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Small fiber neuropathy

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https://www.readbyqxmd.com/read/28915853/low-threshold-unmyelinated-mechanoafferents-can-modulate-pain
#1
Kathrin Habig, Anne Schänzer, Wolfgang Schirner, Gothje Lautenschläger, Benjamin Dassinger, Håkan Olausson, Frank Birklein, Elke R Gizewski, Heidrun H Krämer
BACKGROUND: Human, hairy skin contains a subgroup of C-fibers, the C-low threshold mechanoreceptive afferents ((C-LTMR) C-tactile or C-touch (CT) fibers) that are linked with the signaling of affective aspects of human touch. Recent studies suggest an involvement of these afferents in the modulation of pain in healthy volunteers. Small fiber neuropathy (SFN) is associated with a damage of C-fibers. Therefore, an impairment of C-LTMRs can be assumed. We aimed to elaborate a possible role of CT-afferents in pain modulation by investigating healthy volunteers and SFN-patients...
September 15, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28902413/frequent-genes-in-rare-diseases-panel-based-next-generation-sequencing-to-disclose-causal-mutations-in-hereditary-neuropathies
#2
Maike F Dohrn, Nicola Glöckle, Lejla Mulahasanovic, Corina Heller, Julia Mohr, Christine Bauer, Erik Riesch, Andrea Becker, Florian Battke, Konstanze Hörtnagel, Thorsten Hornemann, Saranya Suriyanarayanan, Markus Blankenburg, Jörg B Schulz, Kristl G Claeys, Burkhard Gess, Istvan Katona, Andreas Ferbert, Debora Vittore, Alexander Grimm, Stefan Wolking, Ludger Schöls, Holger Lerche, G Christoph Korenke, Dirk Fischer, Bertold Schrank, Urania Kotzaeridou, Gerhard Kurlemann, Bianca Dräger, Anja Schirmacher, Peter Young, Beate Schlotter-Weigel, Saskia Biskup
Hereditary neuropathies comprise a wide variety of chronic diseases associated to more than 80 genes identified to date. We herein examined 612 index patients with either a Charcot-Marie-Tooth phenotype, hereditary sensory neuropathy, familial amyloid neuropathy, or small fiber neuropathy using a customized multigene panel based on the next generation sequencing (NGS) technique. In 121 cases (19.8%), we identified at least one putative pathogenic mutation. Out of these, 54.4% showed an autosomal dominant, 33...
September 13, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28898515/age-related-changes-in-and-determinants-of-macular-ganglion-cell-inner-plexiform-layer-thickness-in-normal-chinese-adults
#3
Yan Jiao Huo, Yan Guo, Lei Li, Huai Zhou Wang, Ya Xing Wang, Ravi Thomas, Ning Li Wang
IMPORTANCE: Consideration of age-related changes in macular ganglion cell-inner plexiform layer (mGCIPL) thickness are important for glaucoma progression analysis. BACKGROUND: To report age-related changes in and the determinants of high-definition optical coherence tomography (HD-OCT) measurements of mGCIPL thickness. DESIGN: Cross-sectional study. PARTICPANTS: 326 healthy adults. METHODS: All subjects underwent Cirrus HD-OCT measurements of mGCIPL...
September 12, 2017: Clinical & Experimental Ophthalmology
https://www.readbyqxmd.com/read/28895058/a-neuropathic-pain-syndrome-associated-with-hantavirus-infection
#4
Dustin Anderson, Grayson Beecher, Christopher Power, Lindsay Bridgland, Douglas W Zochodne
Hantaviruses are a group of single-stranded RNA viruses of the Bunyaviridae family. "New World" hantaviruses cause hantavirus cardiopulmonary syndrome (HCPS) in North America. HCPS carries with it significant mortality and those patients who survive the disease are often left with substantial morbidity. Neurologic complications of hantavirus infections are rare, with only sparse cases of central nervous system involvement having been documented in the literature. To our knowledge, there are no reports of hantavirus infection contributing to peripheral nervous system dysfunction...
September 11, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/28894389/quantification-of-small-fiber-pathology-in-patients-with-sarcoidosis-and-chronic-pain-using-cornea-confocal-microscopy-and-skin-biopsies
#5
Linda Cj Oudejans, Marieke Niesters, Michael Brines, Albert Dahan, Monique van Velzen
Small fiber pathology with concomitant chronic neuropathic pain is a common complication of sarcoidosis. The gold standard of diagnosis of small fiber neuropathy (SFN) is the quantification of small nerve fibers in skin biopsies in combination with patient history and psychophysical tests; a new technique is the quantification of small nerve fibers in the cornea using cornea confocal microscopy (CCM). Here, we studied small fiber morphology in sarcoidosis patients with neuropathic pain using skin biopsies, CCM, and quantitative sensory testing (QST)...
2017: Journal of Pain Research
https://www.readbyqxmd.com/read/28884982/comparison-of-somatic-and-sudomotor-nerve-fibers-in-type-2-diabetes-mellitus
#6
Eun Hee Sohn, Kyu Sang Song, Ju Yeon Lee, Ae Young Lee
BACKGROUND AND PURPOSE: The objective of this study was to find a sensitive method for the early detection of diabetic polyneuropathy (DPN) and determine the relationship between the functions of somatic and autonomic small nerve fibers in DPN. METHODS: Patients with type 2 diabetes mellitus and DPN based on clinical symptoms, signs, intraepidermal nerve fiber density (IENFD), and findings in the quantitative sudomotor axon reflex test (QSART) were enrolled retrospectively...
September 4, 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/28882562/candesartan-prevents-resiniferatoxin-induced-sensory-small-fiber-neuropathy-in-mice-by-promoting-angiotensin-ii-mediated-at2-receptor-stimulation
#7
Flavien Bessaguet, Aurore Danigo, Laurent Magy, Franck Sturtz, Alexis Desmoulière, Claire Demiot
Sensory defects associated with small-fiber neuropathy (SFN) can lead to profound disabilities. The relationship between the sensory nervous system and modulation of the renin-angiotensin system (RAS) has been described and focused on pain and neurodegeneration in several animal models. We have recently developed an experimental model of functional sensory neuropathy showing thermal hypoalgesia and neuropeptide depletion without nerve fiber degeneration. Here, we aimed to determine whether the modulation of angiotensin II (Ang II) activity could prevent sensory neuropathy induced by RTX...
September 5, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28870595/evidence-of-small-fiber-neuropathy-sfn-in-two-patients-with-unexplained-genital-sensory-loss-and-sensory-urinary-cystopathy
#8
Mahmoud A AbdelRazek, Bart Chwalisz, Anne Louise Oaklander, Nagagopal Venna
No abstract text is available yet for this article.
September 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28867384/downregulations-of-trpm8-expression-and-membrane-trafficking-in-dorsal-root-ganglion-mediate-the-attenuation-of-cold-hyperalgesia-in-cci-rats-induced-by-gfr%C3%AE-3-knockdown
#9
Lin Su, Ruichen Shu, Chengcheng Song, Yonghao Yu, Guolin Wang, Yazhuo Li, Changxiao Liu
BACKGROUND: Cold hyperalgesia is an intractable sensory abnormality commonly seen in peripheral neuropathies. Although glial cell line-derived neurotrophic factor family receptor alpha3 (GFRα3) is required for the formation of pathological cold pain has been revealed, potential transduction mechanism is poorly elucidated. We have previously demonstrated the contribution of enhanced activity of transient receptor potential melastatin 8 (TRPM8) to cold hyperalgesia in neuropathic pain using a rat model of chronic constriction injury (CCI) to the sciatic nerve...
September 1, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28855885/laser-doppler-assessment-of-vasomotor-axon-reflex-responsiveness-to-evaluate-neurovascular-function
#10
REVIEW
Marie Luise Kubasch, Anne Sophie Kubasch, Juliana Torres Pacheco, Sylvia J Buchmann, Ben Min-Woo Illigens, Kristian Barlinn, Timo Siepmann
The vasomotor axon reflex can be evoked in peripheral epidermal nociceptive C-fibers to induce local vasodilation. This neurogenic flare response is a measure of C-fiber functional integrity and therefore shows impairment in patients with small fiber neuropathy. Laser Doppler flowmetry (LDF) and laser Doppler imaging (LDI) are both techniques to analyze vasomotor small fiber function by quantifying the integrity of the vasomotor-mediated axon reflex. While LDF assesses the flare response following acetylcholine iontophoresis with temporal resolution at a single defined skin point, LDI records flare responses with spatial and temporal resolution, generating a two-dimensional map of superficial blood flow...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28828222/longitudinal-study-of-neuropathy-microangiopathy-and-autophagy-in-sural-nerve-implications-for-diabetic-neuropathy
#11
Simin Mohseni, Medeea Badii, Axel Kylhammar, Niels O B Thomsen, Karl-Fredrik Eriksson, Rayaz A Malik, Ingmar Rosén, Lars B Dahlin
OBJECTIVES: The progression and pathophysiology of neuropathy in impaired glucose tolerance (IGT) and type 2 diabetes (T2DM) is poorly understood, especially in relation to autophagy. This study was designed to assess whether the presence of autophagy-related structures was associated with sural nerve fiber pathology, and to investigate if endoneurial capillary pathology could predict the development of T2DM and neuropathy. PATIENTS AND METHODS: Sural nerve physiology and ultrastructural morphology were studied at baseline and 11 years later in subjects with normal glucose tolerance (NGT), IGT, and T2DM...
August 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28827489/pyridoxine-toxicity-small-fiber-neuropathy-with-dysautonomia-a-case-report
#12
Rae Bacharach, Max Lowden, Aiesha Ahmed
Pyridoxine (vitamin B6) toxicity is a well-known cause of primary sensory, length-dependent, axonal polyneuropathy. Although sensory symptoms predominate, autonomic symptoms have also been reported in some cases. To date, there is no objective evidence of autonomic dysfunction reported in the literature. We present the case of a 41-year-old woman with 2 years of progressive burning pain, numbness, tingling, and weakness in a stocking-glove distribution who was found to have severe pyridoxine toxicity. Concurrent presence of large and small fiber nerve dysfunction was noted in the form of abnormal electromyography/nerve conduction study demonstrating a chronic sensory polyneuropathy and autonomic testing demonstrating abnormal responses to quantitative sweat testing and cardiovagal function testing...
September 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28827487/clinical-and-laboratory-profiles-of-idiopathic-small-fiber-neuropathy-in-children-case-series
#13
Jafar Kafaie, Ali Al Balushi, Minsoo Kim, Alan Pestronk
The role of autoimmune mechanisms in idiopathic small fiber neuropathy (SFN) is not completely understood. Serum IgM binding to trisulfated disaccharide IdoA2S-GlcNS-6S (TS-HDS) and IgG to fibroblast growth factor receptor 3 were associated with sensory motor polyneuropathies and sensory neuronopathy among others. In this retrospective case review, we describe the clinical and laboratory findings of idiopathic SFN in a small cohort of pediatric patients. Eight children were diagnosed with SFN clinically and confirmed by reduced epidermal nerve fiber density...
September 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28827483/less-is-more-in-diabetic-neuropathy-diagnosis-comparison-of-quantitative-sudomotor-axon-reflex-and-skin-biopsy
#14
Ahmad R Abuzinadah, Patricia Kluding, Douglas Wright, Linda DʼSilva, Janelle Ryals, Bill Hendry, Omar Jawdat, Laura Herbelin, April L McVey, Richard J Barohn, Mazen M Dimachkie, Mamatha Pasnoor
OBJECTIVES: To compare the frequency of abnormalities in epidermal nerve fiber density (ENFD) and quantitative sudomotor axon reflex (QSART) in patients with diabetic distal symmetric polyneuropathy (DSPN). METHODS: Nerve conduction studies, ENFD, and QSART data were obtained pre- and postexercise, in patients enrolled in a prospective diabetic neuropathy study. McNemar's test was applied to compare the yield of ENFD and QSART. RESULTS: Eighteen patients (58 ± 4 years) were enrolled, with 36 data collection points...
September 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28810595/sudoscan-an-effective-tool-for-screening-chronic-kidney-disease-in-patients-with-type-2-diabetes
#15
Fei Mao, Siying Liu, Xiaona Qiao, Hangping Zheng, Qian Xiong, Jie Wen, Shuo Zhang, Zhaoyun Zhang, Hongying Ye, Hongli Shi, Bin Lu, Yiming Li
SUDOSCAN is a non-invasive method of measuring peripheral small fiber and autonomic nerve activity by detection of abnormal sweat gland function through electrochemical skin conductance. It has been reported to be an effective screening tool in early detection of microvascular type 2 diabetes mellitus (T2DM) complications including diabetic neuropathy and nephropathy in recent studies. However, previous studies used estimated glomerular filtration rate (eGFR) as the golden standard, which has a 90% chance of being within 30% of the measured GFR at best...
August 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28797868/neuropathic-pain-and-nerve-growth-factor-in-chemotherapy-induced-peripheral-neuropathy-prospective-clinical-pathological-study
#16
R Velasco, X Navarro, M Gil-Gil, M Herrando-Grabulosa, A Calls, J Bruna
CONTEXT: Neuropathic pain can be present in patients developing chemotherapy-induced peripheral neuropathy (CIPN). Nerve growth factor (NGF) is trophic to small sensory fibers and regulates nociception. OBJECTIVES: We investigated the changes in serum NGF and intraepidermal nerve fiber density (IENFD) in skin biopsies of cancer patients receiving neurotoxic chemotherapy in a single-center prospective observational study. METHODS: Patients were evaluated before and after chemotherapy administration...
August 7, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/28794847/neuro-otological-and-peripheral-nerve-involvement-in-fabry-disease
#17
Sergio Carmona, Romina Weinschelbaum, Ana Pardal, Cintia Marchesoni, Paz Zuberbuhler, Patricia Acosta, Guillermo Cáceres, Isaac Kisinovsky, Luciana Bayón, Ricardo Reisin
Fabry disease (FD) is an X-linked lysosomal storage disease, with multisystemic glycosphingolipids deposits. Neuro-otological involvement leading to hearing loss and vestibular dysfunctions has been described, but there is limited information about the frequency, site of lesion, or the relationship with peripheral neuropathy. The aim was to evaluate the presence of auditory and vestibular symptoms, and assess neurophysiological involvement of the VIII cranial nerve, correlating these findings with clinical and neurophysiological features of peripheral neuropathy...
July 18, 2017: Audiology Research
https://www.readbyqxmd.com/read/28791051/a-unique-case-for-spinal-cord-stimulation-successful-treatment-of-small-fiber-neuropathy-pain-using-multiple-spinal-cord-stimulators
#18
Maxim Eckmann, Alexander Papanastassiou, Mark Awad
Spinal cord stimulators have commonly been used to treat multiple pain conditions. This case report represents a unique case of using multiple spinal cord stimulators for widespread small fiber neuropathy pain. This case report concerns patient JJ who first presented with generalized neuropathic pain. His pain was an intermittent burning, stinging quality that originally focused in both of his feet and progressed to include his legs and arms and eventually involved his entire body. The pain would last moments to hours at least daily...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28747449/loss-of-cutaneous-large-and-small-fibers-in-naive-and-l-dopa-treated-pd-patients
#19
Maria Nolano, Vincenzo Provitera, Fiore Manganelli, Rosa Iodice, Annamaria Stancanelli, Giuseppe Caporaso, Annamaria Saltalamacchia, Francesca Califano, Bernardo Lanzillo, Marina Picillo, Paolo Barone, Lucio Santoro
OBJECTIVE: To study small and large fiber pathology in drug-naive and l-dopa-treated patients affected by Parkinson disease (PD) in early phases, before the occurrence of neuropathic electrophysiologic abnormalities. METHODS: We enrolled 85 patients with idiopathic PD (male/female 49/36, age 61.3 ± 9.7 years) without electrophysiologic signs of neuropathy, including 48 participants naive to l-dopa treatment. All patients underwent clinical, functional, and morphologic assessment of sensory and autonomic nerves through dedicated questionnaires, quantitative sensory testing, sympathetic skin response, dynamic sweat test, and punch biopsies from glabrous and hairy skin...
August 22, 2017: Neurology
https://www.readbyqxmd.com/read/28728619/restless-genital-syndrome-differential-diagnosis-and-treatment-with-pramipexole
#20
Emilia Sforza, David Hupin, Frédéric Roche
Restless genital syndrome refers to excessive and persistent sensations of genital and clitoral arousal with the absence of conscious feeling of sexual desire. These sensations have been linked to symptoms of restless legs syndrome (RLS) or an overactive bladder. Restless genital syndrome may be related to small fiber sensory neuropathy of the dorsal nerve of the clitoris, so it more frequently occurs in women. The association with RLS suggests a possible dopaminergic mechanism. We report a case of a 74-year-old woman who presented restless genital symptoms every night in association with RLS symptoms...
September 15, 2017: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
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