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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
Stefan Wietek
AIM: To present data from three studies of a Post-Authorization Safety Surveillance (PASS) program for the subset of patients receiving octagam® 5% or 10% for chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Data on patients with CIDP treated with octagam were analyzed to assess its safety and tolerability. RESULTS: Of 2314 patients included in the studies, 58 patients (mean age: 64.6 years) received octagam for CIDP, mean dose of which was 0...
March 8, 2018: Neurodegenerative Disease Management
Yan-Qin Wang, Han Chen, Wu-Ping Zhuang, Hong-Lei Li
BACKGROUND: Combined central and peripheral demyelination (CCPD) is rare and has never been reported as a spectrum disease in Han Chinese population. OBJECTIVES: To study the clinical features of CCPD in Han Chinese patients. METHODS: Twenty-two CCPD patients were selected from 788 demyelination cases. We reviewed and compared the clinical manifestation, laboratory data, electrophysiological examination, MRI and the prognosis. RESULTS: CCPD patients presented with sensory disturbance (86...
April 15, 2018: Journal of Neuroimmunology
Masayoshi Yamamoto, Yoko Shibata, Nobuyuki Oka, Manabu Inoue, Naoko Tachibana, Toshiaki Hamano
The patient was a 58-year-old man, who was hospitalized with progressive bilateral leg weakness, and sensory impairment in all four extremities. Chronic inflammatory demyelinating polyneuropathy (CIDP) was suspected based on an electrophysiological examination, and intravenous immunoglobulin therapy (IVIg) was initiated. However, his symptoms progressed. The serum and cerebrospinal fluid were positive for human T-lymphotropic virus type 1 (HTLV-1) antibodies; hence the patient was diagnosed as having HTLV-1 associated neuropathy...
February 28, 2018: Rinshō Shinkeigaku, Clinical Neurology
Haruki Koike, Ryoji Nishi, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Naoki Atsuta, Tomohiko Nakamura, Masaaki Hirayama, Hidenori Ogata, Ryo Yamasaki, Jun-Ichi Kira, Masahisa Katsuno, Gen Sobue
We herein report a woman with chronic inflammatory demyelinating polyneuropathy (CIDP) in whom positivity for anti-neurofascin 155 antibodies was revealed 23 years after the onset of neuropathy. The patient initially reported numbness in the face at 50 years of age and subsequently manifested features compatible to typical CIDP. Steroid administration initiated at 54 years of age ameliorated her neuropathic symptoms. Although the nerve conduction indices at 59 years of age deteriorated, those at 68, 72, and 73 years of age showed a gradual recovery...
February 28, 2018: Internal Medicine
Gwendal Le Masson, Guilhem Solé, Claude Desnuelle, Emilien Delmont, Marc Gauthier-Darnis, Sophie Puget, Isabelle Durand-Zaleski
Background: Prior clinical trials have suggested that home-based Ig treatment in multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and its variant Lewis-Sumner syndrome (LSS) is safe and effective and is less costly than hospital-administered intravenous immunoglobulin (IVIg). Methods: A French prospective, dual-center, cost minimization analysis was carried out to evaluate IVIg administration (5% concentrated) at home versus in hospital with regard to costs, patients' autonomy, and patients' quality of life...
February 2018: Brain and Behavior
Oliver Findling, Rens van der Logt, Krassen Nedeltchev, Lutz Achtnichts, John H J Allum
INTRODUCTION: We compared changes in balance control due to chronic inflammatory demyelinating polyneuropathy (CIDP) and non-inflammatory (non-inf) polyneuropathy (PNP) to each other and with respect to healthy controls (HCs). Differences in patients' subjective impressions of balance capabilities were also compared. METHODS: Balance control of 11 CIDP patients (mean age 61.1±(sd) 11, 8 male) and 10 non-inf PNP patients (mean age 68.5±11.7, all male) was examined and compared to that of 18 age- and gender-matched healthy controls...
2018: PloS One
Hillary Elizabeth Lockemer, Kathryn Maria Sumpter, Sandy Cope-Yokoyama, Abhimanyu Garg
BACKGROUND: Acquired lipodystrophy, craniopharyngioma and chronic inflammatory demyelinating polyneuropathy (CIDP) are individually rare disorders, and have never before been reported in a single patient. CASE PRESENTATION: A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year. He was diagnosed with CIDP, craniopharyngioma and acquired lipodystrophy. The patient underwent tumor debulking and cranial irradiation for the craniopharyngioma, and received monthly intravenous immunoglobulin for the CIDP...
February 19, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
Florian Härtig, Marlene Ross, Nele Maria Dammeier, Nadin Fedtke, Bianka Heiling, Hubertus Axer, Bernhard F Décard, Eva Auffenberg, Marilin Koch, Tim W Rattay, Markus Krumbholz, Antje Bornemann, Holger Lerche, Natalie Winter, Alexander Grimm
As reliable biomarkers of disease activity are lacking, monitoring of therapeutic response in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) remains a challenge. We sought to determine whether nerve ultrasound and electrophysiology scoring could close this gap. In CIDP patients (fulfilling EFNS/PNS criteria), we performed high-resolution nerve ultrasound to determine ultrasound pattern sum scores (UPSS) and predominant echotexture nerve conduction study scores (NCSS) as well as Medical Research Council sum scores (MRCSS) and inflammatory neuropathy cause and treatment disability scores (INCAT) at baseline and after 12 months of standard treatment...
February 12, 2018: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Xiaolei Liu, Roi Treister, Magdalena Lang, Anne Louise Oaklander
Objectives: Small-fiber polyneuropathy (SFPN) has various underlying causes, including associations with systemic autoimmune conditions. We have proposed a new cause; small-fiber-targeting autoimmune diseases akin to Guillain-Barré and chronic inflammatory demyelinating polyneuropathy (CIDP). There are no treatment studies yet for this 'apparently autoimmune SFPN' (aaSFPN), but intravenous immunoglobulin (IVIg), first-line for Guillain-Barré and CIDP, is prescribed off-label for aaSFPN despite very high cost...
2018: Therapeutic Advances in Neurological Disorders
Fu Liong Hiew, Jun-Jean Ong, Shanthi Viswanathan, Santhi Puvanarajah
Long-term outcome in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is very limited, especially from Asian countries. We aimed to determine the outcome of our cohort of CIDP patients and to define the relevant clinical, electrophysiological and laboratory determinants of disease activity, progression and treatment response. We retrospectively reviewed records of 23 CIDP patients attending our Neurology service at Kuala Lumpur Hospital, Malaysia between January 2000 and December 2016. We analysed data on neurological deficits, electrophysiological and laboratory parameters to determine diagnostic characteristics, correlation with disease activity and clinical outcomes following treatment...
February 2, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Naoyuki Shiraishi, Makoto Kinoshita, Mikito Shimizu, Hiroyuki Sumikura, Kei Fukada
At 37 years of age, the patient initially presented with symptoms of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) during her 1st pregnancy. She was treated with intravenous immunoglobulin (IVIg), and showed favorable recovery, becoming almost asymptomatic by the age of 38. At 39 years of age, during her puerperal period of her second pregnancy, she developed symmetrical muscle weakness and sensory disturbance of the upper and lower limbs. Nerve conduction studies revealed diffuse demyelination of peripheral nerves, and she was diagnosed with recurrence of CIDP...
January 31, 2018: Rinshō Shinkeigaku, Clinical Neurology
Thorsten Lichtenstein, Alina Sprenger, Kilian Weiss, Karin Slebocki, Barbara Cervantes, Dimitrios Karampinos, David Maintz, Gereon R Fink, Tobias D Henning, Helmar C Lehmann
Objective: To evaluate the utility of nerve diffusion tensor imaging (DTI), nerve cross-sectional area, and muscle magnetic resonance imaging (MRI) multiecho Dixon for assessing proximal nerve injury in chronic inflammatory demyelinating polyneuropathy (CIDP). Methods: In this prospective observational cohort study, 11 patients with CIDP and 11 healthy controls underwent a multiparametric MRI protocol with DTI of the sciatic nerve and assessment of muscle proton-density fat fraction of the biceps femoris and the quadriceps femoris muscles by multiecho Dixon MRI...
January 2018: Annals of Clinical and Translational Neurology
Collin-Jamal Smith, Denise E Allard, Yan Wang, James F Howard, Stephanie A Montgomery, Maureen A Su
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a debilitating condition caused by autoimmune demyelination of peripheral nerves. CIDP is associated with increased IL-10, a cytokine with well-described anti-inflammatory effects. However, the role of IL-10 in CIDP is unclear. In this study, we demonstrate that IL-10 paradoxically exacerbates autoimmunity against peripheral nerves. In IL-10-deficient mice, protection from neuropathy was associated with an accrual of highly activated CD4+ T cells in draining lymph nodes and absence of infiltrating immune cells in peripheral nerves...
January 24, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Aleksandra Kacar, Bogdan Bjelica, Ivo Bozovic, Stojan Peric, Ana Nikolic, Mina Cobeljic, Milutin Petrovic, Aleksandar Stojanov, Gordana Djordjevic, Zoran Vukojevic, Aleksandra Dominovic-Kovacevic, Miroslav Stojanovic, Zorica Stevic, Vidosava Rakocevic-Stojanovic, Dragana Lavrnic, Ivana Basta
To date, generic questionnaires have been used to investigate quality of life (QoL) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients. Although these measures are very useful, they are not usually precise enough to measure all specific characteristics of the disease. Our aim was to investigate QoL using the neuromuscular disease-specific questionnaire (INQoL) in a large cohort of patients with CIDP. Our study comprised 106 patients diagnosed with CIDP. INQoL questionnaire, MRC sum score, INCAT disability score, Visual Analogue Pain Scale, Beck Depression Inventory and Krupp's Fatigue Severity Scale were used in our study...
January 23, 2018: Journal of the Peripheral Nervous System: JPNS
Ilse M Lucke, Stojan Peric, Gwen G A van Lieverloo, Luuk Wieske, Camiel Verhamme, Ivo N van Schaik, Ivana Basta, Filip Eftimov
Cerebrospinal fluid (CSF) examination is often part of the diagnostic work-up of a patient suspected of having chronic inflammatory demyelinating polyneuropathy (CIDP). According to the EFNS/PNS criteria, an elevated protein level without pleocytosis (leukocytes <10 cells/μl) is supportive of the diagnosis CIDP. It is unclear how many CSF leukocytes are compatible with the diagnosis CIDP and how extensive the diagnostic work-up should be in patients with a demyelinating neuropathy and pleocytosis. We performed a retrospective study at two tertiary neuromuscular referral clinics and identified 14 out of 273 (6%) patients with CIDP with elevated CSF leukocytes (≥10 cells/μl)...
January 17, 2018: Journal of the Peripheral Nervous System: JPNS
Juliane Klehmet, Stefanie Märschenz, Klemens Ruprecht, Benjamin Wunderlich, Thomas Büttner, Rico Hiemann, Dirk Roggenbuck, Andreas Meisel
BACKGROUND: Unlike for acute immune-mediated neuropathies (IN), anti-ganglioside autoantibody (aGAAb) testing has been recommended for only a minority of chronic IN yet. Thus, we used a multiplex semi-quantitative line immunoassay (LIA) to search for aGAAb in chronic-inflammatory demyelinating polyneuropathy (CIDP) and its clinical variants. METHODS: Anti-GAAb to 11 gangliosides and sulfatide (SF) were investigated by LIA in 61 patients with IN (27 typical CIDP, 12 distal-acquired demyelinating polyneuropathy, 6 multifocal-acquired demyelinating sensory/motor polyneuropathy, 10 sensory CIDP, 1 focal CIDP and 5 multifocal-motoric neuropathy), 40 with other neuromuscular disorders (OND) (15 non-immune polyneuropathies, 25 myasthenia gravis), 29 with multiple sclerosis (MS) and 54 healthy controls (HC)...
January 12, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
Benjamin R Wakerley, Nobuhiro Yuki
No abstract text is available yet for this article.
January 12, 2018: Nature Reviews. Neurology
Filip Eftimov, Carina Bunschoten, Yusuf Rajabally, Luis Querol
No abstract text is available yet for this article.
February 2018: Neuromuscular Disorders: NMD
Gülden Akdal, Tural Tanrıverdizade, İhsan Şengün, Fikret Bademkıran, Koray Koçoğlu, Ayşe Nur Yüceyar, Özgül Ekmekçi, Hatice Karasoy, G Michael Halmágyi
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a common, treatable, autoimmune peripheral neuropathy considered to produce imbalance by weakness and proprioceptive impairment rather than vestibular impairment. We measured semicircular canal vestibular function in 21 CIDP patients (15M/6F) by the video head impulse test and postural stability with a battery comprising the modified Clinical Test of Sensory Integration and Balance, the Berg Balance Scale, the Dynamic Gait Index, the Fall Efficiency Scale, and the International Cooperative Ataxia Rating Scale...
February 2018: Journal of Neurology
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