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Sensorimotor polyneuropathy

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https://www.readbyqxmd.com/read/29147011/lower-serum-extracellular-superoxide-dismutase-levels-are-associated-with-polyneuropathy-in-recent-onset-diabetes
#1
Alexander Strom, Kirti Kaul, Jutta Brüggemann, Iris Ziegler, Ilka Rokitta, Sonja Püttgen, Julia Szendroedi, Karsten Müssig, Michael Roden, Dan Ziegler
Increased oxidative stress is implicated in the pathogenesis of experimental diabetic neuropathy, but translational evidence in recent-onset diabetes is scarce. We aimed to determine whether markers of systemic oxidative stress are associated with diabetic sensorimotor polyneuropathy (DSPN) in recent-onset diabetes. In this cross-sectional study, we measured serum concentrations of extracellular superoxide dismutase (SOD3), thiobarbituric acid reactive substances (TBARS), and reduced glutathione (GSH) in 107 type 1 and 215 type 2 diabetes patients from the German Diabetes Study baseline cohort and 37 glucose-tolerant individuals (controls)...
November 17, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/29137918/spinobulbar-muscular-atrophy-combined-with-atypical-hereditary-neuropathy-with-liability-to-pressure-palsy
#2
Kyomin Choi, So Hyun Ahn, Seol-Hee Baek, Jun-Soon Kim, Seok-Jin Choi, Je-Young Shin, Sung-Min Kim, Yoon-Ho Hong, Jung-Joon Sung
Spinobulbar muscular atrophy (SBMA) is an X-linked recessive disease, presenting motor weakness and wasting of facial, bulbar and limb muscles. Hereditary neuropathy with liability to pressure palsy (HNPP) is autosomal dominant disorder characterized by recurrent neuropathies at common entrapment sites. We report a case of co-existence of SBMA and atypical HNPP with genetic confirmation of CAG expansion in the androgen receptor (AR) gene and deletion of the peripheral myelin protein 22 (PMP22) gene. A 62-year-old man presented with progressive muscle weakness, fasciculations in upper and lower limbs and dysesthesia predominantly in the distal regions...
November 11, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29137090/diabetic-and-sympathetic-influences-on-the-water-permeability-barrier-function-of-human-skin-as-measured-using-transepidermal-water-loss-a-case-control-study
#3
Seung Hoon Han, Ji Woong Park
The presence of long-standing hyperglycemic conditions has been suggested to lead to many skin problems associated with an impaired skin barrier function. However, the relationship between impaired skin barrier status and altered peripheral nervous system function has not yet been determined. The purpose of this study was to investigate the water evaporation rate as a measure of the permeability barrier function of diabetic skin and its relationship to diabetic sensorimotor polyneuropathy (DSPN) and peripheral autonomic neuropathy (PAN) using well-controlled confounding variables...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29130507/neurofascin-155-igg4-neuropathy-pathophysiological-insights-spectrum-of-clinical-severity-and-response-to-treatment
#4
Nidhi Garg, Susanna B Park, Con Yiannikas, Steve Vucic, James Howells, Yu-Ichi Noto, Emily K Mathey, John D Pollard, Matthew C Kiernan
INTRODUCTION: Sensorimotor neuropathy associated with IgG4 antibodies to neurofascin-155 (NF155) was recently described. The clinical phenotype is typically associated with young onset, distal weakness and in some cases, tremor. METHODS: From a consecutive cohort of 55 patients diagnosed with chronic inflammatory demyelinating polyneuropathy, screening for anti-NF155 antibodies was undertaken. Patients underwent clinical assessment, diagnostic neurophysiology, including peripheral axonal excitability studies and nerve ultrasound...
November 11, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/29119607/impaired-peripheral-nerve-regeneration-in-type-2-diabetic-mouse-model
#5
Pham Minh Vuong, H Tu Nguyen, Tayo Katano, Shinji Matsumura, Akira Saito, Akihiro Yamada, Hidemasa Furue, Seiji Ito
Peripheral neuropathy is one of the most common and serious complications of type-2 diabetes. Diabetic neuropathy is characterized by a distal symmetrical sensorimotor polyneuropathy, and its incidence increases in patients 40 years of age or older. In spite of extensive research over decades, there are few effective treatments for diabetic neuropathy besides glucose control and improved lifestyle. The earliest changes in diabetic neuropathy occur in sensory nerve fibers, with initial degeneration and regeneration resulting in pain...
November 9, 2017: European Journal of Neuroscience
https://www.readbyqxmd.com/read/29063451/diabetic-polyneuropathy-deep-white-matter-lesions-and-carotid-atherosclerosis-is-there-any-association
#6
Sevgi Ferik, Hayat Güven, Mehlika Panpallı Ateş, Işık Conkbayır, Selçuk Çomoğlu, Bülent Güven
The morphologic and functional damages of diabetes mellitus (DM) on microcirculation can play a role in the pathogenesis of both polyneuropathy and cerebral white matter lesions. The aim of this study is to investigate the relation between polyneuropathy and cerebral deep white matter lesions (DWMLs) and carotid atherosclerosis in patients with type 2 DM. Sixty-six patients with type 2 DM without any disorder that may cause polyneuropathy, and vascular risk factors except for DM and hyperlipidemia were included in the study...
October 23, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29056130/an-adult-with-polyneuropathy-and-hypogonadism-due-to-poems-syndrome
#7
Saba Zaidi, Sidra Sattar, Khealani Bhojo Asumal
POEMS (acronym for polyneuropathy, organomegaly, endocrinopathy, M protein myeloma and skin changes), is a rare disease which occurs in the setting of plasma cell dyscrasias. We describe a case of an adult lady who presented with gradual onset weakness of all four limbs and multisystem involvement characterized by pedal edema, ascites, hyperpigmentation and hypogonadism. Nerve conduction study showed severe sensorimotor polyneuropathy. Serum immunofixation showed lambda light chain restricted monoclonal gammopathy...
October 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29052096/life-paths-of-patients-with-transthyretin-related-familial-amyloid-polyneuropathy-val30met-a-descriptive-study
#8
Alice Lopes, Alexandra Sousa, Isabel Fonseca, Margarida Branco, Carla Rodrigues, Teresa Coelho, Jorge Sequeiros, Paula Freitas
Transthyretin-related familial amyloid polyneuropathy Val30Met is a fatal progressive disease. It is a rare hereditary amyloidosis, manifesting as a sensorimotor neuropathy and autonomic dysfunction. It begins during adulthood and is a disabling disease, posing a great psychological burden to patients and their families. Our aim was to describe and characterize life events related to the disease and discuss its psychosocial implications. Social and demographic data and a questionnaire on history of family and personal disease, and biographic events, were applied to 209 subjects attending an outpatient specialized clinic...
October 19, 2017: Journal of Community Genetics
https://www.readbyqxmd.com/read/28968368/immune-axonal-polyneuropathy
#9
Jinny O Tavee
PURPOSE OF REVIEW: Immune axonal polyneuropathy is caused by a diverse group of disorders that share similar presentations and treatment regimens. This article focuses on the clinical findings, evaluation, and management of immune-mediated causes of axonal polyneuropathy, focusing primarily on large fiber sensorimotor polyneuropathy. RECENT FINDINGS: Specific characteristics of an immune-mediated polyneuropathy have been incorporated in a new diagnostic screening tool that is highly sensitive and can easily be used in the outpatient clinic setting...
October 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28968367/axonal-sensorimotor-polyneuropathies
#10
Yuebing Li
PURPOSE OF REVIEW: This article describes clinical features of axonal sensorimotor polyneuropathies based on selected etiologies. RECENT FINDINGS: Axonal sensorimotor polyneuropathies have been well described for some time. Recent advances include the assessment of the incidence of peripheral neuropathy in the elderly, the recognition of the limited influence of electrodiagnostic testing on the clinical management of uncomplicated axonal sensorimotor polyneuropathy, the development of guidelines for treatment of painful neuropathy, the identification of risk factors predisposing patients for chemotherapy-induced neuropathy, a report on the association of metabolic syndrome and idiopathic axonal sensorimotor neuropathy, and the availability of more cost-effective genetic testing for identifying inherited polyneuropathies...
October 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28959382/nerve-decompression-and-neuropathy-complications-in-diabetes-are-attitudes-discordant-with-evidence
#11
REVIEW
D Scott Nickerson
External neurolysis of the nerve at fibro-osseous tunnels has been proprosed to treat or prevent signs, symptoms, and complications in the lower extremity of diabetes patients with sensorimotor polyneuropathy. Nerve decompression is justified in the presence of symptomatic compressed nerves in the several fibro-osseous tunnels of the extremities, which are known to be frequent in diabetes. Quite a body of literature has accumulated reporting results after such nerve decompression in the leg, describing pain relief and sensibility improvement, as well as balance recovery, diabetic foot ulcer prevention, curtailed ulcer recurrence risk, and amputation avoidance...
2017: Diabetic Foot & Ankle
https://www.readbyqxmd.com/read/28948076/peripheral-neuropathy-in-limbic-encephalitis-with-anti-glutamate-receptor-antibodies-case-report-and-systematic-literature-review
#12
Yi-Chia Wei, Chin-Chang Huang, Chi-Hung Liu, Hung-Chou Kuo, Jainn-Jim Lin
INTRODUCTION: Autoantibodies to the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor and N-methyl-d-aspartate (NMDA) receptor are known to be the causes of autoimmune encephalitis particularly limbic encephalitis. The involvement of the peripheral nervous system is rarely reported. METHODS: We analyzed the serial nerve conduction studies of a previously reported case of anti-AMPA receptor encephalitis, who was presented with conscious disturbance and quadriplegia...
September 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28914336/patterns-of-cutaneous-nerve-fibre-loss-and-regeneration-in-type-2-diabetes-with-painful-and-painless-polyneuropathy
#13
Gidon J Bönhof, Alexander Strom, Sonja Püttgen, Bernd Ringel, Jutta Brüggemann, Kálmán Bódis, Karsten Müssig, Julia Szendroedi, Michael Roden, Dan Ziegler
AIMS/HYPOTHESIS: The determinants and mechanisms of the development of diabetic sensorimotor polyneuropathy as a painful (DSPN+p) or painless (DSPN-p) entity remain unclear. We examined the degree of cutaneous nerve fibre loss and regeneration in individuals with type 2 diabetes with DSPN+p or DSPN-p compared with individuals with recent-onset type 2 diabetes and corresponding healthy volunteers. METHODS: In this cross-sectional study, skin biopsies taken from the distal lateral calf were obtained from individuals with recent-onset type 2 diabetes (n = 32) from the German Diabetes Study, with DSPN+p (n = 34) and DSPN-p (n = 32) from the PROPANE study, and volunteers with normal glucose tolerance (n = 50)...
December 2017: Diabetologia
https://www.readbyqxmd.com/read/28895640/the-application-of-sudoscan-for-screening-diabetic-peripheral-neuropathy-in-chinese-population-screening-dpn-by-sudoscan
#14
Jiewen Jin, Weimin Wang, Tianwei Gu, Wei Chen, Jing Lu, Yan Bi, Dalong Zhu
Purpose Diabetic peripheral neuropathies are the common chronic complications of diabetes, but the diagnosis is insensitive by physical examination in busy outpatients. Here we evaluated the performance of SUDOSCAN in screening diabetic peripheral neuropathies in Chinese type 2 diabetic patients. Methods The study enrolled 180 patients for annually screening. All patients underwent neurological symptoms assessment, clinical examination, nerve conduction studies and cardiovascular autonomic reflex tests. SUDOSCAN was tested and evaluated with electrochemical skin conductance in hands and feet, asymmetry ratio in hands and feet and predicted cardiac neuropathy...
September 11, 2017: Experimental and Clinical Endocrinology & Diabetes
https://www.readbyqxmd.com/read/28859335/a-new-mutation-in-fig4-causes-a-severe-form-of-cmt4j-involving-trpv4-in-the-pathogenic-cascade
#15
Benoit J Gentil, Erin O'Ferrall, Colin Chalk, Luis F Santana, Heather D Durham, Rami Massie
Mutations in FIG4, coding for a phosphoinositol(3,5) bisphosphate 5' phosphatase and involved in vesicular trafficking and fusion, have been shown causing a recessive form of Charcot-Marie-Tooth (CMT). We have identified a novel intronic mutation in the FIG4 in a wheel-chair bound patient presenting with a severe form of CMT4J and provide a longitudinal study. Investigations indicated a demyelinating sensorimotor polyneuropathy with diffuse active denervation and severe axonal loss. Genetic testing revealed that the patient is heterozygous for 2 FIG4 mutations, p...
September 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28795553/-chronic-demyelinating-polyradiculoneuropathy-revealing-a-solitary-plasmacytoma-of-the-right-iliac-bone
#16
J Oumerzouk, A Bourazza
Solitary bone plasmacytoma (pso) is a rare tumor, made of malignant plasma cells observed in a single bone, and without systemic proliferation. We report the case of a 33 year-old man who was admitted with chronic demyelinating sensorimotor polyneuropathy. Immunoelectrophoresis with immunofixation of serum proteins showed a monoclonal peak igg with lambda light chain and the workup was in favour of right hipbone secreting solitary plasmacytoma. The patient was treated with radiotherapy and steroids, and the evolution has been marked by a partial recovery of the motor deficit...
July 2017: Revue Médicale de Liège
https://www.readbyqxmd.com/read/28719236/applying-an-artificial-neural-network-model-for-developing-a-severity-score-for-patients-with-hereditary-amyloid-polyneuropathy
#17
Shenia Novis, Felipe Machado, Victor B Costa, Debora Foguel, Marcia W Cruz, José Manoel de Seixas
Hereditary (familial) amyloid polyneuropathy (FAP) is a systemic disease that includes a sensorimotor polyneuropathy related to transthyretin (TTR) mutations. So far, a scale designed to classify the severity of this disease has not yet been validated. This work proposes the implementation of an artificial neural network (ANN) in order to develop a severity scale for monitoring the disease progression in FAP patients. In order to achieve this goal, relevant symptoms and laboratory findings were collected from 98 Brazilian patients included in THAOS - the Transthyretin Amyloidosis Outcomes Survey...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28716288/uric-acid-levels-correlate-with-the-severity-of-diabetic-sensorimotor-polyneuropathy
#18
Alon Abraham, Ari Breiner, Carolina Barnett, Hans D Katzberg, Leif E Lovblom, Bruce A Perkins, Vera Bril
INTRODUCTION: Diabetic sensorimotor polyneuropathy (DSP) is the most frequent complication in patients with diabetes mellitus (DM), and is associated with age, DM duration, and HbA1c levels. In addition, higher uric acid (UA) levels are reported in patients with DSP. OBJECTIVES: To explore whether UA levels correlate with DSP severity. METHODS: We extracted the demographic data, clinical history, neurological and electrophysiological examinations and laboratory findings of 115 patients diagnosed with DSP from January 2012 to December 2015...
August 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28694632/late-onset-familial-amyloidotic-polyneuropathy-with-bence-jones-proteinuria-and-cardiomyopathy
#19
Sira Carrasco García de León, Amalia Hernández González, Carmen Orellana Alonso, Laura Burriel Lobo
Familial amyloidotic polyneuropathy is a genetically determined disease characterized by deposition of an anomalous transthyretin. A high index of suspicion is needed for this multisymptomatic and lethal disease to be diagnosed. The patient was a 70-year-old male examined due to hypesthesia in the hands and feet, plus difficulty walking. A neurophysiological study delivered the diagnosis of axonal sensorimotor polyneuropathy. He later developed cardiac symptoms and diarrhea. Urine laboratory analyses revealed a monoclonal spike of light chains (kappa)...
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28694071/first-clinical-and-genetic-description-of-a-family-diagnosed-with-late-onset-pompe-disease-from-costa-rica
#20
Gabriel Torrealba-Acosta, María Consuelo Rodríguez-Roblero, Sixto Bogantes-Ledezma, Kenneth Carazo-Céspedes, Claude Desnuelle
Glycogen storage disease type II, also known as Pompe disease, is an autosomal recessive disorder caused by deficiency of enzymatic activity of acid alpha-glucosidase. The wide phenotypical variation of this disease relates to the amount of residual enzymatic activity depending on the combination of mutations on each allele. We confirmed Pompe disease in a patient that presented with progressive weakness, recurrent episodes of respiratory failure associated with pneumonia, a predominantly demyelinating mixed sensorimotor polyneuropathy and paraspinal complex repetitive discharges...
June 20, 2017: Neuromuscular Disorders: NMD
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