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J Jin, W Wang, T Gu, C Chen, J Sun, W Chen, Y Bi, D Zhu
AIM: To investigate the association between serum bilirubin and distal symmetrical polyneuropathy (DSPN) in Chinese patients with type 2 diabetes (T2D). METHODS: A total of 1800 inpatients with T2D (including 68 with 1-year follow-ups) were consecutively enrolled between June 2014 and March 2017. DSPN was diagnosed according to criteria recommended by the Toronto Diabetic Neuropathy Expert Group in 2010. Clinical data were retrospectively collected. RESULTS: Patients with vs...
February 22, 2018: Diabetes & Metabolism
Ángela Grillo Pizarro, Eduardo Achú Peralta, María Teresa Muñoz-Quezada, Boris Lucero Mondaca
OBJECTIVE: Organophosphate pesticides (OP) are used massively for their low cost and for their low environmental persistence and their high effectiveness in insect pest control, however, agricultural workers, when exposed to OP, have negative consequences on their health mainly neurological. For the first time, a research is reported in a Latin American population that studied the association between labor exposure to OP and the presence of peripheral polyneuropathy in agricultural workers...
March 22, 2018: Revista Española de Salud Pública
Kalliopi Pitarokoili, Moritz Kronlage, Philip Bäumer, Daniel Schwarz, Ralf Gold, Martin Bendszus, Min-Suk Yoon
Background: We present a clinical, electrophysiological, sonographical and magnetic resonance neurography (MRN) study examining the complementary role of two neuroimaging methods of the peripheral nervous system for patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Furthermore, we explore the significance of cross-sectional area (CSA) increase through correlations with MRN markers of nerve integrity. Methods: A total of 108 nerve segments on the median, ulnar, radial, tibial and fibular nerve, as well as the lumbar and cervical plexus of 18 CIDP patients were examined with high-resonance nerve ultrasound (HRUS) and MRN additionally to the nerve conduction studies...
2018: Therapeutic Advances in Neurological Disorders
Sonia Torres-Sanchez, Gisela Borges, Juan A Mico, Esther Berrocoso
Tapentadol is an analgesic that acts as an agonist of mu-opioid receptors (MOR) and that inhibits noradrenaline reuptake. Data from healthy rats show that tapentadol inhibits neuronal activity in the locus coeruleus (LC), a nucleus regulated by both the noradrenergic and opioid systems. Thus, we set out to investigate the effect of tapentadol on LC activity in streptozotocin (STZ)-induced diabetic rats, a model of diabetic polyneuropathy, by analyzing single-unit extracellular recordings of LC neurons. Four weeks after inducing diabetes, tapentadol dose-response curves were obtained from animals pre-treated with RX821002 or naloxone (alpha2-adrenoceptors and opioid receptors antagonists, respectively)...
March 15, 2018: Neuropharmacology
Michael Brines, Daniel A Culver, Maryam Ferdousi, Martijn R Tannemaat, Monique van Velzen, Albert Dahan, Rayaz A Malik
Small fiber neuropathy (SFN) is a common feature of many inflammatory diseases, often presenting with pain and disability. SFN is diagnosed using symptoms, thermal threshold testing, and intra-epidermal nerve fiber quantification. Corneal confocal microscopy (CCM) is an ophthalmic imaging technique which non-invasively quantifies corneal nerve fiber (CNF) density, branch density and length, and has comparable diagnostic and superior ability to identify nerve regeneration compared to skin biopsy. CNF size (width and area) depends upon the number of fibers within each nerve, as well as pathology (e...
March 16, 2018: Scientific Reports
Judit Bene, Kinga Hadzsiev, Bela Melegh
Type 2 diabetes is a highly prevalent chronic metabolic disorder characterized by hyperglycemia and associated with several complications such as retinopathy, hyperlipidemia and polyneuropathy. The dysregulated fatty acid metabolism along with tissue lipid accumulation is generally assumed to be associated in the development of insulin resistance and T2D. Moreover, several studies suggest a central role for oxidative stress in the pathogenesis of the disease. Since L-carnitine (LC) has an indispensable role in lipid metabolism via its involvement in the β-oxidation of long-chain fatty acids and it has antioxidant properties as well, carnitine supplementation may prove to be an effective tool in the management of the clinical course of T2D...
March 7, 2018: Nutrition & Diabetes
Antonella Sferra, Fabiana Fattori, Teresa Rizza, Elsabetta Flex, Emanuele Bellacchio, Alessandro Bruselles, Stefania Petrini, Serena Cecchetti, Massimo Teson, Fabrizia Restaldi, Andrea Ciolfi, Filippo M Santorelli, Ginevra Zanni, Sabina Barresi, Claudia Castiglioni, Marco Tartaglia, Enrico Bertini
Microtubules participate in fundamental cellular processes, including chromosomal segregation and cell division, migration, and intracellular trafficking. Their proper function is required for correct central nervous system development and operative preservation, and mutations in genes coding tubulins, the constituting units of microtubules, underlie a family of neurodevelopmental and neurodegenerative diseases, collectively known as "tubulinopathies", characterized by a wide range of neuronal defects resulting from defective proliferation, migration, and function...
March 14, 2018: Human Molecular Genetics
Ryutaro Tomita, Tetsuju Sekiryu, Hiroaki Shintake, Kuniharu Saito
PURPOSE: To report a 67-year-old woman with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, showing bilateral serous retinal detachment. The retinal lesion was evaluated by multimodal imaging using spectral domain optical coherence tomography and an adaptive optics (AO) camera. METHODS: A case report. RESULTS: Optical coherence tomography showed hyperreflective foci mainly in the inner retina in both eyes...
March 15, 2018: Retinal Cases & Brief Reports
Christoph Niemietz, Christoph Röcken, Matthias Schilling, Jörg Stypman, Constantin E Uhlig, Hartmut H-J Schmidt
Transthyretin-related Familial Amyloid Polyneuropathy (ATTR Amyloidosis, former FAP, here called TTR-FAP) is a rare, progressive autosomal dominant inherited amyloid disease ending fatal within 5 - 15 years after final diagnosis. TTR-FAP is caused by mutations of transthyretin (TTR), which forms amyloid fibrils affecting peripheral and autonomic nerves, the heart and other organs. Due to the phenotypic heterogeneity and partly not specific enough clinical symptoms, diagnosis of TTR-FAP can be complicated...
March 2018: Deutsche Medizinische Wochenschrift
Amey Dilip Sonavane, Sanjiv Saigal, Abhishek Kathuria, Narendra S Choudhary, Neeraj Saraf
Extrahepatic syndromes are uncommon manifestations of acute and chronic hepatitis B. The pathogenesis likely involves an aberrant immunologic response to extrahepatic viral proteins. Antiviral therapy reduces the availability of these viral protein antigens and thus halts immune activation. Approximately 1% of all cases of acute inflammatory demyelinating polyneuropathy are associated with hepatitis B. Guillain-Barre syndrome (GBS) is a remarkably clinically diverse disorder with distinctive variants characterised by an immune-mediated attack to components of the peripheral nervous system...
March 14, 2018: Clinical Journal of Gastroenterology
Jeffrey A Allen, Kenneth C Gorson, Deborah Gelinas
Introduction: We explored adherence to the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines for the diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) by reviewing data from a specialty pharmacy database. Materials and Methods: Clinical and electrophysiologic data were reviewed for 65 consecutive patients treated with intravenous immunoglobulin (IVIG) for CIDP. Three neuromuscular neurologists independently classified cases according to EFNS/PNS criteria as (1) fulfilling CIDP criteria; (2) non-CIDP (neither clinical nor electrophysiologic criteria met); or (3) unknown (insufficient information)...
March 2018: Brain and Behavior
Yasutaka Tajima, Mariko Matsumura, Hiroaki Yaguchi, Yasunori Mito
A 27-year-old woman with optic neuritis and cervical myelitis developed hypertrophic demyelinating polyneuropathy. It was hypothesized that the diagnosis was combined central and peripheral demyelination. A hypertrophic nerve was observed subcutaneously, and magnetic resonance imaging demonstrated marked hypertrophy of the nerve roots. The patient was negative for anti-aquaporin 4 antibodies. Her anti-neurofascin 155 antibody levels was slightly elevated, but it was not definitely positive. Pulsed steroid therapy and the administration of immunoglobulin ameliorated her symptoms...
2018: Internal Medicine
C Shivaprasad, Amit Goel, Alice Vilier, Jean-Henri Calvet
Context: Electrochemical skin conductance (ESC) test is a widely accepted objective technique for quantitatively assessing sudomotor dysfunction, which is one of the earliest-detected neurophysiologic abnormalities in diabetic patients with distal symmetric polyneuropathy. Aims: This study aimed to provide normative data for ESC values among healthy Indian participants and assess the potential influence of age, sex, and body mass index (BMI) on ESC measurements...
January 2018: Indian Journal of Endocrinology and Metabolism
Masaki Kobayashi, Douglas W Zochodne
Diabetic polyneuropathy (DPN) continues to be generally considered as a "microvascular" complication of diabetes mellitus alongside nephropathy and retinopathy. The microvascular hypothesis, however, may be tempered by the concept that diabetes directly targets dorsal root ganglion sensory neurons. This neuron specific concept, supported by accumulating evidence, might account for important features of DPN, such as its early sensory neuron degeneration. Diabetic sensory neurons develop neuronal atrophy alongside a series of mRNA changes related to declines in structural proteins, increases in heat shock protein (HSP), increases in the receptor for advanced glycation endproducts (RAGE), declines in growth factor signaling and other changes...
March 13, 2018: Journal of Diabetes Investigation
Peter Chung, Hope Northrup, Misbah Azmath, Ricardo A Mosquera, Shade Moody, Aravind Yadav
Distal hereditary motor neuropathies (dHMN) are a rare heterogeneous group of inherited disorders specifically affecting the motor axons, leading to distal limb neurogenic muscular atrophy. The GARS gene has been identified as a causative gene responsible for clinical features of dHMN type V in families from different ethnic origins and backgrounds. We present the first cohort of family members of Nigerian descent with a novel heterozygous p.L272R variant on the GARS gene. We postulate that this variant is the cause of dHMN-V in this family, leading to variable phenotypical expressions that are earlier than reported in previous cases...
2018: Case Reports in Pediatrics
Broes Martens, Michel De Pauw, Jan L De Bleecker
Familial amyloid polyneuropathy (FAP) is a most often length-dependent axonal neuropathy, often part of a multisystem disorder also affecting other organs, such as cardiac, gastrointestinal, genitourinary, renal, meningeal and eye tissue. It is most frequently the result of a mutation in the TTR gene, most commonly a p.Val50Met mutation. TTR-FAP is a rare autosomal dominant heritable disabling, heterogeneous disease in which early diagnosis is of pivotal importance when attempting treatment. This paper discusses the course of four Belgian FAP patients with different TTR mutations (p...
March 9, 2018: Acta Neurologica Belgica
Maarten van Beek, Denise Hermes, Wiel M Honig, Bengt Linderoth, Sander M J van Kuijk, Maarten van Kleef, Elbert A Joosten
OBJECTIVES: This study utilizes a model of long-term spinal cord stimulation (SCS) in experimental painful diabetic polyneuropathy (PDPN) to investigate the behavioral response during and after four weeks of SCS (12 hours/day). Second, we investigated the effect of long-term SCS on peripheral cutaneous blood perfusion in experimental PDPN. METHODS: Mechanical sensitivity was assessed in streptozotocin induced diabetic rats (n = 50) with von Frey analysis. Hypersensitive rats (n = 24) were implanted with an internal SCS battery, coupled to an SCS electrode covering spinal levels L2-L5...
March 9, 2018: Neuromodulation: Journal of the International Neuromodulation Society
Dan Ziegler, Rüdiger Landgraf, Ralf Lobmann, Karlheinz Reiners, Kristian Rett, Oliver Schnell, Alexander Strom
AIMS: We conducted a nationwide educational initiative to determine the prevalence and risk factors of diagnosed and undiagnosed painful and painless distal sensory polyneuropathy (DSPN). METHODS: Among 1,850 participants, 781 had no history of diabetes (ND), 126 had type 1 diabetes (T1D), and 943 had type 2 diabetes (T2D). Painful DSPN was defined as polyneuropathy detected by bedside tests with pain and/or burning in the feet, while painless DSPN was defined as polyneuropathy with paresthesias, numbness, or absence of symptoms...
March 5, 2018: Diabetes Research and Clinical Practice
Bo Hu, Megan Mccollum, Vignesh Ravi, Sezgi Arpag, Daniel Moiseev, Ryan Castoro, Bret C Mobley, Bryan W Burnette, Carly Siskind, John W Day, Robin Yawn, Shawna Feely, Yuebing Li, Qing Yan, Michael E Shy, Jun Li
OBJECTIVE: Charcot-Marie-Tooth type 4J (CMT4J) is a rare autosomal recessive neuropathy caused by mutations in FIG4 that result in loss of FIG4 protein. This study investigates the natural history and mechanisms of segmental demyelination in CMT4J. METHODS: Over the past 9 years, we have enrolled and studied a cohort of 12 CMT4J patients, including 6 novel FIG4 mutations. We evaluated these patients and related mouse models using morphological, electrophysiological and biochemical approaches...
March 8, 2018: Annals of Neurology
Irene Pulido-Valdeolivas, David Gómez-Andrés, Juan Andrés Martín-Gonzalo, Irene Rodríguez-Andonaegui, Javier López-López, Samuel Ignacio Pascual-Pascual, Estrella Rausell
The Hereditary Spastic Paraplegias (HSP) are a group of heterogeneous disorders with a wide spectrum of underlying neural pathology, and hence HSP patients express a variety of gait abnormalities. Classification of these phenotypes may help in monitoring disease progression and personalizing therapies. This is currently managed by measuring values of some kinematic and spatio-temporal parameters at certain moments during the gait cycle, either in the doctor´s surgery room or after very precise measurements produced by instrumental gait analysis (IGA)...
2018: PloS One
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