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Small fiber neuropathy

Jenny L Davies, JaNean K Engelstad, Linde E Gove, Linda K Linbo, Rickey E Carter, Christopher Lynch, Nathan P Staff, Christopher J Klein, P James B Dyck, David N Herrmann, Peter J Dyck
INTRODUCTION: For sequential and somatotopic assessment of small fiber neuropathy (SFN), heat pain (HP) tests of hypoalgesia might be used instead of decreased counts of epidermal nerve fibers (ENFs), but then healthy subject reference values of HP thresholds are needed. METHODS: Using the CASE IVc system, HP thresholds of hypoalgesia were estimated for ten unilateral sites and counts of ENFs for four of them in healthy subjects. RESULTS: In healthy subjects, small but statistically significant differences of both HP thresholds of hypoalgesia and counts of ENFs were observed among tested sites...
March 15, 2018: Muscle & Nerve
Raquel Guimarães-Costa, Yoland Schoindre, Arnaud Metlaine, Jean-Pascal Lefaucheur, Jean-Philippe Camdessanché, Thierry Maisonobe, Jean-Marc Léger
A 59-year old woman presented with progressive paresthesias of all of her limbs for 4 years, associated with neuropathic pain, tingling in the tongue and allodynia, consistent with small-fiber neuropathy (SFN). Several systemic symptoms and signs were found on clinical examination and laboratory work-up. Neurological investigations including neurophysiologic test and skin biopsy supported the diagnosis of SFN. Chronic exposure to N-hexane was then disclosed and suspected to be the cause of the disease. Following the discontinuation of chronic N-hexane exposure, the patient had a progressive improvement of all signs and symptoms, reinforcing the correlation between exposure to N-hexane and development of SFN...
March 15, 2018: Journal of the Peripheral Nervous System: JPNS
Nirupa D Jayaraj, Bula J Bhattacharyya, Abdelhak A Belmadani, Dongjun Ren, Craig A Rathwell, Sandra Hackelberg, Brittany E Hopkins, Herschel R Gupta, Richard J Miller, Daniela M Menichella
Painful diabetic neuropathy (PDN) is an intractable complication of diabetes that affects 25% of patients. PDN is characterized by neuropathic pain and small-fiber degeneration, accompanied by dorsal root ganglion (DRG) nociceptor hyperexcitability and loss of their axons within the skin. The molecular mechanisms underlying DRG nociceptor hyperexcitability and small-fiber degeneration in PDN are unknown. We hypothesize that chemokine CXCL12/CXCR4 signaling is central to this mechanism, as we have shown that CXCL12/CXCR4 signaling is necessary for the development of mechanical allodynia, a pain hypersensitivity behavior common in PDN...
March 13, 2018: Journal of Clinical Investigation
Pia Flossdorf, Walter F Haupt, Anna Brunn, Martina Deckert, Gereon R Fink, Helmar C Lehmann, Gilbert Wunderlich
BACKGROUND: Small fiber neuropathy (SFN) is a challenging subtype of peripheral neuropathies. Once the diagnosis has been established, there is an uncertainty how SFN may progress, whether larger fibers will become involved over time, whether quality of life may be compromised, or whether repeated diagnostic workup in patients with unknown underlying cause may increase the yield of treatable causes of SFN. METHODS: We evaluated 16 patients with documented long-time course of idiopathic SFN...
March 8, 2018: European Neurology
Leonel J F Estofan, Stanislav Naydin, Gediminas Gliebus
We present a case report of a 20-year-old male with diabetes mellitus type 1, who developed severe painful peripheral neuropathy while on the second of a 10-day course with levofloxacin for the treatment of epididymitis. The intensity of the pain rapidly reached scores of 10/10 in a numeric scale 0/10, and the patient was transferred to an inpatient pain unit where he was treated aggressively with minimal improvement. A skin biopsy revealed small fiber neuropathy. Then the patient was treated with intravenous immunoglobulin, which improved the pain...
January 2018: Journal of Investigative Medicine High Impact Case Reports
Vincenzo Provitera, Christopher H Gibbons, Gwen Wendelschafer-Crabb, Vincenzo Donadio, Dino F Vitale, Adam Loavenbruck, Annamaria Stancanelli, Giuseppe Caporaso, Rocco Liguori, Ningshan Wang, Lucio Santoro, William R Kennedy, Maria Nolano
BACKGROUND: We aimed to test the clinical utility of leg to thigh intraepidermal nerve fiber (IENF) density ratio as a parameter to discriminate between length-dependent small fiber neuropathy (SFN) and small fiber sensory ganglionopathy (SFSG) in subjects with signs and symptoms of small fiber pathology. METHODS: We retrospectively evaluated thigh and leg IENF density in 314 subjects with small fiber pathology (173 with distal symmetrical length-dependent SFN and 141 with non length-dependent SFSG)...
March 1, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
L Misery
No abstract text is available yet for this article.
February 24, 2018: Annales de Dermatologie et de Vénéréologie
Julius Birnbaum
No abstract text is available yet for this article.
December 2017: Neurology. Clinical Practice
Brian C Callaghan, Rong Xia, Evan Reynolds, Mousumi Banerjee, Charles Burant, Amy Rothberg, Rodica Pop-Busui, Emily Villegas-Umana, Eva L Feldman
OBJECTIVE: To determine the comparative diagnostic characteristics of neuropathy measures in an obese population. METHODS: We recruited obese participants from the University of Michigan's Weight Management Program. Receiver operative characteristic analysis determined the area under the curve (AUC) of neuropathy measures for distal symmetric polyneuropathy (DSP), small fiber neuropathy (SFN), and cardiovascular autonomic neuropathy (CAN). The best test combinations were determined using stepwise and Score subset selection models...
March 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Mathilde Duchesne, Aurore Danigo, Laurence Richard, Jean-Michel Vallat, Shahram Attarian, Pierre-Marie Gonnaud, Arnaud Lacour, Yann Péréon, Tania Stojkovic, Klaus-Armin Nave, Viviane Bertrand, Serguei Nabirotchkin, Daniel Cohen, Claire Demiot, Laurent Magy
Charcot-Marie-Tooth disease type 1A (CMT1A), the most common form of Charcot-Marie-Tooth diseases, is a demyelinating neuropathy caused by a deletion encompassing the gene coding for PMP22, a myelin protein of the peripheral nervous system. Although myelinated fibers are mostly involved in CMT1A, some patients experience neuropathic pain. We thus investigated whether unmyelinated fibers are lost in CMT1A. Skin biopsies were taken from the distal portion of the leg of 80 patients with CMT1A as part of the PXT30003-01 study and processed for quantification of intraepidermal nerve fiber density (IENFD)...
February 2, 2018: Journal of Neuropathology and Experimental Neurology
S Lieber, M Blankenburg, K Apel, G Hirschfeld, P Hernáiz Driever, T Reindl
BACKGROUND: Chemotherapy-induced Peripheral Neuropathy (CIPN) of large-fibers affects up to 20% of survivors of pediatric acute lymphoblastic leukemia (ALL). We aimed to describe small-fiber toxicity and pain sensitization in this group. METHODS: In a cross-sectional, bicentric study we assessed 46 survivors of pediatric ALL (Mean age: 5.7 ± 3.5 years at diagnosis, median 2.5 years after therapy; males: 28). INCLUSION CRITERIA: ≥6 years of age, ≥3 months after last administration of Vincristine, and cumulative dose of Vincristine 12 mg/m2...
January 5, 2018: European Journal of Paediatric Neurology: EJPN
Steven E Harte, Daniel J Clauw, John M Hayes, Eva L Feldman, Irene C St Charles, Christopher J Watson
Introduction: Neuroimaging reveals increased glutamate within the insula of patients with fibromyalgia (FM), suggesting a link between FM symptoms and increased central excitatory neurotransmission. Many patients with FM also present with decreased intraepidermal nerve fiber density (IENFD), consistent with small fiber pathology. It remains unknown, however, whether either of these mechanistic findings represent a cause or a consequence of the other. This study tests the hypothesis that an excitatory imbalance within the insula leads to small fiber pathology...
May 2017: Pain Reports (Baltimore, Md.)
Adnan Khan, Ioannis N Petropoulos, Georgios Ponirakis, Robert A Menzies, Omar Chidiac, Jennifer Pasquier, Charbel A Khalil, Talal K Talal, Rayaz A Malik
AIMS/INTRODUCTION: The aim of this study was to identify the extent of small fibre neuropathy in patients with Charcot neuroarthropathy (CN). MATERIALS AND METHODS: 20 patients with CN were compared to 20 age and diabetes duration-matched patients with Type 2 diabetes and 20 age-matched controls. All patients underwent CCM with quantification of corneal nerve morphology and assessment for vibration perception threshold and a subset of patients with CN underwent assessment of sudomotor function and neuropathic pain...
January 30, 2018: Journal of Diabetes Investigation
Nicolas Collongues, Brigitte Samama, Catherine Schmidt-Mutter, Ludivine Chamard-Witkowski, Marc Debouverie, Jean-Baptiste Chanson, Maria-Cristina Antal, Karelle Benardais, Jérôme de Seze, Michel Velten, Nelly Boehm
BACKGROUND: Skin biopsy is the most relevant tool to diagnose small-fiber neuropathy. A well-documented normal dataset for intraepidermal nerve fiber in the distal leg is required to improve its diagnostic value. METHODS: Three hundred healthy subjects were enrolled in the study, after clinical and biological screening to exclude neurological and systemic pathologies. A distal leg biopsy was taken and intraepidermal nerve fiber density after protein gene product-9...
2018: PloS One
Alexandra Dimitrova
Background: Peripheral neuropathy (PN) is defined as damage to the peripheral nervous system caused by a primary lesion or dysfunction. Multiple recent trials have suggested that acupuncture is beneficial for treating neuropathic pain. One challenge in acupuncture research is the lack of standardization of point selection, number of needles used, needle-retention time, needling depth, amount of needle manipulation, and use of moxibustion and electroacupuncture (EA). Objectives: This article presents a standardized acupuncture protocol for the treatment of PN that incorporates structural acupuncture principles based on proximity to peripheral nerves and on traditional approaches to the treatment of neuropathic pain...
December 1, 2017: Medical Acupuncture
Heung Yong Jin, Tae Sun Park
Diabetic peripheral neuropathy (DPN) is the most common chronic neurological complication of diabetes1 . Small and large peripheral nerve fibers may be involved in DPN. Large nerve fiber damage causes paresthesia, sensory loss, and muscle weakness and small nerve fiber damage is associated with pain, anesthesia, foot ulcer, and autonomic symptoms. However, the exact pathogenic mechanisms and diagnostic criteria for DPN are not firmly established currently. The present DPN prevention, diagnosis, and treatment strategies are incomplete and unsuccessful due to the various pathogenesis of systemic and cellular disturbance in glucose and lipid metabolism...
December 26, 2017: Journal of Diabetes Investigation
Mengyu Wang, Tobias Elze, Dian Li, Neda Baniasadi, Kerstin Wirkner, Toralf Kirsten, Joachim Thiery, Markus Loeffler, Christoph Engel, Franziska G Rauscher
Optical coherence tomography (OCT) manufacturers graphically present circumpapillary retinal nerve fiber layer thickness (cpRNFLT) together with normative limits to support clinicians in diagnosing ophthalmic diseases. The impact of age on cpRNFLT is typically implemented by linear models. cpRNFLT is strongly location-specific, whereas previously published norms are typically restricted to coarse sectors and based on small populations. Furthermore, OCT devices neglect impacts of lens or eye size on the diameter of the cpRNFLT scan circle so that the diameter substantially varies over different eyes...
December 2017: Journal of Biomedical Optics
Jennifer J Brown, Shana L Pribesh, Kimberly G Baskette, Aaron I Vinik, Sheri R Colberg
Objective: Examine the effectiveness of the 128 Hz tuning fork, two monofilaments, and Norfolk Quality of Life Diabetic Neuropathy (QOL-DN) questionnaire as tools for the early detection of diabetic peripheral neuropathy (DPN) in overweight, obese, and inactive (OOI) adults or those who have prediabetes (PD) or type 2 diabetes (T2D). Research Design and Methods: Thirty-four adults (mean age 58.4 years ± 12.1) were divided by glycemia (10 OOI normoglycemic, 13 PD, and 11 T2D)...
2017: Journal of Diabetes Research
Violaine Plante-Bordeneuve
Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a progressive, fatal, inherited disorder first identified in Portugal and now recognized in all continents. Over the past decade, thanks to the availability of the genetic test, our knowledge on the range of clinical expressions of this disorder has expanded, including different patterns and progression rates of the neuropathy, as well as aspects of the cardiomyopathy, which can be prominent. In the mean time, new tools are being developed to detect earlier TTR amyloid deposition such as cardiac scintigraphy with technetium-labelled pyrophosphate tracers or small nerve fiber alterations from skin biopsies, or using neurophysiological approaches as well as magnetic resonance neurography (MRN)...
December 16, 2017: Journal of Neurology
Celine Hendriks, Marjolein Drent, Willemien De Kleijn, Marjon Elfferich, Petal Wijnen, Jolanda De Vries
BACKGROUND: Fatigue is a major and disabling problem in sarcoidosis. Knowledge concerning correlates of the development of fatigue and possible interrelationships is lacking. OBJECTIVE: A conceptual model of fatigue was developed and tested. METHODS: Sarcoidosis outpatients (n = 292) of Maastricht University Medical Center completed questionnaires regarding trait anxiety, depressive symptoms, cognitive failure, dyspnea, social support, and small fiber neuropathy (SFN) at baseline...
November 20, 2017: Respiratory Medicine
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