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Panagiotis Kokotis, Martin Schmelz, Aikaterini E Papagianni, Thomas Zambelis, Nikos Karandreas
INTRODUCTION: In chronic inflammatory demyelinating polyradiculopathy (CIDP) the impairment of unmyelinated nerve fibers appears unexpected. The measurement of the electrically induced axon flare reflex is a clinical test to assess the peripheral C-nociceptor function. In this study we compared the flare area in patients suffering from chronic inflammatory demyelinating polyradiculopathy with healthy subjects. MATERIALS AND METHODS: We examined 18 patients fulfilling the criteria for CIDP (11 male, mean age 51...
October 18, 2016: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
Gerd Meyer Zu Horste, Steffen Cordes, Johannes Pfaff, Christian Mathys, Anne K Mausberg, Martin Bendszus, Mirko Pham, Hans-Peter Hartung, Bernd C Kieseier
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disabling autoimmune disorder of the peripheral nervous system (PNS). Intravenous immunoglobulins (IVIg) are effective in CIDP, but the treatment response varies greatly between individual patients. Understanding this interindividual variability and predicting the response to IVIg constitute major clinical challenges in CIDP. We previously established intercellular adhesion molecule (ICAM)-1 deficient non-obese diabetic (NOD) mice as a novel animal model of CIDP...
2016: PloS One
Motomi Arai
This report concerns a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with frequent treatment-dependent relapses. A 75-year-old male presented with a 2-month history of progressive weakness of the limbs with no sensory symptoms. Neurological examination revealed normal cranial nerves, MRC grade 4 power in the proximal and distal muscles of the limbs, and generalized areflexia. However, the sensory examination results, serum immunoelectrophoresis, anti-HIV antibody, and vitamins B1 and B12 levels were normal...
September 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
Hiroyuki Naito, Ikuko Takeda, Akiko Segawa, Masataka Tsuge, Hirofumi Maruyama, Masayasu Matsumoto
We report the case of a 42-year-old man with chronic hepatitis B virus infection who developed weakness and paresthesia in the extremities 2 months after administration of pegylated interferon (Peg-IFN)α-2a. Nerve conduction studies conducted 6 months after onset showed abnormal temporal dispersions in both tibial nerves. We diagnosed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) resulting from treatment with Peg-IFNα-2a. Neurological symptoms were prolonged despite suspension of the treatment...
September 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
Abhijeet R Joshi, Laura Holtmann, Ilja Bobylev, Christian Schneider, Christian Ritter, Joachim Weis, Helmar C Lehmann
BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is often associated with chronic disability, which can be accounted to incomplete regeneration of injured axons. We hypothesized that Schwann cell support for regenerating axons may be altered in CIDP, which may account for the poor clinical recovery seen in many patients. METHODS: We exposed human and rodent Schwann cells to sera from CIDP patients and controls. In a model of chronic nerve denervation, we transplanted these conditioned Schwann cells intraneurally and assessed their capacity to support axonal regeneration by electrophysiology and morphometry...
September 27, 2016: Journal of Neuroinflammation
Anand M Navarasala, Vasilios Stambolis
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Vikram A Londhey
SLE (systemic lupus erythematosus) is a multisystem autoimmune disorder of unknown aetiology which can present with myriad clinical presentation. The neurological manifestations of SLE consist of central nervous system (CNS) and peripheral nervous system manifestations (PNS). The CNS manifestations are aseptic meningitis, cerebrovascular accidents (stroke), demyelinating disorders, headache, involuntary movements like chorea, myelopathy, acute confusional states, cognitive dysfunction, mood disorder, seizures, psychosis and cranial nerve palsies...
December 2015: Journal of the Association of Physicians of India
Juan M Racosta, Luciano A Sposato, Kurt Kimpinski
INTRODUCTION: High-dose intravenous immunoglobulin (IVIg) is an evidence-based treatment for multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyneuropathy (CIDP). Recently, subcutaneous immunoglobulin (SC-Ig) has received increasing attention. METHODS: We performed a meta-analysis of reports of efficacy and safety of SC-Ig versus IVIg for inflammatory demyelinating polyneuropathies. RESULTS: A total of 8 studies comprising 138 patients (50 with MMN and 88 with chronic CIDP) were included in the meta-analysis...
September 20, 2016: Muscle & Nerve
Isaak Quast, Christian W Keller, Falk Hiepe, Björn Tackenberg, Jan D Lünemann
Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune neuropathy. While both cell-mediated and humoral mechanisms contribute to its pathogenesis, the rapid clinical response to plasmapheresis implicates a circulating factor responsible for peripheral nerve injury. We report that treatment-naïve patients with CIDP show increased serum and CSF levels of the anaphylatoxin C5a and the soluble terminal complement complex (sTCC). Systemic terminal complement activation correlates with clinical disease severity as determined by the Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale...
September 2016: Annals of Clinical and Translational Neurology
R A C Hughes, M P T Lunn
Chronic inflammatory demyelinating polyradiculoneuropathy is an orphan disease of poorly understood cause. While first line treatments with corticosteroids, intravenous immunoglobulin and plasma exchange have at least short-term efficacy, no trial has shown that immunosuppressants work. In our dream, we will take advantage of the recently improved EU regulations to launch a Europe wide trial which will investigate the cause of the disease. It will compare three parallel groups, the anti-B cell agent rituximab, the anti-T cell agent abatacept and usual care...
October 2016: Revue Neurologique
Shintaro Hayashi, Shun Nagamine, Kouki Makioka, Susumu Kusunoki, Koichi Okamoto
A 71-year-old woman with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with IgA-λ monoclonal gammopathy of undetermined significance (MGUS) showed the acute development of tetraplegia, respiratory failure, and a marked fluctuation of the blood pressure. Intravenous (IV) high-dose steroid therapy (methylprednisolone: 1 g/day × 3 days), followed by oral prednisolone (PSL) (40 mg/day), and IV immunoglobulin (IVIg, 0.4 g/kg/day × 5 days) administrations resulted in the amelioration of these symptoms...
September 29, 2016: Rinshō Shinkeigaku, Clinical Neurology
Kazuhiro Itaya, Manabu Inoue, Natsuko Iizuka, Yuki Shimizu, Nobuhiro Yuki, Hiroo Ichikawa
A 17-year-old male with no medical history noticed weakness of his limbs with imbalance and subsequent finger tremors. Physical examination revealed features of polyneuropathy, including diffuse weakness, distal symmetrical numbness with impaired deep sensation and areflexia in all limbs. Postural tremor was present in fingers. Ataxia was apparent in both lower limbs, causing a wide-based gait with a positive Romberg sign. Cerebrospinal fluid contained elevated total protein without pleocytosis. A nerve conduction study disclosed demyelinating features with prolonged terminal latencies, slow velocities with delayed F-wave latencies, and prominent temporal dispersion...
September 29, 2016: Rinshō Shinkeigaku, Clinical Neurology
Tomomasa Ishikawa, Kunihiko Asakura, Yasuaki Mizutani, Akihiro Ueda, Ken-Ichiro Murate, Chika Hikichi, Sayuri Shima, Madoka Kizawa, Masako Komori, Kazuhiro Murayama, Hiroshi Toyama, Shinji Ito, Tatsuro Mutoh
INTRODUCTION: To visualize peripheral nerves in patients with chronic inflammatory demyelinating polyneuropathy (CIDP), we used magnetic resonance (MR) imaging. We also quantified the volumes of the brachial and lumbar plexus and their nerve roots. METHODS: Thirteen patients with CIDP and 12 healthy volunteers were enrolled. Whole-body MR neurography based on diffusion-weighted whole-body imaging with background body signal suppression (DWIBS) was performed. Peripheral nerve volumes were calculated from serial axial MR images...
August 8, 2016: Muscle & Nerve
G Stoll, K Reiners
The Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are the most common immune-mediated polyneuropathies, which can show variable clinical and electrophysiological manifestations. Rarer immune-mediated neuropathies encompass paraproteinemic neuropathies (PPN), multifocal motor neuropathy (MMN) and vasculitic neuropathies. The diagnosis usually relies on the history of symptom evolution, distribution of nerve dysfunction and particularly on characteristic features in nerve conduction studies, aided by cerebrospinal fluid (CSF) examination and nerve biopsy findings...
August 2016: Der Nervenarzt
Chenghe Fan, Haiqiang Jin, Hongjun Hao, Feng Gao, Yongan Sun, Yuanyuan Lu, Yuanyuan Liu, Pu Lv, Wei Cui, Yuming Teng, Yining Huang
INTRODUCTION: We investigated the relationships between anti-ganglioside antibodies and Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Samples from 48 patients diagnosed with GBS and 18 patients diagnosed with CIDP were retrospectively reviewed in China. RESULTS: In GBS patients, 62.5% were classified as having acute inflammatory demyelinating polyneuropathy (AIDP), 27.1% were found to have acute motor axonal neuropathy (AMAN), and 10...
July 27, 2016: Muscle & Nerve
Alexander Grimm, Victoria Schubert, Hubertus Axer, Ulf Ziemann
INTRODUCTION: Nerve enlargement (NE) is described in inflammatory and inherited neuropathies. It is commonly multifocal and moderate in the former and homogeneous and generalized in the latter. We describe 4 cases of massive NE in inflammatory neuropathies. METHODS: Patients presented with symptoms of polyneuropathy that progressed over months to years. Nerve conduction studies (NCS), laboratory analysis, nerve MRI, and nerve ultrasound were performed. RESULTS: NCS revealed demyelinating neuropathy in all with multifocal conduction blocks or increased terminal latency indices...
July 27, 2016: Muscle & Nerve
Artem Kaplan, Thomas H Brannagan
INTRODUCTION: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a common treatable neuropathy, however nearly 20% of patients remain refractory to standard therapy for unclear reasons. METHODS: We did a retrospective review of 37 patients referred for CIDP refractory to intravenous immunoglobulin (IVIg), plasmapheresis, and/or corticosteroids. Clinical findings, electrophysiological studies, and response to further therapeutic interventions were assessed...
July 27, 2016: Muscle & Nerve
Pierre R Bourque, Thanh B Nguyen, Jocelyn Zwicker, Jodi Warman Chardon
No abstract text is available yet for this article.
July 26, 2016: Neurology
Ivo N van Schaik, Nan van Geloven, Vera Bril, Hans-Peter Hartung, Richard A Lewis, Gen Sobue, John-Philip Lawo, Orell Mielke, David R Cornblath, Ingemar S J Merkies
BACKGROUND: Subcutaneous administration of Ig (SCIg) has gained popularity as an alternative route of administration but has never been rigorously examined in chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS/DESIGN: The primary objective of the PATH study (Polyneuropathy and Treatment with Hizentra) is to determine the efficacy of two different doses of SCIg IgPro20 (0.2 g/kg bw or 0.4 g/kg bw) in a 24-week maintenance treatment of CIDP in comparison to placebo...
2016: Trials
Kuo-Hsuan Chang, Rong-Kuo Lyu, Yen-Shi Ro, Yi-Chun Chen, Long-Sun Ro, Hong-Shiu Chang, Ching-Chang Huang, Ming-Feng Liao, Yih-Ru Wu, Hong-Chou Kuo, Chun-Che Chu, Chiung-Mei Chen
BACKGROUND: Guillain-Barré syndrome (GBS) is an acquired demyelinating peripheral neuropathy. It has shown that macrophage activation contribute to the pathogenesis of GBS. Therefore macrophage-mediated factors could be the potential markers for disease diagnosis and status of GBS. METHODS: We measured serum concentrations of 4 macrophage-mediated factors, including interleukin-6 (IL-6), transforming growth factor-β1 (TGF-β1), vascular cell adhesion protein 1 (VCAM-1) and vascular endothelial growth factor (VEGF), in 23 chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 28 GBS, 11 Miller-Fisher syndrome (MFS), 40 multiple sclerosis (MS), and 12 Alzheimer's disease (AD) patients, as well as 15 healthy controls...
October 1, 2016: Clinica Chimica Acta; International Journal of Clinical Chemistry
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