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https://www.readbyqxmd.com/read/28796305/electron-microscopic-abnormality-and-therapeutic-efficacy-in-chronic-inflammatory-demyelinating-polyneuropathy-with-anti-neurofascin155-igg4-antibody
#1
Motoi Kuwahara, Hidekazu Suzuki, Nobuyuki Oka, Hidenori Ogata, Satoshi Yanagimoto, Shuji Sadakane, Yuta Fukumoto, Masaki Yamana, Yoshiko Yuhara, Keisuke Yoshikawa, Miyuki Morikawa, Shigeru Kawai, Masahiro Okazaki, Toru Tsujimoto, Jun-Ichi Kira, Susumu Kusunoki
INTRODUCTION: Neurofascin155 (NF155) is a target antigen for autoantibodies in a subset of chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: We report the cases of 4 patients with anti-NF155 IgG4 antibody-positive CIDP who underwent sural nerve biopsies. RESULTS: All patients were relatively young at onset. Three experienced tremors and 2 had severe ataxia. Although the response to intravenous immunoglobulin (IVIg) was poor in all patients, plasma exchange (PE) and corticosteroids were at least partially effective...
August 10, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28770373/early-predictive-factors-of-disability-in-cidp
#2
Emanuele Spina, Antonietta Topa, Rosa Iodice, Stefano Tozza, Lucia Ruggiero, Raffaele Dubbioso, Marcello Esposito, Dario Bruzzese, Lucio Santoro, Fiore Manganelli
The objective of this study was to identify early clinical, biochemical and electrophysiological prognostic factors of disability in CIDP. We evaluated a dataset from 60 CIDP patients that included sex, age of onset, type of onset, phenotype, disease duration, response to treatment, disability at the time of diagnosis assessed using the modified Rankin Scale (baseline mRS), cerebrospinal fluid protein levels and electrophysiological data. All patients had clinical assessment of disability through the mRS within the last 6 months (last mRS) before enrollment in the study...
August 2, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28768822/intravenous-immunoglobulin-for-maintenance-treatment-of-chronic-inflammatory-demyelinating-polyneuropathy-a-multicentre-open-label-52-week-phase-iii-trial
#3
Satoshi Kuwabara, Masahiro Mori, Sonoko Misawa, Miki Suzuki, Kazutoshi Nishiyama, Tatsuro Mutoh, Shizuki Doi, Norito Kokubun, Mikiko Kamijo, Hiroo Yoshikawa, Koji Abe, Yoshihiko Nishida, Kazumasa Okada, Kenji Sekiguchi, Ko Sakamoto, Susumu Kusunoki, Gen Sobue, Ryuji Kaji
OBJECTIVE: Short-term efficacy of induction therapy with intravenous immunoglobulin (Ig) in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) is well established. However, data of previous studies on maintenance therapy were limited up to 24-week treatment period. We aimed to investigate the efficacy and safety of longer-term intravenous Ig therapy for 52 weeks. METHODS: This study was an open-label phase 3 clinical trial conducted in 49 Japanese tertiary centres...
August 2, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28763304/chronic-inflammatory-demyelinating-polyneuropathy
#4
Richard A Lewis
PURPOSE OF REVIEW: As a syndrome with typical and atypical cases, chronic inflammatory demyelinating polyneuropathy (CIDP) has been a difficult disorder to diagnose and treat. The pathophysiologic basis for CIDP has not been established, contributing to the challenges in dealing with these patients. However, as one of only a handful of treatable peripheral neuropathies, there has been a tendency to diagnose CIDP to attempt a therapeutic intervention. We are also aware that there has also been overtreatment of some patients...
July 29, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28762574/a-comparative-study-of-brachial-plexus-sonography-and-magnetic-resonance-imaging-in-chronic-inflammatory-demyelinating-neuropathy-and-multifocal-motor-neuropathy
#5
H S Goedee, B A Jongbloed, J-T H van Asseldonk, J Hendrikse, A F J E Vrancken, H Franssen, S Nikolakopoulos, L H Visser, W L van der Pol, L H van den Berg
BACKGROUND AND PURPOSE: To compare the performance of neuroimaging techniques, i.e. high-resolution ultrasound (HRUS) and magnetic resonance imaging (MRI), when applied to the brachial plexus, as part of the diagnostic work-up of chronic inflammatory demyelinating neuropathy (CIDP) and multifocal motor neuropathy (MMN). METHODS: Fifty-one incident, treatment-naive patients with CIDP (n = 23) or MMN (n = 28) underwent imaging of the brachial plexus using (i) a standardized MRI protocol to assess enlargement or T2 hyperintensity and (ii) bilateral HRUS to determine the extent of nerve (root) enlargement...
August 1, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28747620/chronic-inflammatory-demyelinating-polyneuropathy-variant-with-creatine-kinase-elevation-and-vanishing-effect-of-immunoglobulins
#6
Josef Finsterer, Rahim Aliyev
BACKGROUND Whether creatine-kinase (CK) is elevated or not in chronic inflammatory demyelinating polyneuropathy (CIDP) and its variants is not comprehensively investigated. CASE REPORT We report the case of a 47-year-old male who developed weakness of the left lower leg and the right index finger at age 42 years. At age 44 years, paresthesias and dysesthesias of both lower legs and mild right lower leg weakness additionally developed. CK was recurrently elevated since age 42 years but paraprotein and anti-myelin-associated glycoprotein (MAG)-antibodies were negative...
July 27, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28708133/autoantibodies-in-chronic-inflammatory-neuropathies-diagnostic-and-therapeutic-implications
#7
REVIEW
Luis Querol, Jérôme Devaux, Ricard Rojas-Garcia, Isabel Illa
The chronic inflammatory neuropathies (CINs) are rare, very disabling autoimmune disorders that generally respond well to immune therapies such as intravenous immunoglobulin (IVIg). The most common forms of CIN are chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy, and polyneuropathy associated with monoclonal gammopathy of unknown significance. The field of CIN has undergone a major advance with the identification of IgG4 autoantibodies directed against paranodal proteins in patients with CIDP...
July 14, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28668438/lumbar-plexus-in-patients-with-chronic-inflammatory-demyelinating-polyneuropathy-evaluation-with-3d-nerve-sheath-signal-increased-with-inked-rest-tissue-rapid-acquisition-of-relaxation-enhancement-imaging-3d-shinkei
#8
Akio Hiwatashi, Osamu Togao, Koji Yamashita, Kazufumi Kikuchi, Ryotato Kamei, Daichi Momosaka, Hidenori Ogata, Ryo Yamasaki, Masami Yoneyama, Jun-Ichi Kira, Hiroshi Honda
PURPOSE: To evaluate whether 3D SHINKEI in the lumbar plexus could identify patients with chronic inflammatory demyelinating polyneuropathy (CIDP). MATERIALS AND METHODS: Twenty-one patients with CIDP and 15 non-CIDP patients were studied in this retrospective study. The SNR, contrast-to-noise ratio (CNR), contrast ratio (CR) and the size of the lumbar ganglions and roots were measured. Statistical analyses were performed with Mann-Whitney U test and receiver operating characteristics (ROC) analysis...
August 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28668085/utility-of-somatosensory-evoked-potentials-in-the-assessment-of-response-to-ivig-in-a-long-lasting-case-of-chronic-immune-sensory-polyradiculopathy
#9
Angelo Maurizio Clerici, Eduardo Nobile-Orazio, Marco Mauri, Federico Sergio Squellati, Giorgio Giovanni Bono
BACKGROUND: Chronic immune sensory polyradiculopathy (CISP) identifies a progressive acquired peripheral dysimmune neuropathy recognized as a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant. We describe a young woman with a thirteen-year history of CISP with a belated variable response to intravenous immunoglobulin (IVIG) and an almost erratic anticipation of symptoms between IVIG cycles. The association of IVIG and corticosteroids, immunosuppressants, plasmapheresis, did not lead to clinical improvement and was characterized by significant side effects...
July 1, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28667663/neuromuscular-ultrasound-findings-in-dads-variant-of-cidp
#10
Quang D Vu, Michael S Cartwright
No abstract text is available yet for this article.
July 1, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28646568/blink-r1-latency-utility-in-diagnosis-and-treatment-assessment-of-poems-and-cidp
#11
Wei Wang, William J Litchy, Michelle L Mauermann, P James B Dyck, Angela Dispenzieri, Jay Mandrekar, Peter J Dyck, Christopher J Klein
INTRODUCTION: In POEMS and CIDP, limb nerve conduction studies (NCS) are limited in identifying demyelination and detecting treatment effects in severely affected patients. Blink R1-latency may improve these assessments. METHODS: POEMS and CIDP patients having undergone NCS and blink reflex were identified. Correlations between R1-latency, limb NCS and neuropathy impairment scores (NIS) were compared. RESULTS: With182 patients (124 POEMS, 58 CIDP) identified, R1-prolongation (>13ms) occurred in 64...
June 23, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28620719/large-coverage-mr-neurography-in-cidp-diagnostic-accuracy-and-electrophysiological-correlation
#12
Moritz Kronlage, Philipp Bäumer, Kalliopi Pitarokoili, Daniel Schwarz, Véronique Schwehr, Tim Godel, Sabine Heiland, Ralf Gold, Martin Bendszus, Min-Suk Yoon
The objective of this study was to evaluate large coverage magnetic resonance neurography (MRN) in chronic inflammatory demyelinating polyneuropathy (CIDP). In this prospective study, 18 patients with CIDP and 18 healthy controls were examined by a standardized MRN protocol at 3 T. Lumbosacral plexus was imaged by a T2-weighted 3D sequence and peripheral nerves of the upper and lower extremity by axial T2-weighted turbo spin-echo sequences. Lesions were characterized by nerve cross-sectional area (CSA) and T2-weighted signal (nT2)...
July 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28601286/autoantibody-responses-to-nodal-and-paranodal-antigens-in-chronic-inflammatory-neuropathies
#13
E K Mathey, N Garg, S B Park, T Nguyen, S Baker, N Yuki, C Yiannikas, C S Lin, J M Spies, R Ghaoui, M H Barnett, S Vucic, J D Pollard, M C Kiernan
Autoantibodies to nodal/paranodal proteins have been reported in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). To determine the frequency of anti-paranodal antibodies in our cohort of CIDP patients and to validate the presence anti-nodal antibodies in MMN, sera were screened for IgG against human neurofascin 155, contactin-1, neurofascin 186 and gliomedin using ELISA. In CIDP patients, 7% were anti-NF155 IgG4 positive and 7% were anti-CNTN1 IgG4 positive...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28597710/comparing-treatment-options-for-chronic-inflammatory-neuropathies-and-choosing-the-right-treatment-plan
#14
Eduardo Nobile-Orazio, Francesca Gallia, Fabrizia Terenghi, Mariangela Bianco
Chronic inflammatory neuropathies are disorders caused by an immune response to peripheral nerve. They include chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN) and neuropathy associated with anti-MAG IgM monoclonal gammopathy and other less frequent neuropathies. Several immune therapies have been proven to be effective in these neuropathies even if the best therapeutic option is still unsettled. Areas covered: The authors reviewed the literature to compare the efficacy and safety of currently used immune therapies in these neuropathies...
June 26, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28576432/anticeramide-antibody-and-butyrylcholinesterase-in-peripheral-neuropathies
#15
Aparna Sykam, V R Gutlapalli, Sandeep P Tenali, A K Meena, Priscilla Chandran, Sujai Suneetha, Lavanya M Suneetha
Ceramide is a glycosphingolipid, a component of nerve and non neuronal cell membrane and plays a role in maintaining the integrity of neuronal tissue. Butyrylcholinesterase (BChE) is a multifunctional enzyme, its involvement in neurodegenerative diseases has been well established. Anticeramide antibody (Ab-Cer) and enzyme BChE have been implicated in peripheral neuropathies. The present study investigates whether there is an association between Ab-Cer and BChE activities and peripheral neuropathies. Patients included: human immunodeficiency virus associated peripheral neuropathy (HIV-PN, n=39), paucibacillary leprosy (PB-L, n=36), multibacillary leprosy (MB-L, n=52), diabetic neuropathy (DN, n=22), demyelinating sensory motor polyneuropathy (DSMN, n=13) and chronic inflammatory demyelinating polyneuropathy (CIDP, n=10)...
August 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28574858/diffusion-tensor-imaging-in-chronic-inflammatory-demyelinating-polyneuropathy-diagnostic-accuracy-and-correlation-with-electrophysiology
#16
Moritz Kronlage, Kalliopi Pitarokoili, Daniel Schwarz, Tim Godel, Sabine Heiland, Min-Suk Yoon, Martin Bendszus, Philipp Bäumer
PURPOSE: The aims of this study were to assess diagnostic accuracy of diffusion tensor imaging (DTI) in chronic inflammatory demyelinating polyneuropathy (CIDP), to correlate DTI with electrophysiological parameters, and to evaluate whether radial diffusivity (RD) and axial diffusivity (AD) might serve as specific biomarkers of demyelinating and axonal pathology. MATERIALS AND METHODS: This prospective study was approved by the institutional ethics committee, and written informed consent was obtained from all participants...
June 1, 2017: Investigative Radiology
https://www.readbyqxmd.com/read/28570324/neurologic-complications-of-lymphoma-leukemia-and-paraproteinemias
#17
Michelle L Mauermann
PURPOSE OF REVIEW: This article reviews the spectrum of neurologic complications associated with lymphoma, leukemia, and paraproteinemic disorders. While leptomeningeal metastasis is the most common complication of lymphoma and leukemia and peripheral neuropathy is the most common complication of paraproteinemic disorders, clinicians need to be familiar with the diverse neurologic complications of these disorders. RECENT FINDINGS: Lymphomatous nervous system involvement can be difficult to diagnose, especially when it is the presenting symptom...
June 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28566163/subcutaneous-immunoglobulin-treatment-in-cidp-and-mmn-efficacy-treatment-satisfaction-and-costs
#18
REVIEW
Lars H Markvardsen, Thomas Harbo
Subcutaneous administration of immunoglobulin (SCIG) in chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) has been reported in several case reports and in a few randomized trials during the last decade. In this review we present the studies on SCIG in CIDP and MMN with special focus on the clinical effects. Moreover, the effect on quality of life, side effects to SCIG and the health economic perspectives are reviewed. Nine case studies, three randomized trials and six long-term, follow-up studies were identified...
July 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28566158/diagnostic-challenges-in-poems-syndrome-presenting-with-polyneuropathy-a-case-series
#19
Yuebing Li, Jason Valent, Payam Soltanzadeh, Nimish Thakore, Bashar Katirji
OBJECTIVE: To analyze the clinical characteristics of patients with POEMS presenting with polyneuropathy and discuss associated diagnostic challenges. METHOD: Retrospective analysis of 6 patients from 2 tertiary-care institutions. RESULTS: Six patients presented with progressive sensorimotor deficits predominantly or exclusively in the lower extremities and were diagnosed with POEMS syndrome. In 4 patients, a diagnosis of chronic inflammatory demyelinating polyradiculopathy (CIDP) was mistakenly made...
July 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28523213/brachial-plexus-magnetic-resonance-imaging-differentiates-between-inflammatory-neuropathies-and-does-not-predict-disease-course
#20
Bas A Jongbloed, Jeroen W Bos, Dirk Rutgers, Willem Ludo van der Pol, Leonard H van den Berg
OBJECTIVE: The main objective of this study was to evaluate the correlation between the distribution of brachial plexus magnetic resonance imaging (MRI) abnormalities and clinical weakness, and to evaluate the value of brachial plexus MRI in predicting disease course and response to treatment in multifocal motor neuropathy (MMN), Lewis-Sumner syndrome (LSS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: Sixty-seven patients with an inflammatory neuropathy diagnosed at our tertiary referral center for neuromuscular diseases had undergone bilateral T2-weighted short tau inversion recovery (STIR) MRI of the brachial plexus...
May 2017: Brain and Behavior
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