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Small fibre neuropathy

Joy K Adewusi, Marios Hadjivassiliou, Ana Vinagre-Aragón, Karen Ruth O'Connor, Aijaz Khan, Richard Adam Grünewald, Panagiotis Zis
BACKGROUND AND PURPOSE: Pain is a frequent and debilitating non-motor symptom of Idiopathic Parkinson's Disease (IPD). The present study investigated the prevalence of pain and specifically peripheral neuropathic pain (PNP) in IPD, and ascertained any impact of PNP on quality of life (QoL). METHODS: Patients with IPD and age- and gender-matched controls were screened for overall pain using the King's Parkinson's Pain Scale (KPPS). PNP was assessed using the Michigan Neuropathy Screening Instrument (MNSI)...
September 15, 2018: Journal of the Neurological Sciences
Hassan Fadavi, Mitra Tavakoli, Philip Foden, Maryam Ferdousi, Ioannis N Petropoulos, Maria Jeziorska, Nishi Chaturvedi, Andrew J M Boulton, Rayaz A Malik, Caroline A Abbott
BACKGROUND: Low foot ulcer risk in South Asian, compared with European, people with type 2 diabetes in the UK has been attributed to their lower levels of neuropathy. We have undertaken a detailed study of corneal nerve morphology and neuropathy risk factors, to establish the basis of preserved small nerve fibre function in South Asians versus Europeans. METHODS: In a cross-sectional, population-based study, age- and sex-matched South Asians (n = 77) and Europeans (n = 78) with type 2 diabetes underwent neuropathy assessment using corneal confocal microscopy, symptoms, signs, quantitative sensory testing, electrophysiology and autonomic function testing...
July 4, 2018: Diabetes/metabolism Research and Reviews
Marco Luigetti, Guido Primiano, Cristina Cuccagna, Daniela Bernardo, Donato Sauchelli, Catello Vollono, Serenella Servidei
OBJECTIVE: Polyneuropathy in mitochondrial diseases (MDs) is relatively common and widely investigated, but few data are instead reported about small fibres involvement. METHODS: In order to investigate the involvement of small fibres in MDs we performed extensive neurophysiological test (nerve conduction studies; sympathetic skin response; sudoscan) in 27 patients with genetic diagnosis of MD (7 m.3243A > G; 4 m.8344A > G; 9 single mtDNA deletion; 7 multiple mtDNA deletions)...
August 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Sanjeev Sharma, Victoria Tobin, Prashant R J Vas, Gerry Rayman
Context: Recent studies using skin biopsy suggest presence of small-fiber neuropathy in subclinical hypothyroidism. This study uses two noninvasive methods-the laser Doppler imager flare technique (LDIFLARE) and corneal confocal microscopy (CCM)-to assess small-fiber function (SFF) and small-fiber structure (SFS), respectively, in newly diagnosed hypothyroidism (HT) before and after adequate treatment. Design and Setting: Single-center, prospective, intervention-based cohort study...
August 1, 2018: Journal of Clinical Endocrinology and Metabolism
Alessandra Bortoluzzi, Ettore Silvagni, Federica Furini, Matteo Piga, Marcello Govoni
In the past years the peripheral nervous system (PNS) involvement in systemic lupus erythematosus (SLE) has received little attention despite its potential significant impact. The true prevalence of PNS in SLE reported in studies is variable and strongly influenced by American College of Rheumatology (ACR) case definition that includes seven PNS manifestations (acute inflammatory demyelinating polyradiculoneuropathy, autonomic disorder, mononeuropathy, myasthenia gravis, cranial neuropathy, plexopathy and polyneuropathy)...
May 24, 2018: Clinical and Experimental Rheumatology
Joy K Adewusi, Marios Hadjivassiliou, Ana Vinagre-Aragón, Karen Ruth O'Connor, Aijaz Khan, Richard Adam Grünewald, Panagiotis Zis
BACKGROUND: Neuropathic symptoms are commonly reported in Parkinson's disease (PD), but robust data on the epidemiology of such symptoms are lacking. The present study sought to investigate the prevalence and determinants of peripheral sensory neuropathic symptoms (PSNS) in idiopathic PD (IPD) and ascertain the effects of such symptoms on the patients' quality of life (QoL). METHODS: Patients with IPD and age-matched and gender-matched controls were screened for neuropathic symptoms using the Michigan Neuropathy Screening Instrument...
September 2018: Acta Neurologica Belgica
Nuria Montero, Alexandre Favà, Eva Rodriguez, Clara Barrios, Josep M Cruzado, Julio Pascual, Maria Jose Soler
BACKGROUND: Hepatitis C virus (HCV)-associated mixed cryoglobulinaemia is the manifestation of an inflammation of small and medium-sized vessels produced by a pathogenic IgM with rheumatoid factor activity generated by an expansion of B-cells. The immune complexes formed precipitate mainly in the skin, joints, kidneys or peripheral nerve fibres. Current therapeutic approaches are aimed at elimination of HCV infection, removal of cryoglobulins and also of the B-cell clonal expansions. The optimal treatment for it has not been established...
May 7, 2018: Cochrane Database of Systematic Reviews
Mathilde Duchesne, Laurence Richard, Jean-Michel Vallat, Laurent Magy
OBJECTIVE: Sudoscan provides a rapid assessment of sudomotor function based on the measurement of electrochemical skin conductance (ESC), which is thought to be proportional to small nerve fibres innervating the sweat glands. However, the relationship between ESC and small nerve fibre density on skin biopsy remains unclear. METHODS: In a retrospective single-centre study, we compared ESC measurements with autonomic sweat gland nerve fibre density (SGNFD) and somatic intraepidermal nerve fibre density (IENFD) in patients with polyneuropathy...
July 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
O Binns-Hall, D Selvarajah, D Sanger, J Walker, A Scott, S Tesfaye
AIMS: To evaluate the feasibility of a one-stop microvascular screening service for the early diagnosis of diabetic distal symmetrical polyneuropathy, painful distal symmetrical polyneuropathy and the at-risk diabetic foot. METHODS: People with diabetes attending retinal screening in hospital and community settings had their feet examined by a podiatrist. Assessment included: Toronto Clinical Neuropathy Score evaluation; a 10-g monofilament test; and two validated, objective and quick measures of neuropathy obtained using the point-of-care devices 'DPN-Check', a hand-held device that measures sural nerve conduction velocity and amplitude, and 'Sudoscan', a device that measures sudomotor function...
July 2018: Diabetic Medicine: a Journal of the British Diabetic Association
H-W Kan, H Chiang, W-M Lin, I-S Yu, S-W Lin, S-T Hsieh
AIMS: Sensory nerve degeneration and consequent abnormal sensations are the earliest and most prevalent manifestations of familial amyloid polyneuropathy (FAP) due to amyloidogenic transthyretin (TTR). FAP is a relentlessly progressive degenerative disease of the peripheral nervous system. However, there is a lack of mouse models to replicate the early neuropathic manifestations of FAP. METHODS: We established human TTR knock-in mice by replacing one allele of the mouse Ttr locus with human wild-type TTR (hTTRwt ) or human TTR with the A97S mutation (hTTRA97S )...
February 8, 2018: Neuropathology and Applied Neurobiology
Adnan Khan, Ioannis N Petropoulos, Georgios Ponirakis, Robert A Menzies, Omar Chidiac, Jennifer Pasquier, Charbel Abi Khalil, Talal K Talal, Rayaz A Malik
AIMS/INTRODUCTION: The aim of the present study was to identify the extent of small fiber neuropathy in diabetic patients with Charcot neuroarthropathy (CN). MATERIALS AND METHODS: A total of 20 patients with CN were compared with 20 age- and diabetes duration-matched patients with type 2 diabetes and 20 age-matched control participants. All patients underwent corneal confocal microscopy 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...
September 2018: Journal of Diabetes Investigation
Astrid J Terkelsen, Páll Karlsson, Giuseppe Lauria, Roy Freeman, Nanna B Finnerup, Troels S Jensen
Small fibre neuropathies are a heterogeneous group of disorders affecting thinly myelinated Aδ-fibres and unmyelinated C-fibres. Although multiple causes of small nerve fibre degeneration have been reported, including via genetic mutations, the cause of small fibre neuropathy remains unknown in up to 50% of cases. The typical clinical presentation of small fibre neuropathy is that of a symmetrical, length-dependent polyneuropathy associated with sensory or autonomic symptoms. More rarely, the clinical presentation is characterised by non-length-dependent, focal, or multifocal symptoms...
November 2017: Lancet Neurology
Praveen Anand, Rosario Privitera, Yiangos Yiangou, Philippe Donatien, Rolfe Birch, Peter Misra
BACKGROUND: Trench foot, or non-freezing cold injury (NFCI), results from cold exposure of sufficient severity and duration above freezing point, with consequent sensory and vascular abnormalities which may persist for years. Based on observations of Trench foot in World War II, the condition was described as a vaso-neuropathy. While some reports have documented nerve damage after extreme cold exposure, sensory nerve fibres and vasculature have not been assessed with recent techniques in NFCI...
2017: Frontiers in Neurology
Sean W Taylor, Ruple S Laughlin, Neeraj Kumar, Brent Goodman, Christopher J Klein, Peter J Dyck, P James B Dyck
INTRODUCTION: Myelopathy is considered the most common neurological complication of copper deficiency. Concurrent peripheral neuropathy has been recognised in association with copper deficiency but has not been well characterised. OBJECTIVES: To characterise the clinical, physiological and pathological features of copper-deficient peripheral neuropathy. METHODS: Patients with simultaneous copper deficiency (<0.78 μg/mL) and peripheral neuropathy seen at the Mayo Clinic from 1985 to 2005 were identified...
October 2017: Journal of Neurology, Neurosurgery, and Psychiatry
Uazman Alam, Maria Jeziorska, Ioannis N Petropoulos, Omar Asghar, Hassan Fadavi, Georgios Ponirakis, Andrew Marshall, Mitra Tavakoli, Andrew J M Boulton, Nathan Efron, Rayaz A Malik
OBJECTIVES: Corneal confocal microscopy (CCM) is a rapid, non-invasive, reproducible technique that quantifies small nerve fibres. We have compared the diagnostic capability of CCM against a range of established measures of nerve damage in patients with diabetic neuropathy. METHODS: In this cross sectional study, thirty subjects with Type 1 diabetes without neuropathy (T1DM), thirty one T1DM subjects with neuropathy (DSPN) and twenty seven non-diabetic healthy control subjects underwent detailed assessment of neuropathic symptoms and neurologic deficits, quantitative sensory testing (QST), electrophysiology, skin biopsy and corneal confocal microscopy (CCM)...
2017: PloS One
Rocco Liguori, Alex Incensi, Silvia de Pasqua, Renzo Mignani, Enrico Fileccia, Marisa Santostefano, Elena Biagini, Claudio Rapezzi, Silvia Palmieri, Ilaria Romani, Walter Borsini, Alessandro Burlina, Roberto Bombardi, Marco Caprini, Patrizia Avoni, Vincenzo Donadio
BACKGROUND: Fabry Disease (FD) is characterized by globotriaosylceramide-3 (Gb3) accumulation in several tissues and a small fibre neuropathy (SFN), however the underlying mechanisms are poorly known. This study aimed to: 1) ascertain the presence of Gb3 deposits in skin samples, by an immunofluorescence method collected from FD patients with classical GLA mutations or late-onset FD variants or GLA polymorphisms; 2) correlate skin GB3 deposits with skin innervation. METHODS: we studied 52 genetically-defined FD patients (32 with classical GLA mutations and 20 with late-onset variants or GLA polymorphisms), 15 patients with SFN associated with a specific cause and 22 healthy controls...
2017: PloS One
Nobuhiro Yuki, Amanda C Chan, Anna Hiu Yi Wong, Takayuki Inoue, Masafumi Yokai, Takashi Kurihara, JérôMe J Devaux, Einar Wilder-Smith
INTRODUCTION: We present a painful small-fiber neuropathy variant of Guillain-Barré syndrome characterized by antecedent infectious symptoms, hyporeflexia, and albuminocytologic dissociation. METHODS: Two patients received intravenous immunoglobulin, one corticosteroids. RESULTS: The patients subsequently improved. Immunoglobulin G (IgG) antibodies in their acute phase sera strongly bound to murine small nerve fibers, and the binding disappeared during the convalescent phase...
February 2018: Muscle & Nerve
Daniele Cazzato, Giuseppe Lauria
PURPOSE OF REVIEW: To provide a review on the state-of-art of clinical features, diagnostics, genetics and treatments of small fibre neuropathy (SFN). RECENT FINDINGS: The spectrum of clinical features has been widened from the classical presentation of burning feet as length-dependent SFN to that of small fibre dysfunction and/or degeneration associated with focal, diffuse and episodic neuropathic pain syndromes. The involvement of small nerve fibres in neurodegenerative diseases has been further defined, challenging the relationship between neuropathic pain symptoms and small fibre loss...
October 2017: Current Opinion in Neurology
E L Schaldemose, F I Fontain, P Karlsson, J R Nyengaard
INTRODUCTION: Corneal confocal microscopy (CCM) is a noninvasive clinical method to analyse and quantify corneal nerve fibres in vivo. Although the CCM technique is in constant progress, there are methodological limitations in terms of sampling of images and objectivity of the nerve quantification. The aim of this study was to present a randomized sampling method of the CCM images and to develop an adjusted area-dependent image analysis. Furthermore, a manual nerve fibre analysis method was compared to a fully automated method...
May 26, 2017: Journal of Microscopy
Sofia von Bischhoffshausen, Dinka Ivulic, Paola Alvarez, Victor C Schuffeneger, Juan Idiaquez, Constanza Fuentes, Pilar Morande, Ignacia Fuentes, Francis Palisson, David L H Bennett, Margarita Calvo
Small fibres in the skin are vulnerable to damage in metabolic or toxic conditions such as diabetes mellitus or chemotherapy resulting in small fibre neuropathy and associated neuropathic pain. Whether injury to the most distal portion of sensory small fibres due to a primary dermatological disorder can cause neuropathic pain is still unclear. Recessive dystrophic epidermolysis bullosa (RDEB) is a rare condition in which mutations of proteins of the dermo-epidermal junction lead to cycles of blistering followed by regeneration of the skin...
May 1, 2017: Brain: a Journal of Neurology
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