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https://www.readbyqxmd.com/read/28108521/nodopathy-chronic-inflammatory-demyelinating-polyneuropathy-with-anti-neurofascin-155-antibodies
#1
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/28108440/autoimmune-hepatitis-and-eosinophilic-granulomatosis-with-polyangiitis-a-rare-association
#2
Saroj Lohani, Salik Nazir, Niranjan Tachamo, Pavani Pagolu
We describe a case of 40-year-old woman who presented to our hospital with symptoms of non-radiating epigastric pain for 4-5 days associated with nausea and vomiting. Her history was significant for asthma with recurrent exacerbations, polyneuropathy and recurrent sinus infections. Liver function tests revealed cholestasis. Antinuclear antibody and antismooth muscle cell antibody were positive. Liver biopsy revealed active chronic hepatitis with cholestasis. A diagnosis of autoimmune hepatitis was made. All other causes of cholestatic jaundice were ruled out...
January 20, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28107689/development-and-validation-of-a-liquid-chromatography-tandem-mass-spectrometry-method-for-the-assay-of-tafamidis-in-rat-plasma-application-to-a-pharmacokinetic-study-in-rats
#3
Hun-Chan Hyun, Jong-Woo Jeong, Hye-Rim Kim, Ji-Hoon Oh, Jong-Hwa Lee, Sungwook Choi, Yeon-Soo Kim, Tae-Sung Koo
Tafamidis is a first-in-class inhibitor of transthyretin amyloid fibril formation. It has been available in Argentina, Japan, and Mexico for the treatment of transthyretin amyloidosis in adult patients with early-stage symptomatic polyneuropathy. In this study, a rapid and sensitive liquid chromatography-tandem mass spectrometry method was developed and validated for the assay of tafamidis in rat plasma. The method was also assessed for its applicability to pharmacokinetic studies in rats. Tafamidis was extracted from rat plasma by the liquid-liquid extraction method using hydrochloric acid and ethyl acetate...
January 10, 2017: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/28105520/type-1-diabetic-patients-with-peripheral-neuropathy-have-pan-enteric-prolongation-of-gastrointestinal-transit-times-and-an-altered-caecal-ph-profile
#4
Adam D Farmer, Anne Grave Pedersen, Birgitte Brock, Poul Erik Jakobsen, Jesper Karmisholt, Sahar D Mohammed, S Mark Scott, Asbjørn Mohr Drewes, Christina Brock
AIMS/HYPOTHESIS: We hypothesised that type 1 diabetic patients with established diabetic sensorimotor polyneuropathy (DSPN) would have segmental and/or pan-enteric dysmotility in comparison to healthy age-matched controls. We aimed to investigate the co-relationships between gastrointestinal function, degree of DSPN and clinical symptoms. METHODS: An observational comparison was made between 48 patients with DSPN (39 men, mean age 50 years, range 29-71 years), representing the baseline data of an ongoing clinical trial (representing a secondary analysis of baseline data collected from an ongoing double-blind randomised controlled trial investigating the neuroprotective effects of liraglutide) and 41 healthy participants (16 men, mean age 49 years, range 30-78) who underwent a standardised wireless motility capsule test to assess gastrointestinal transit...
January 20, 2017: Diabetologia
https://www.readbyqxmd.com/read/28100909/impact-of-pretransplant-induction-therapy-on-autologous-stem-cell-transplantation-for-patients-with-newly-diagnosed-poems-syndrome
#5
J Li, M-H Duan, C Wang, X-F Huang, W Zhang, X-X Cao, T-N Zhu, J-L Zhuang, B Han, H Cai, H-C Cai, D-B Zhou
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, Monoclonal gammopathy and skin changes) is a rare plasma cell dyscrasia sometimes treated with a haematopoietic cell autotransplant. We analyzed data from 138 subjects with newly-diagnosed POEMS syndrome receiving a autotransplant at our centre. Thirty-two subjects with severe end-organ dysfunction ineligible for immediate autotransplant received pretransplant therapy which made a subsequent autotransplant feasible. Pretransplant therapy resulted in vascular endothelial growth factor (VEGF) remissions in 15 (47%)...
January 19, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28091345/propofol-as-a-risk-factor-for-icu-acquired-weakness-in-septic-patients-with-acute-respiratory-failure
#6
Peter A Abdelmalik, Goran Rakocevic
BACKGROUND: Critical illness polyneuropathy (CIN) and critical illness myopathy (CIM), together "ICU-Acquired weakness (ICUAW)," occur frequently in septic patients. One of the proposed mechanisms for ICUAW includes prolonged inactivation of sodium channels. Propofol, used commonly in patients with acute respiratory failure (ARF), primarily acts via enhancement of GABAergic transmission but may also increase sodium channel inactivation, suggesting a potential interaction. METHODS: Electronic medical records and EMG reports of patients with ICUAW and a diagnosis of either sepsis, septicaemia, severe sepsis, or septic shock, concurrent with a diagnosis of acute respiratory failure (ARF), were retrospectively analyzed in a single center university hospital...
January 16, 2017: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/28090046/an-isolated-case-of-late-onset-amyloidogenic-transthyretin-type-familial-amyloid-polyneuropathy-associated-with-a-mutant-transthyretin-substituting-methionine-for-valine-at-position-30-showing-latent-progressive-cardiac-involvement-confirmed-by-serial-annual
#7
Chikako Sato, Tomofumi Takaya, Shumpei Mori, Kohei Hasegawa, Fumitaka Soga, Hidekazu Tanaka, Yoshiaki Watanabe, Tatsuya Nishii, Atsushi K Kono, Yukiko Morinaga, Hatsue Ishibashi-Ueda, Ken-Ichi Hirata
Late-onset amyloidogenic transthyretin (ATTR) type familial amyloid polyneuropathy (FAP) shows features distinct from those of early-onset hereditary ATTR type FAP. We herein describe an asymptomatic 68-year-old man with late-onset ATTR type FAP whose serial annual electrocardiograms demonstrated progressive left bundle branch block. Latent but severe cardiac involvement seems to be one feature of late-onset ATTR type FAP, similar to senile systemic amyloidosis (SSA). Early differential diagnosis of late-onset ATTR type FAP from SSA is important because, currently, only the former has new therapeutic options available in Japan...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28073817/paranodal-dissection-in-chronic-inflammatory-demyelinating-polyneuropathy-with-antineurofascin-155-and-anticontactin-1-antibodies
#8
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
#9
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/28069916/flight-lieutenant-peach-s-observations-on-burning-feet-syndrome-in-far-eastern-prisoners-of-war-1942-45
#10
N T Roocroft, E Mayhew, M Parkes, W A Frankland, G V Gill, D Bouhassira, A S C Rice
INTRODUCTION: 'Burning Feet Syndrome' affected up to one third of Far Eastern Prisoners of War in World War 2. Recently discovered medical records, produced by RAF Medical Officer Nowell Peach whilst in captivity, are the first to detail neurological examinations of patients with this condition. METHODS: The 54 sets of case notes produced at the time were analysed using modern diagnostic criteria to determine if the syndrome can be retrospectively classed as neuropathic pain...
November 15, 2016: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28063957/initial-development-and-validation-of-a-patient-reported-symptom-survey-for-small-fiber-polyneuropathy
#11
Roi Treister, Mette Lodahl, Magdalena Lang, Shelley S Tworoger, Shlomo Sawilowsky, Anne Louise Oaklander
: Small-fiber polyneuropathy SFPN affects unmyelinated and thinly myelinated peripheral axons. Several questionnaires have been developed to assess polyneuropathy from diabeties or chemotherapy, but none for SFPN from other or unknown causes. A comprehensive survey could help clinicians diagnose and assess treatment responses, define prevalence natural history and cures, and identify research subjects. Thus, we developed the one-page Small-fiber Symptom Survey (SSS), using input from patients and 21 medical/scientific experts...
January 4, 2017: Journal of Pain: Official Journal of the American Pain Society
https://www.readbyqxmd.com/read/28063170/assessing-mnis-7ionis-and-international-neurologists-proficiency-in-an-fap-trial
#12
Peter J Dyck, John C Kincaid, P James B Dyck, Vinay Chaudhry, Namita A Goyal, Christina Alves, Hayet Salhi, Janice F Wiesman, Celine Labeyrie, Jessica Robinson-Papp, Márcio Cardoso, Matilde Laura, Katherine Ruzhansky, Andrea Cortese, Thomas H Brannagan, Julie Khoury, Sami Khella, Márcia Waddington-Cruz, João Ferreira, Annabel K Wang, Marcus V Pinto, Samar S Ayache, Merrill D Benson, John L Berk, Teresa Coelho, Michael Polydefkis, Peter Gorevic, David H Adams, Violaine Plante-Bordeneuve, Carol Whelan, Giampaolo Merlini, Stephen Heitner, Brian M Drachman, Isabel Conceição, Christopher J Klein, Morie A Gertz, Elizabeth J Ackermann, Steven G Hughes, Michelle L Mauermann, Rito Bergemann, Karen A Lodermeier, Jenny L Davies, Rickey E Carter, William J Litchy
INTRODUCTION: Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7Ionis ), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. METHODS: We assessed: 1) Proficiency of grading neurologic signs and correlation with neurophysiologic tests, and 2) clinometric performance of mNIS+7Ionis and its subscores and correlation with disability and health scores...
January 7, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28057506/patient-perspectives-on-peripheral-neuropathic-pain-experience-within-the-community
#13
Devada Singh-Franco, Robin J Jacobs
Purpose of this cross-sectional study was to explore the relationship between neuropathic physical complaints (NPC) and quality-of-life (QoL) in community-dwelling patients with diabetes in Broward County, Florida. Adult patients were invited to complete a 10-minute paper questionnaire at a community hospital between October 2014-April 2016. Analysis of data from 124 participants (60 with distal symmetric polyneuropathy (DSPN) diagnosis versus 64 without DSPN diagnosis) with NPC, showed those with DSPN had a longer duration of diabetes, suffered a higher number of NPC, and had a lower QoL (all p≤0...
December 22, 2016: Diabetes & Metabolic Syndrome
https://www.readbyqxmd.com/read/28054000/a-comparative-study-of-cidp-in-a-cohort-of-hiv-infected-and-hiv-uninfected-patients
#14
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
https://www.readbyqxmd.com/read/28051995/in-vitro-modelling-of-familial-amyloidotic-polyneuropathy-allows-quantitative-detection-of-transthyretin-amyloid-fibril-like-structures-in-hepatic-derivatives-of-patient-specific-induced-pluripotent-stem-cells
#15
Jeannine Hoepfner, Mandy Kleinsorge, Oliver Papp, Susanne Alfken, Robin Heiringhoff, Andreas Pich, Vanessa Sauer, Andree Zibert, Gudrun Göhring, Hartmut Schmidt, Malte Sgodda, Tobias Cantz
The transthyretin protein is thermodynamically destabilised by mutations in the transthyretin gene, promoting the formation of amyloid fibrils in various tissues. Consequently, impaired autonomic organ function is observed in patients suffering from transthyretin-related Familial Amyloidotic Polyneuropathy (FAP). The influence of individual genetic backgrounds on fibril formation as a potential cause of genotype-phenotype variations needs to be investigated in order to ensure efficient patient-specific therapies...
January 4, 2017: Biological Chemistry
https://www.readbyqxmd.com/read/28051009/spinal-metastases-of-glioblastoma-multiforme-in-a-patient-with-polyneuropathy
#16
Leslaw W Zub, Marta Waliszewska-Prosól, Tomasz Szczepañski, Zdzislaw Woÿniak, Maria Ejma
No abstract text is available yet for this article.
July 2016: National Medical Journal of India
https://www.readbyqxmd.com/read/28049985/demyelinating-peripheral-neuropathy-due-to-renal-cell-carcinoma
#17
Kenya Nishioka, Motoki Fujimaki, Kazuaki Kanai, Yuta Ishiguro, Tomoko Nakazato, Ryota Tanaka, Kazumasa Yokoyama, Nobutaka Hattori
Renal cell carcinoma (RCC) patients who develop a paraneoplastic syndrome may present with neuromuscular disorders. We herein report the case of a 50-year-old man who suffered from progressive gait disturbance and muscle weakness. The results of a nerve conduction study fulfilled the criteria of chronic inflammatory demyelinating polyneuropathy. An abdominal CT scan detected RCC, the pathological diagnosis of which was clear cell type. After tumor resection and a single course of intravenous immunoglobulin therapy, the patient's symptoms drastically improved over the course of one year...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28042370/review-of-critical-illness-myopathy-and-neuropathy
#18
Starane Shepherd, Ayush Batra, David P Lerner
Critical illness myopathy (CIM) and neuropathy are underdiagnosed conditions within the intensive care setting and contribute to prolonged mechanical ventilation and ventilator wean failure and ultimately lead to significant morbidity and mortality. These conditions are often further subdivided into CIM, critical illness polyneuropathy (CIP), or the combination-critical illness polyneuromyopathy (CIPNM). In this review, we discuss the epidemiology and pathophysiology of CIM, CIP, and CIPNM, along with diagnostic considerations such as detailed clinical examination, electrophysiological studies, and histopathological review of muscle biopsy specimens...
January 2017: Neurohospitalist
https://www.readbyqxmd.com/read/28032668/might-genetics-play-a-role-in-understanding-and-treating-diabetic-polyneuropathy
#19
Vincenza Spallone
Despite the high prevalence and impact on quality of life, costs and survival, there are still unresolved issues regarding diabetic polyneuropathy (DPN): the lack of definite knowledge of its pathogenesis; the limited preventive action of glycaemic control in type 2 diabetes; and the unavailability of effective evidence-based disease-modifying treatment. How can genetics provide the tools to address these gaps? Ziegler et al for the GDS Group explore the novel hypothesis that genetic variability in transketolase (TKT) might contribute to susceptibility to DPN in patients with newly diagnosed type 1 and type 2 diabetes (well characterised for DPN)...
December 29, 2016: Diabetes/metabolism Research and Reviews
https://www.readbyqxmd.com/read/28030863/real-world-clinical-practice-of-intensified-chemotherapies-for-metastatic-pancreatic-cancer-results-from-a-pan-european-questionnaire-study
#20
Nha Le, Alessio Vinci, Marvin Schober, Sebastian Krug, Muhammad A Javed, Thomas Kohlmann, Malin Sund, Albrecht Neesse, Georg Beyer
INTRODUCTION: Recently, FOLFIRINOX and gemcitabine + nab-paclitaxel have been introduced as a novel intensified chemotherapy regimen for patients with metastasized pancreatic cancer. This study aims to analyze the real-world clinical practice with FOLFIRINOX and gemcitabine + nab-paclitaxel across Europe. METHODS: Invitations to participate in an anonymous web-based questionnaire were sent via e-mail to 5,420 doctors in 19 European countries through the network of national gastroenterological, oncological, surgical and pancreatic societies as well as the European Pancreatic Club...
2016: Digestion
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