not strictly correlate with the extent of abnormalities on common clinical laboratory testing. Fortunately, with increasing understanding into the biologic mechanisms of underlying hematologic diseases, additional biomarkers have recently been developed, thus improving our diagnostic capacity...
April 10, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28355569/severity-of-demyelinating-and-axonal-neuropathy-mouse-models-is-modified-by-genes-affecting-structure-and-function-of-peripheral-nodes
#4
Kathryn H Morelli, Kevin L Seburn, David G Schroeder, Emily L Spaulding, Loiuse A Dionne, Gregory A Cox, Robert W Burgess
Charcot-Marie-Tooth (CMT) disease is a clinically and genetically heterogeneous group of inherited polyneuropathies. Mutations in 80 genetic loci can cause forms of CMT, resulting in demyelination and axonal dysfunction. The clinical presentation, including sensory deficits, distal muscle weakness, and atrophy, can vary greatly in severity and progression. Here, we used mouse models of CMT to demonstrate genetic interactions that result in a more severe neuropathy phenotype. The cell adhesion molecule Nrcam and the Na(+) channel Scn8a (NaV1...
March 28, 2017: Cell Reports
https://www.readbyqxmd.com/read/28345260/resistance-training-and-aerobic-training-improve-muscle-strength-and-aerobic-capacity-in-chronic-inflammatory-demyelinating-polyneuropathy
#5
Lars H Markvardsen, Kristian Overgaard, Karen Heje, Søren H Sindrup, Ingelise Christiansen, John Vissing, Henning Andersen
INTRODUCTION: We investigated the effects of aerobic and resistance exercise in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Eighteen CIDP patients treated with subcutaneous immunoglobulin performed 12 weeks of aerobic exercise and 12 weeks of resistance exercise after a run-in period of 12 weeks without exercise. Three times weekly the participants performed aerobic exercise on an ergometer bike or resistance exercise with unilateral training of knee and elbow flexion/extension...
March 27, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28336415/sncam-as-a-specific-marker-of-peripheral-demyelination
#6
Adam Niezgoda, Sławomir Michalak, Jacek Losy, Alicja Kalinowska-Łyszczarz, Wojciech Kozubski
Adhesion molecules are involved in nerve growth, synaptic plasticity and myelin formation and maintenance process. Neural cell adhesion molecule (CD56 or NCAM) seems to play a crucial role in all the above-mentioned events. Having found poly-sialylated NCAM increased re-expression on demyelinated axons within multiple sclerosis plaques we assessed soluble NCAM (sNCAM) in sera of patients with various types of peripheral nerve affections - demyelinating, axonal "inflammatory", axonal metabolic polyneuropathies and healthy controls...
March 21, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28332470/adult-onset-demyelinating-neuropathy-associated-with-fbln5-gene-mutation
#7
Si Cheng, He Lv, Wei Zhang, Zhaoxia Wang, Xin Shi, Wei Liang, Yun Yuan
Rare forms of autosomal-dominant Charcot-Marie-Tooth disease (AD-CMT) may be associated with mutations in Fibulin-5 (FBLN5) as AD-CMT is genetically heterogeneous. Here, we report the first pathological study of an Asian family. The proband was a 46-year-old man with slowly progressive distal numbness and weakness for 12 years. He had a history of diabetes mellitus for 12 years. His mother was 81 years old and had mild polyneuropathy. His 16-year-old daughter was asymptomatic. The nerve conduction velocities (NCVs) and compound muscular action potential (CMAP) amplitudes were moderately to severely reduced in the proband, and moderately reduced in his daughter and mother...
March 23, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28283677/guillain-barr%C3%A3-syndrome-presenting-with-sinus-node-dysfunction-and-refractory-shock
#8
Le Dung Ha, Farrukh Abbas, Mohan Rao
BACKGROUND Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy that is usually associated with preceding respiratory or gastrointestinal infection and has the hallmark manifestation of ascending flaccid paralysis. We report an atypical presentation of GBS. CASE REPORT A 76-year-old male presented with acute onset of diaphoresis and altered mental status. He subsequently developed severe bradycardia and refractory hypotension, which initially responded to dopamine infusion. A temporary pacemaker wire was placed to stabilize the heart rate but hypotension persisted...
March 11, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28283332/corrigendum-to-challenges-in-pediatric-chronic-inflammatory-demyelinating-polyneuropathy-neuromuscular-disorders-26-12-2016-817-824
#9
Göknur Haliloğlu, Deniz Yüksel, Cağri Mesut Temoçin, Haluk Topaloğlu
No abstract text is available yet for this article.
March 7, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28257341/decreased-axon-flare-reaction-to-electrical-stimulation-in-patients-with-chronic-demyelinating-inflammatory-polyneuropathy
#10
Panagiotis Kokotis, Martin Schmelz, Aikaterini E Papagianni, Thomas Zambelis, Nikos Karandreas
PURPOSE: In chronic inflammatory demyelinating polyradiculopathy (CIDP), the impairment of unmyelinated nerve fibers appears unexpected. The measurement of the electrically induced axon flare reflex is a clinical test to assess the peripheral C-nociceptor function. In this study, we compared the flare area in patients suffering from CIDP with healthy subjects. METHODS: We examined 18 patients fulfilling the criteria for CIDP (11 men, mean age 51.8 years, SD 15.1) and 18 age-matched adult healthy volunteers (control group) (11 men, mean age 51...
March 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28247959/therapeutic-plasma-exchange-in-chronic-dysimmune-peripheral-neuropathies-a-10-year-retrospective-study
#11
Philippe Codron, Maud Cousin, Jean-François Subra, Vivien Pautot, Franck Letournel, Christophe Verny, Julien Cassereau
INTRODUCTION: Therapeutic plasma exchange (TPE) can be proposed in the treatment of chronic dysimmune peripheral neuropathies (CDPN). Actual guidelines are however based on few studies, and indications and protocols still remain to be clarified. We conducted a 10-year retrospective study in order to assess the effectiveness and tolerance of TPE in CDPN. METHODS: All patients treated for CDPN with TPE from October 2006 to March 2016 in the university hospital of Angers were included...
March 1, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28221307/utility-of-neurological-imaging-in-sensory-variant-of-chronic-inflammatory-demyelinating-polyneuropathy-a-case-report
#12
Alexander G Gevorgyan, Akshay Shah, Said R Beydoun
No abstract text is available yet for this article.
March 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28215575/fibronectin-connecting-segment-1-peptide-inhibits-pathogenic-leukocyte-trafficking-and-inflammatory-demyelination-in-experimental-models-of-chronic-inflammatory-demyelinating-polyradiculoneuropathy
#13
Chaoling Dong, Kelsey M Greathouse, Rebecca L Beacham, Steven P Palladino, E Scott Helton, Eroboghene E Ubogu
The molecular determinants of pathogenic leukocyte migration across the blood-nerve barrier (BNB) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are unknown. Specific disease modifying therapies for CIDP are also lacking. Fibronectin connecting segment-1 (FNCS1), an alternatively spliced fibronectin variant expressed by microvascular endothelial cells at sites of inflammation in vitro and in situ, is a counterligand for leukocyte α4 integrin (also known as CD49d) implicated in pathogenic leukocyte trafficking in multiple sclerosis and inflammatory bowel disease...
June 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28194064/therapeutic-plasma-exchange-in-patients-with-neurologic-disorders-review-of-63-cases
#14
Anil Tombak, Mehmet Ali Uçar, Aydan Akdeniz, Arda Yilmaz, Hakan Kaleagası, Mehmet Ali Sungur, Eyup Naci Tiftik
Therapeutic plasma exchange (TPE) is a procedure that reduces circulating autoantibodies of the patients. TPE is commonly used in neurological disorders where autoimmunity plays a major role. We report our experience with regard to the indications, adverse events and outcomes of plasma exchange in neurological disorders. Sixty-three patients were included to this retrospective study. Median age was 48 years (range 1-85), there was a predominance of males. Neurological indications included Guillain-Barrè syndrome (n = 22), myasthenia gravis (n = 21), chronic inflammatory demyelinating polyneuropathy (n = 7), polymyositis (n = 3), multifocal motor neuropathy (n = 2), acute disseminated encephalomyelitis (n = 2), neuromyelitis optica (n = 2), multiple sclerosis (n = 2), limbic encephalitis (n = 1) and transverse myelitis (n = 1)...
March 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28163843/outcome-of-guillain-barr%C3%A3-syndrome-in-children-a-prospective-cohort-study-in-a-tertiary-hospital-in-upper-egypt
#15
Abdelrahim Abdrabou Sadek, Ashraf Abou-Taleb, Wafaa Ahmed Ali
INTRODUCTION: Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, and defined as an acute inflammatory polyneuropathy. The objective of this study was to assess the clinico-laboratory profile, and outcome of Guillain-Barré syndrome in children at Sohag University Hospital. METHODS: This prospective cohort observational study was conducted in 2014-2015. The included children were subjected to through medical history and detailed systemic and neurological examination...
December 2016: Electronic Physician
https://www.readbyqxmd.com/read/28131554/-hereditary-neuropathy-with-liability-to-pressure-palsies-in-childhood-report-of-three-cases
#16
C Bar, F Villéga, C Espil, M Husson, J-M Pedespan, M-F Rouanet
Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant neuropathy. It is characterized by recurrent sensory and motor nerve palsies, usually precipitated by minor trauma or compression. Even though rare in childhood, this disorder is probably underdiagnosed given its wide spectrum of clinical symptoms. We review three separate cases of HNPP diagnosed in children with various phenotypes: fluctuating and distal paresthesias disrupting learning at school, cramps related to intensive piano practice, and discrete muscle weakness with no functional complaint...
March 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28108521/nodopathy-chronic-inflammatory-demyelinating-polyneuropathy-with-anti-neurofascin-155-antibodies
#17
EDITORIAL
Satoshi Kuwabara, Sonoko Misawa, Masahiro Mori
No abstract text is available yet for this article.
January 20, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28073817/paranodal-dissection-in-chronic-inflammatory-demyelinating-polyneuropathy-with-antineurofascin-155-and-anticontactin-1-antibodies
#18
Haruki Koike, Masato Kadoya, Ken-Ichi Kaida, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Daisuke Kato, Hidenori Ogata, Ryo Yamasaki, Noriyuki Matsukawa, Jun-Ichi Kira, Masahisa Katsuno, Gen Sobue
OBJECTIVE: To investigate the morphological features of chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies directed against paranodal junctional molecules, particularly focusing on the fine structures of the paranodes. METHODS: We assessed sural nerve biopsy specimens obtained from 9 patients with CIDP with antineurofascin-155 antibodies and 1 patient with anticontactin-1 antibodies. 13 patients with CIDP without these antibodies were also examined to compare pathological findings...
January 10, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28073143/demyelinating-polyneuropathy-in-a-case-of-anti-lgi1-encephalitis
#19
Gemma Tumminelli, Carla Battisti, Chiara Cioni, Andrea Mignarri, Pasquale Annunziata, And Antonio Federico
No abstract text is available yet for this article.
January 10, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28054000/a-comparative-study-of-cidp-in-a-cohort-of-hiv-infected-and-hiv-uninfected-patients
#20
Kaminie Moodley, Pierre L A Bill, Vinod Bhagu Patel
OBJECTIVE: To investigate differences in clinical presentation, electrodiagnostic measures, CSF changes, and treatment outcome measures in HIV-infected and HIV-uninfected patients with chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: A retrospective analysis of medical records of all patients meeting the European Federation of Neurology diagnostic criteria for idiopathic CIDP was performed in 2 neuromuscular units in Kwa-Zulu Natal between 2003 and 2015...
March 2017: Neurology® Neuroimmunology & Neuroinflammation
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