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Sensorimotor polyneuropathy

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https://www.readbyqxmd.com/read/29314079/the-high-frequency-of-monoclonal-gammopathy-in-patients-with-diabetic-sensorimotor-polyneuropathy
#1
Alon Abraham, Carolina Barnett, Hans D Katzberg, Leif E Lovblom, A Perkins Bruce, Vera Bril
INTRODUCTION: Monoclonal gammopathy has been reported in several polyneuropathies. In this study, we aimed to explore the frequency and characteristics of monoclonal gammopathy in patients with diabetic sensorimotor polyneuropathy (DSP). METHODS: Patients with type 1 and 2 diabetes mellitus (DM 1, DM 2), and controls without diabetes, were evaluated between November 2008 and December 2013. RESULTS: 50 controls, 66 patients with DM 1, and 106 patients with DM 2, were included, with an average age of 43 ± 18, 45 ± 17, and 65 ± 10 years respectively...
January 4, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29279594/poems-syndrome
#2
W W T Nyunt, R Remli, F A Abdul Muttlib, C F Leong, N Masir, N R Tumian, S F S Abdul Wahid
POEMS syndrome is the syndrome of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and typical Skin changes. A 65-year-old lady presented with the 2-day-history of inability to walk, 4-month-history of progressive worsening of muscle weakness of both lower limbs and 1-year-history of progressive worsening of bilateral numbness of lower limbs. Nerve conduction study revealed generalized sensorimotor demyelinating polyneuropathy. She was initially treated as chronic inflammatory demyelinating polyradiculoneuropathy with intravenous immunoglobulin (IVIG) and high-dose prednisolone...
December 2017: Malaysian Journal of Pathology
https://www.readbyqxmd.com/read/29278894/asymmetric-ataxia-depression-memory-loss-epilepsy-and-axonal-neuropathy-associated-with-a-heterozygous-dna-polymerase-gamma-variant-of-uncertain-significance-c1370g-a-r457q
#3
Nivedita U Jerath, Michael E Shy
INTRODUCTION: Mutations in the gene encoding DNA polymerase gamma (POLG) impair its ability to proofread mitochondrial DNA (mtDNA) during replication [1]. This results in a high frequency of randomly distributed mtDNA mutations and thus a wide range of phenotypes, including seizures, neuropathy, and cerebellar ataxia [1, 2]. We document a phenotype associated with the rare POLG variant c.1370G>A (p.R457Q). METHODS: Over 10 years, we performed electrodiagnostic and neuropsychologic on a patient who presented with a variety of neurologic symptoms...
December 20, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/29194856/toronto-clinical-neuropathy-score-is-valid-for-a-wide-spectrum-of-polyneuropathies
#4
A Abraham, C Barnett, H D Katzberg, L E Lovblom, B A Perkins, V Bril
BACKGROUND AND PURPOSE: The Toronto Clinical Neuropathy Score (TCNS) is a valid and reliable scale for the diagnosis and staging of diabetic sensorimotor polyneuropathy. In this study, we aimed to explore the performance of the TCNS in non-diabetic polyneuropathies. METHODS: We performed a prospective study from November 2016 to May 2017 of patients with non-diabetic polyneuropathy. Patients had clinical, electrophysiological and functional assessments of their polyneuropathy, and the findings were correlated with the TCNS...
November 30, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29189887/eosinophilic-granulomatosis-with-polyangiitis-without-respiratory-symptoms-or-asthma-in-an-adolescent-case-report-and-literature-review
#5
REVIEW
Gülçin Otar Yener, Zahide Ekici Tekin, Neşe Çallı Demirkan, Selçuk Yüksel
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain...
November 30, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29147011/lower-serum-extracellular-superoxide-dismutase-levels-are-associated-with-polyneuropathy-in-recent-onset-diabetes
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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