keyword
MENU ▼
Read by QxMD icon Read
search

polyneuropathy

keyword
https://www.readbyqxmd.com/read/27932436/multifactorial-pathological-hip-subluxation-in-neurofibromatosis-type-1-nf1-due-to-intra-articular-plexiform-neurofibroma-lumbar-radiculopathy-and-neurofibromatous-polyneuropathy
#1
Waqar Waheed, Diego F Diego F Lemos, Nelms Nathaniel Nelms, Rup Tandan
Neurofibromatosis type-1 (NF1) is a multisystem disorder with very rare descriptions of hip instability. We report a case of a 37-year-old man with known NF1 and childhood-onset of left foot drop, who developed persistent left hip pain following a minor trauma. Physical examination revealed left-sided mild foot drop, hip abductor weakness, bilateral sensory loss in feet and an antalgic gait. Work-up revealed anterolateral subluxation of the left femoral head along with left hip plexiform neurofibroma (PN), dysplastic and degenerative changes, neurofibromatous neuropathy and chronic left L5 radiculopathy...
December 8, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27932182/recovery-after-critical-illness-polyneuropathy-in-a-patient-with-orthotopic-liver-transplantation-a-case-report
#2
J Watanabe, E Ito, M Hatano, T Tohyama, Y Okada, Y Takada
After liver transplantation, some patients show neuromuscular abnormalities. A 43-year-old man with liver cirrhosis due to hepatitis C virus underwent living-donor liver transplantation. He developed severe neuromuscular dysfunction after sepsis, and acute respiratory distress syndrome. After the inflammatory reaction gradually improved, we observed bilateral weakness of the extremities and foot drop. Electrophysiological studies indicated primary axonal degeneration of peripheral motor and sensory fibers without inflammation...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27931573/survival-predictors-in-liver-transplantation-time-varying-effect-of-red-blood-cell-transfusion
#3
C Real, D Sobreira Fernandes, P Sá Couto, F Correia de Barros, S Esteves, I Aragão, L Fonseca, J Aguiar, T Branco, Z Fernandes Moreira
BACKGROUND: Many attempts have been undertaken to better predict outcome after liver transplantation. The aim of this study was to identify the pre- and intraoperative variables that may influence the survival after liver transplantation, at a single institution. METHODS: Anesthetic records from 543 consecutive patients who underwent liver transplantation from June 2006 to June 2014 were reviewed in this retrospective study. Patients undergoing retransplantation were excluded from the analysis, as were patients with familial amyloid polyneuropathy...
December 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27931132/guillain-barr%C3%A3-syndrome-during-adalimumab-therapy-for-crohn%C3%A2-s-disease-coincidence-or-consequence
#4
Guilherme Grossi Lopes Cançado, Eduardo Garcia Vilela
We report the case of a 64-year-old patient diagnosed with extensive ileal Crohn´s disease who developed Guillain-Barré syndrome after starting biological therapy with adalimumab. Neurologic involvement associated with inflammatory bowel diseases is recognized as an extra-intestinal manifestation. After the breakthrough of antitumor necrosis factor alpha (anti-TNF-α) agents, an increasing number of cases of acute inflammatory demyelinating polyneuropathies have been reported; however, only one case has been described in a patient with Crohn´s disease...
December 8, 2016: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27930572/acute-onset-chronic-inflammatory-demyelinating-polyneuropathy-in-hantavirus-and-hepatitis-b-virus-coinfection-a-case-report
#5
Jong Youb Lim, Young-Ho Lim, Eun-Hi Choi
INTRODUCTION: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disorder with progressive weakness. Acute-onset CIDP resembles Guillain-Barre syndrome (GBS), a rapidly progressive disorder, and follows a chronic course. To our knowledge, no case of acute-onset CIDP in hantavirus and hepatitis B virus (HBV) coinfection has been reported previously. CLINICAL FINDINGS: We report a case of acute-onset CIDP that was initially diagnosed as GBS...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27927940/diagnostic-value-of-sonography-in-treatment-naive-chronic-inflammatory-neuropathies
#6
H Stephan Goedee, W Ludo van der Pol, Jan-Thies H van Asseldonk, Hessel Franssen, Nicolette C Notermans, Alexander J F E Vrancken, Michael A van Es, Stavros Nikolakopoulos, Leo H Visser, Leonard H van den Berg
OBJECTIVE: To determine the diagnostic value of high-resolution ultrasound (HRUS) for detection of chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis-Sumner syndrome (LSS), and multifocal motor neuropathy (MMN). METHODS: Between January 2013 and January 2015, we enrolled 75 consecutive treatment-naive patients with chronic inflammatory neuropathies and 70 disease controls. We performed extensive nerve conduction and standardized HRUS studies bilaterally of large arm and leg nerves and brachial plexus...
December 7, 2016: Neurology
https://www.readbyqxmd.com/read/27920716/acute-clinical-worsening-after-steroid-administration-in-cervical-myelitis-may-reveal-a-subdural-arteriovenous-fistula
#7
Silvia Rain, Jan Udding, Daniel Broere
Subdural arteriovenous fistula (SDAVF) is a rare condition characterized by clinical manifestations ranging from mild bilateral sensory deficits to quadriplegia. The diagnosis is often delayed due to unspecific neurological symptoms, initially diagnosed as polyneuropathy or myelopathy. The diagnosis can be delayed for as long as 1-15 years. The following report describes a cervical SDAVF case initially misdiagnosed as myelitis transversa and treated with intravenous steroids. A 56-year-old male presented with sensory deficits and mild leg and right arm weakness...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27919414/early-diagnosis-in-patients-with-transthyretin-familial-amyloid-polyneuropathy-a-comparative-study
#8
Manuel Raya-Cruz, Juan Buades-Reines, Cristina Gállego-Lezáun, Tomás Ripoll-Vera, Mercedes Usón-Martín, Eugenia Cisneros-Barroso
INTRODUCTION AND OBJECTIVE: Transthyretin-associated familial amyloid polyneuropathy (TTR-FAP) is a disease caused by the deposit of abnormal transthyretin on tissues, mainly nerves. Small nerve fibers are altered earlier during the course of the disease; hence, detection of their involvement may have serious consequences on the natural history of disease. METHODS: A cross-sectional, observational study, was carried out on symptomatic patients, involving the conduct of several tests for small nerve fibers: Vibration, Touch Pressure (TP) and Heat Pain (HP)...
December 2, 2016: Medicina Clínica
https://www.readbyqxmd.com/read/27918219/a-new-treatment-regimen-with-high-dose-and-fractioned-immunoglobulin-in-a-special-subgroup-of-severe-and-dependent-cidp-patients
#9
Rabab Debs, Pauline Reach, Corina Cret, Sophie Demeret, Samir Saheb, Thierry Maisonobe, Karine Viala
BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is treated with intravenous immunoglobulins (IVIg), corticosteroids or plasma exchange (PE). IVIg dosage is not universal and markers for treatment management are needed. METHODS: We report the response to high-dose and fractioned IVIg in a subgroup of definite CIDP patients, resistant to corticosteroids and PE, responders to IVIg but with an efficacy window <15 days. RESULTS: Four patients were included with similar predominantly clinical motor form and conduction abnormalities...
December 5, 2016: International Journal of Neuroscience
https://www.readbyqxmd.com/read/27916751/-autoantibodies-in-chronic-inflammatory-neuropathies
#10
Ryo Yamasaki
Autoantibodies in chronic demyelinating neuropathies have been explored for several years. Recently, the peptides in the nodes of Ranvier have been the focus of attention in finding targets of autoantibodies. Until now, the most popular autoantibodies have been contactin-1 and neurofascin-155 for chronic demyelinating polyradiculoneuropathy (CIDP), GM1-ganglioside for multifocal motor neuropathy, and myelin-associated glycoprotein for polyneuropathy associated with monoclonal gammopathy of unknown significance...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27904737/a-clinical-update-on-the-role-of-carfilzomib-in-the-treatment-of-relapsed-or-refractory-multiple-myeloma
#11
REVIEW
B Franken, N W C J van de Donk, J C Cloos, S Zweegman, H M Lokhorst
Even though the prognosis of patients with multiple myeloma is continuing to improve, all patients eventually develop relapsed refractory disease. Several novel therapeutics have been developed in the last few years including the second-generation proteasome inhibitor carfilzomib which has been approved for patients with relapsed and refractory multiple myeloma in the United States since 2012. Recently data from several phase III studies have become available showing the promising efficacy of carfilzomib in combination with lenalidomide, which led to the renewed approval of carfilzomib in combination with lenalidomide and dexamethasone for relapsed myeloma in 2015...
December 2016: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/27902997/chronic-inflammatory-demyelinating-polyradiculoneuropathy-cidp-clinical-features-diagnosis-and-current-treatment-strategies
#12
Jacques Reynolds, George Sachs, Kara Stavros
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder characterized by weakness and sensory deficits that can lead to significant neurological disability. The diagnosis is based on a combination of clinical examination findings, electrodiagnostic studies, and other supportive evidence. Recognizing CIDP and distinguishing it from other chronic polyneuropathies is important because many patients with CIDP are highly responsive to treatment with immunosuppressive or immunomodulatory therapies...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27898033/towards-clinical-application-of-neurotrophic-factors-to-the-auditory-nerve-assessment-of-safety-and-efficacy-by-a-systematic-review-of-neurotrophic-treatments-in-humans
#13
REVIEW
Aren Bezdjian, Véronique J C Kraaijenga, Dyan Ramekers, Huib Versnel, Hans G X M Thomeer, Sjaak F L Klis, Wilko Grolman
Animal studies have evidenced protection of the auditory nerve by exogenous neurotrophic factors. In order to assess clinical applicability of neurotrophic treatment of the auditory nerve, the safety and efficacy of neurotrophic therapies in various human disorders were systematically reviewed. Outcomes of our literature search included disorder, neurotrophic factor, administration route, therapeutic outcome, and adverse event. From 2103 articles retrieved, 20 randomized controlled trials including 3974 patients were selected...
November 26, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27894792/pyruvate-dehydrogenase-e1%C3%AE-deficiency-presenting-as-recurrent-acute-proximal-muscle-weakness-of-upper-and-lower-extremities-in-an-8-year-old-boy
#14
Bülent Kara, Hülya Maraş Genç, Emek Uyur-Yalçın, Ayfer Sakarya-Güneş, Uğur Topçu, Serap Mülayim, Serdar Ceylaner
The mitochondrial pyruvate dehydrogenase enzyme complex (PDHC) plays an important role in aerobic energy metabolism and acid-base equilibrium. PDHC contains of 5 enzymes, 3 catalytic (E1, E2, E3) and 2 regulatory, as well as 3 cofactors and an additional protein (E3-binding protein) encoded by nuclear genes. The clinical presentation of PDHC deficiency ranges from fatal neonatal lactic acidosis to chronic neurologic dysfunction without lactic acidosis. Paroxysmal neurologic problems such as intermittent ataxia, episodic weakness, exercise-induced dystonia and recurrent demyelination may also be seen although they are rare...
November 9, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27894750/risk-factors-for-autonomic-and-somatic-nerve-dysfunction-in-different-stages-of-glucose-tolerance
#15
Rumyana Dimova, Tsvetalina Tankova, Velina Guergueltcheva, Ivailo Tournev, Nevena Chakarova, Greta Grozeva, Lilia Dakovska
AIM: The present study evaluates autonomic and somatic nerve function in different stages of glucose tolerance and its correlation with different cardio-metabolic parameters. MATERIAL AND METHODS: Four hundred seventy-eight subjects, mean age 49.3±13.7years and mean BMI 31.0±6.2kg/m2, divided according to glucose tolerance: 130 with normal glucose tolerance (NGT), 227 with prediabetes (125 with impaired fasting glucose (IFG) and 102 with isolated impaired glucose tolerance (iIGT)), and 121 with newly-diagnosed T2D (NDT2D), were enrolled...
November 6, 2016: Journal of Diabetes and its Complications
https://www.readbyqxmd.com/read/27891705/spinal-cord-stimulation-in-experimental-chronic-painful-diabetic-polyneuropathy-delayed-effect-of-high-frequency-stimulation
#16
M van Beek, M van Kleef, B Linderoth, S M J van Kuijk, W M Honig, E A Joosten
BACKGROUND: Spinal cord stimulation (SCS) has been shown to provide pain relief in painful diabetic polyneuropathy (PDPN). As the vasculature system plays a great role in the pathophysiology of PDPN, a potential beneficial side-effect of SCS is peripheral vasodilation, with high frequency (HF) SCS in particular. We hypothesize that HF-SCS (500 Hz), compared with conventional (CON) or low frequency (LF)-SCS will result in increased alleviation of mechanical hypersensitivity in chronic experimental PDPN...
November 28, 2016: European Journal of Pain: EJP
https://www.readbyqxmd.com/read/27891585/novel-mutations-in-kars-cause-hypertrophic-cardiomyopathy-and-combined-mitochondrial-respiratory-chain-defect
#17
Daniela Verrigni, Daria Diodato, Michela Di Nottia, Alessandra Torraco, Emanuele Bellacchio, Teresa Rizza, Giulia Tozzi, Margherita Verardo, Fiorella Piemonte, Giorgio Tasca, Adele D'Amico, Enrico Bertini, Rosalba Carrozzo
Mutations in KARS, which encodes for both mitochondrial and cytoplasmic lysyl-tRNA synthethase, have been so far associated with three different phenotypes: the recessive form of Charcot Mary-Tooth polyneuropathy, the autosomal recessive non-syndromic hearing loss and the last recently described condition related to congenital visual impairment and progressive microcephaly. Here we report the case of a 14-years-old-girl with severe cardiomyopathy associated to mild psychomotor delay and mild myopathy; moreover, a diffuse reduction of cytochrome C oxidase (COX, complex IV) and a combined enzymatic defect of complex I (CI) and IV (CIV) was evident in muscle biopsy...
November 28, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/27891417/a-commonly-missed-well-known-entity-acute-intermittent-porphyria-a-case-report
#18
Smilu Mohanlal, Radha Gulati Ghildiyal, Alpana Kondekar, Poonam Wade, Richa Sinha
Acute Intermittent Porphyria (AIP) usually presents with abdominal pain, peripheral neuropathy and psychiatric manifestations. Incidence of AIP being 5 in 1,00,000. We present a case of an 11-year-old male child with multiple cranial nerve involvement, quadriparesis, focal convulsions, hypertension, hyponatremia with history of recurrent abdominal pain. His complete haemogram, ultrasonography (USG) abdomen, renal function tests were normal, he was also evaluated for tuberculosis which was negative. On further evaluation Electroencephalography (EEG) was suggestive of a generalised seizure disorder, MRI Brain suggestive of Posterior Reversible Encephalopathy Syndrome (PRES), Electromyography revealed a sensory motor axonal polyneuropathy and urine UV fluoresence test was positive for porphobilinogen which clinched the diagnosis of AIP...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27890673/functional-validation-of-abhd12-mutations-in-the-neurodegenerative-disease-pharc
#19
Angèle Tingaud-Sequeira, Demetrio Raldúa, Julie Lavie, Guilaine Mathieu, Magali Bordier, Anja Knoll-Gellida, Pierre Rambeau, Isabelle Coupry, Michèle André, Eva Malm, Claes Möller, Sten Andreasson, Nanna D Rendtorff, Lisbeth Tranebjærg, Michel Koenig, Didier Lacombe, Cyril Goizet, Patrick J Babin
ABHD12 mutations have been linked to neurodegenerative PHARC (polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, and early-onset cataract), a rare, progressive, autosomal, recessive disease. Although ABHD12 is suspected to play a role in the lysophosphatidylserine and/or endocannabinoid pathways, its precise functional role(s) leading to PHARC disease had not previously been characterized. Cell and zebrafish models were designed to demonstrate the causal link between an identified new missense mutation p...
November 23, 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/27884058/efficiency-of-silencing-rna-for-removal-of-transthyretin-v30m-in-a-ttr-leptomeningeal-animal-model
#20
Paula Gonçalves, Helena Martins, Susete Costelha, Luis F Maia, Maria Joao Saraiva
Some TTR mutants target the central nervous system (CNS). Familial amyloid polyneuropathy (FAP) with leptomeningeal involvement has been described in 9% of transthyretin (TTR) mutations and in valine for methionine at position 30 (V30M) patients. These individuals present dementia, ataxia, brain hemorrhages and focal neurological episodes (FNEs). FNEs occurred also in V30M FAP patients with longer disease duration, who have undergone liver transplant to remove the source of plasma mutant TTR as a form of treatment...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
keyword
keyword
16305
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"