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Sensorimotor polyneuropathy

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
Xin Chen, Jim Graham, Ioannis N Petropoulos, Georgios Ponirakis, Omar Asghar, Uazman Alam, Andrew Marshall, Maryam Ferdousi, Shazli Azmi, Nathan Efron, Rayaz A Malik
Objective: Corneal confocal microscopy (CCM), an in vivo ophthalmic imaging modality, is a noninvasive and objective imaging biomarker for identifying small nerve fiber damage. We have evaluated the diagnostic performance of previously established CCM parameters to a novel automated measure of corneal nerve complexity called the corneal nerve fiber fractal dimension (ACNFrD). Methods: A total of 176 subjects (84 controls and 92 patients with type 1 diabetes) with and without diabetic sensorimotor polyneuropathy (DSPN) underwent CCM...
February 1, 2018: Investigative Ophthalmology & Visual Science
S V Kotov, E V Isakova, V Yu Leidvoll, Yu A Belova, T V Volchenkova, A V Borodin, V A Shvedov
AIM: To study the efficacy of the complex therapy, including cocarnit (group B vitamins, triphosadenine and nicotinamide), of diabetic neuropathy. MATERIAL AND METHODS: Forty-one patients with diabetes mellitus type 2 and distal symmetric sensorimotor polyneuropathy were examined. Patients were divided into 2 groups. Patients of the main group (n=26) received complex therapy, including cocarnit, and patients of the comparison group (n=15) received standard treatment...
2018: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
Kazuhiro Hirayasu, Hideyuki Sasaki, Shohei Kishimoto, Seigo Kurisu, Koji Noda, Kenichi Ogawa, Hiroto Tanaka, Yumiko Sakakibara, Shohei Matsuno, Hiroto Furuta, Mikio Arita, Keigo Naka, Kishio Nanjo
AIM/INTRODUCTION: Studies on a novel point-of-care device for nerve conduction study called DPNCheck have been limited to Westerners. We aimed to clarify Japanese normal limits of nerve action potential (Amp) and conduction velocity (CV) by DPNCheck (Investigation-I), and validity of DPNCheck to identify diabetic symmetric sensorimotor polyneuropathy (DSPN) (Investigation-II). MATERIALS AND METHODS: Investigation-I: 463 non-neuropathic Japanese subjects underwent DPNCheck examinations...
February 11, 2018: Journal of Diabetes Investigation
Marinos C Dalakas
Polyneuropathy with immunoglobulin M (IgM) monoclonal gammopathy is the most common paraproteinemic neuropathy, comprising a clinicopathologically and immunologically distinct entity. The clinical spectrum spans from distal paresthesias and mild gait imbalance to more severe sensory ataxia, with falls and a varying degree of distal sensorimotor deficits. In approximately 75% of patients, the monoclonal IgM immunoreacts with myelin-associated glycoprotein (MAG) and sulfoglucuronyl glycosphingolipid (SGPG), or other peripheral nerve glycolipids that serve as antigens...
2018: Therapeutic Advances in Neurological Disorders
Tatsuhito Himeno, Hideki Kamiya, Jiro Nakamura
PURPOSE OF REVIEW: After a prolonged warm-up period of basic research, several modalities of cell replacement therapies are under development for diseases with no available cure. Diabetic polyneuropathy (DPN) is one of the most prevalent chronic diabetes complications that causes sensorimotor dysfunction, subsequent high risks for lower limb amputations, and high mortality. Currently, no disease modifying therapy exists for DPN. RECENT FINDINGS: Several types of well-documented stem/progenitor cells have been utilized for cell transplantation therapies in DPN model rodents: mesenchymal stromal cells (MSCs), endothelial progenitor cells (EPCs), and cells with similar characteristics of MSCs or EPCs derived from embryonic stem cells or induced pluripotent stem cells...
January 30, 2018: Current Diabetes Reports
Giulia Iapadre, Giovanni Morana, Maria Stella Vari, Francesca Pinto, Paola Lanteri, Alessandra Tessa, Filippo Maria Santorelli, Pasquale Striano, Alberto Verrotti
BACKGROUND: Charcot-Marie-Tooth (CMT) neuropathies represent the most common forms of inherited polyneuropathies. CMT2A, the axonal form, accounts for about one third of all CMT cases. Variants in the MFN2 gene have been recognized to be a major cause of CMT2A. To date, more than 100 pathogenetic mutations in MFN2 have been identified, leading to different neurological clinical spectrum, varying from hereditary neuropathies to more severe clinical phenotypes. Pathogenic variants in MFN2 mainly act in a dominant manner, although in a few sporadic or familial cases, homozygous or compound heterozygous mutations have been reported...
January 5, 2018: European Journal of Paediatric Neurology: EJPN
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
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
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
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...
March 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
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
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
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
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)
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
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
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
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
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
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