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Demyelinating polyneuropathy

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https://www.readbyqxmd.com/read/28194064/therapeutic-plasma-exchange-in-patients-with-neurologic-disorders-review-of-63-cases
#1
Anil Tombak, Mehmet Ali Uçar, Aydan Akdeniz, Arda Yilmaz, Hakan Kaleagası, Mehmet Ali Sungur, Eyup Naci Tiftik
Therapeutic plasma exchange (TPE) is a procedure that reduces circulating autoantibodies of the patients. TPE is commonly used in neurological disorders where autoimmunity plays a major role. We report our experience with regard to the indications, adverse events and outcomes of plasma exchange in neurological disorders. Sixty-three patients were included to this retrospective study. Median age was 48 years (range 1-85), there was a predominance of males. Neurological indications included Guillain-Barrè syndrome (n = 22), myasthenia gravis (n = 21), chronic inflammatory demyelinating polyneuropathy (n = 7), polymyositis (n = 3), multifocal motor neuropathy (n = 2), acute disseminated encephalomyelitis (n = 2), neuromyelitis optica (n = 2), multiple sclerosis (n = 2), limbic encephalitis (n = 1) and transverse myelitis (n = 1)...
March 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28163843/outcome-of-guillain-barr%C3%A3-syndrome-in-children-a-prospective-cohort-study-in-a-tertiary-hospital-in-upper-egypt
#2
Abdelrahim Abdrabou Sadek, Ashraf Abou-Taleb, Wafaa Ahmed Ali
INTRODUCTION: Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, and defined as an acute inflammatory polyneuropathy. The objective of this study was to assess the clinico-laboratory profile, and outcome of Guillain-Barré syndrome in children at Sohag University Hospital. METHODS: This prospective cohort observational study was conducted in 2014-2015. The included children were subjected to through medical history and detailed systemic and neurological examination...
December 2016: Electronic Physician
https://www.readbyqxmd.com/read/28131554/-hereditary-neuropathy-with-liability-to-pressure-palsies-in-childhood-report-of-three-cases
#3
C Bar, F Villéga, C Espil, M Husson, J-M Pedespan, M-F Rouanet
Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant neuropathy. It is characterized by recurrent sensory and motor nerve palsies, usually precipitated by minor trauma or compression. Even though rare in childhood, this disorder is probably underdiagnosed given its wide spectrum of clinical symptoms. We review three separate cases of HNPP diagnosed in children with various phenotypes: fluctuating and distal paresthesias disrupting learning at school, cramps related to intensive piano practice, and discrete muscle weakness with no functional complaint...
January 25, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28108521/nodopathy-chronic-inflammatory-demyelinating-polyneuropathy-with-anti-neurofascin-155-antibodies
#4
EDITORIAL
Satoshi Kuwabara, Sonoko Misawa, Masahiro Mori
No abstract text is available yet for this article.
January 20, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28073817/paranodal-dissection-in-chronic-inflammatory-demyelinating-polyneuropathy-with-antineurofascin-155-and-anticontactin-1-antibodies
#5
Haruki Koike, Masato Kadoya, Ken-Ichi Kaida, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Daisuke Kato, Hidenori Ogata, Ryo Yamasaki, Noriyuki Matsukawa, Jun-Ichi Kira, Masahisa Katsuno, Gen Sobue
OBJECTIVE: To investigate the morphological features of chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies directed against paranodal junctional molecules, particularly focusing on the fine structures of the paranodes. METHODS: We assessed sural nerve biopsy specimens obtained from 9 patients with CIDP with antineurofascin-155 antibodies and 1 patient with anticontactin-1 antibodies. 13 patients with CIDP without these antibodies were also examined to compare pathological findings...
January 10, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28073143/demyelinating-polyneuropathy-in-a-case-of-anti-lgi1-encephalitis
#6
Gemma Tumminelli, Carla Battisti, Chiara Cioni, Andrea Mignarri, Pasquale Annunziata, And Antonio Federico
No abstract text is available yet for this article.
January 10, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28054000/a-comparative-study-of-cidp-in-a-cohort-of-hiv-infected-and-hiv-uninfected-patients
#7
Kaminie Moodley, Pierre L A Bill, Vinod Bhagu Patel
OBJECTIVE: To investigate differences in clinical presentation, electrodiagnostic measures, CSF changes, and treatment outcome measures in HIV-infected and HIV-uninfected patients with chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: A retrospective analysis of medical records of all patients meeting the European Federation of Neurology diagnostic criteria for idiopathic CIDP was performed in 2 neuromuscular units in Kwa-Zulu Natal between 2003 and 2015...
March 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28049985/demyelinating-peripheral-neuropathy-due-to-renal-cell-carcinoma
#8
Kenya Nishioka, Motoki Fujimaki, Kazuaki Kanai, Yuta Ishiguro, Tomoko Nakazato, Ryota Tanaka, Kazumasa Yokoyama, Nobutaka Hattori
Renal cell carcinoma (RCC) patients who develop a paraneoplastic syndrome may present with neuromuscular disorders. We herein report the case of a 50-year-old man who suffered from progressive gait disturbance and muscle weakness. The results of a nerve conduction study fulfilled the criteria of chronic inflammatory demyelinating polyneuropathy. An abdominal CT scan detected RCC, the pathological diagnosis of which was clear cell type. After tumor resection and a single course of intravenous immunoglobulin therapy, the patient's symptoms drastically improved over the course of one year...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28025411/a-case-of-anti-neurofascin-155-antibody-positive-combined-central-and-peripheral-demyelination-successfully-treated-with-plasma-exchange
#9
Mikito Shimizu, Tohru Koda, Yuji Nakatsuji, Hidenori Ogata, Jun-Ichi Kira, Hideki Mochizuki
A 21-year-old man was admitted to our hospital in June 2015. He felt paresthesia of toes in April 2015, which had been spreading upward, and he became difficult to walk in June. Nerve conduction study showed peripheral demyelinating neuropathy that met the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP), and the cerebrospinal fluid (CSF) examination revealed the remarkably increased protein level. In addition, magnetic resonance imaging of his brain showed a few plaques in white matter, so he was finally diagnosed with combined central and peripheral demyelination (CCPD)...
December 23, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28018472/concurrency-of-guillain-barre-syndrome-and-acute-transverse-myelitis-a-case-report-and-review-of-literature
#10
Orkun Tolunay, Tamer Çelik, Ümit Çelik, Mustafa Kömür, Zeynep Tanyeli, Abdurrahman Sönmezler
Guillain-Barré syndrome and acute transverse myelitis manifest as demyelinating diseases of the peripheral and central nervous system. Concurrency of these two disorders is rarely documented in literature. A 4-year-old girl presenting with cough, fever, and an impaired walking ability was admitted to hospital. She had no previous complaints in her medical history. A physical examination revealed lack of muscle strength of the lower extremities and deep tendon reflexes. MRI could not be carried out due to technical problems; therefore, both Guillain-Barré syndrome and acute transverse myelitis were considered for the diagnosis...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28017218/clinical-characteristics-and-impairment-and-disability-scale-scores-for-different-cidp-disease-activity-status-classes
#11
Majed Alabdali, Alon Abraham, Abdulla Alsulaiman, Ari Breiner, Carolina Barnett, Hans D Katzberg, Leif E Lovblom, Bruce A Perkins, Vera Bril
INTRODUCTION: Selecting proper outcome measures is important for clinical practice and clinical studies assessing treatable neuropathies, such as chronic inflammatory demyelinating polyneuropathy (CIDP). OBJECTIVES: To explore clinical characteristics and impairment and disability scores in CIDP patients, and assess their relationship to different CIDP Disease Activity Status (CDAS) classes. METHODS: We performed a retrospective chart review of CIDP patients attending the neuromuscular clinic between January 2014 and December 2015, and explored clinical characteristics, and various impairment and disability scores for different CDAS classes...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28010677/cochlear-implantation-in-chronic-demyelinating-inflammatory-polyneuropathy
#12
Sarah E Mowry, Sarah King
OBJECTIVE: To describe a case of chronic inflammatory demyelinating polyneuropathy (CDIP) with bilateral sudden sensorineural hearing loss who subsequently benefited from unilateral cochlear implantation. METHODS: case history review and review of the literature for the terms CDIP, hearing loss, cochleovestibular dysfunction, and cochlear implantation. RESULTS: A 49-year-old woman presented with bilateral rapidly progressive sensorineural hearing loss (SNHL) 1 month after an upper respiratory tract infection...
December 23, 2016: Cochlear Implants International
https://www.readbyqxmd.com/read/28003344/differentiating-lower-motor-neuron-syndromes
#13
REVIEW
Nidhi Garg, Susanna B Park, Steve Vucic, Con Yiannikas, Judy Spies, James Howells, William Huynh, José M Matamala, Arun V Krishnan, John D Pollard, David R Cornblath, Mary M Reilly, Matthew C Kiernan
Lower motor neuron (LMN) syndromes typically present with muscle wasting and weakness and may arise from pathology affecting the distal motor nerve up to the level of the anterior horn cell. A variety of hereditary causes are recognised, including spinal muscular atrophy, distal hereditary motor neuropathy and LMN variants of familial motor neuron disease. Recent genetic advances have resulted in the identification of a variety of disease-causing mutations. Immune-mediated disorders, including multifocal motor neuropathy and variants of chronic inflammatory demyelinating polyneuropathy, account for a proportion of LMN presentations and are important to recognise, as effective treatments are available...
December 21, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28000311/subcutaneous-immunoglobulin-as-first-line-therapy-in-treatment-naive-patients-with-chronic-inflammatory-demyelinating-polyneuropathy-randomized-controlled-trial-study
#14
L H Markvardsen, S H Sindrup, I Christiansen, N K Olsen, J Jakobsen, H Andersen
BACKGROUND AND PURPOSE: Subcutaneous immunoglobulin (SCIG) is effective as maintenance treatment in chronic inflammatory demyelinating polyneuropathy (CIDP). We investigated whether multiple subcutaneous infusions are as effective as conventional therapy with intravenous loading doses in treatment-naive patients with CIDP. METHODS: Twenty patients fulfilling the clinical and electrophysiological criteria for CIDP were included and treated with either SCIG (0.4 g/kg/week) for 5 weeks or intravenous immunoglobulin (IVIG) (0...
February 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/27997686/electrophysiological-and-neuromuscular-stability-of-persons-with-chronic-inflammatory-demyelinating-polyneuropathy-cidp
#15
Kevin J Gilmore, Matti D Allen, Timothy J Doherty, Kurt Kimpinski, Charles L Rice
OBJECTIVE: We assessed motor unit (MU) properties and neuromuscular stability in the tibialis anterior (TA) of CIDP patients using decomposition-based quantitative electromyography (DQEMG). METHODS: Dorsiflexion strength was assessed, and surface and concentric needle electromyography (EMG) were sampled from the TA. Estimates of MU numbers were derived using DQEMG and spike-triggered averaging. Neuromuscular transmission stability was assessed from concentric needle-detected motor unit potentials (MUPs)...
December 20, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27986399/paranodal-lesions-in-chronic-inflammatory-demyelinating-polyneuropathy-associated-with-anti-neurofascin-155-antibodies
#16
Jean-Michel Vallat, Nobuhiro Yuki, Kenji Sekiguchi, Norito Kokubun, Nobuyuki Oka, Stéphane Mathis, Laurent Magy, Diane L Sherman, Peter J Brophy, Jérôme J Devaux
Antibodies to Contactin-1 and Neurofascin 155 (Nfasc155) have recently been associated with subsets of patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Contactin-1 and Nfasc155 are cell adhesion molecules that constitute the septate-like junctions observed by electron microscopy in the paranodes of myelinated axons. Antibodies to Contactin-1 have been shown to affect the localization of paranodal proteins both in patient nerve biopsies and in animal models after passive transfer. However, it is unclear whether these antibodies alter the paranodal ultrastructure...
October 24, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27974705/are-neurological-complications-of-monoclonal-gammopathy-of-undetermined-significance-underestimated
#17
Normann Steiner, Angelika Schwärzler, Georg Göbel, Wolfgang Löscher, Julia Wanschitz, Eberhard Gunsilius
OBJECTIVES: Monoclonal gammopathy of undetermined significance (MGUS) is a premalignancy preceding multiple myeloma (MM) or related disorders. Neurological symptoms caused by the monoclonal immunoglobulins or free light-chains are often associated with a high morbidity. We analyzed the prevalence of neuropathy, clinical features and the long-term outcome in 223 patients (pts.) with MGUS. PATIENTS AND METHODS: Between 1/2005 and 3/2015, 223 adult pts. with MGUS were identified in our database...
December 10, 2016: Oncotarget
https://www.readbyqxmd.com/read/27951607/-chronic-inflammatory-demyelinating-polyneuropathy
#18
M Balke, G Wunderlich, A Brunn, G R Fink, H C Lehmann
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic progressive or relapsing autoimmune neuropathy with heterogeneous clinical presentation. Symptoms typically include symmetrical, proximal and/or distal paresis and sensory loss. Atypical CIDP variants are increasingly recognized, including subtypes with rapid onset as well as variants with pure sensory, focal or marked asymmetrical deficits. Diagnosis is established by compatible symptoms, characteristic electrophysiological features and cerebrospinal fluid analysis...
December 2016: Fortschritte der Neurologie-Psychiatrie
https://www.readbyqxmd.com/read/27939129/guillain-barr%C3%A3-syndrome-is-induced-in-non-obese-diabetic-nod-mice-following-campylobacter-jejuni-infection-and-is-exacerbated-by-antibiotics
#19
J L St Charles, J A Bell, B J Gadsden, A Malik, H Cooke, L K Van de Grift, H Y Kim, E J Smith, L S Mansfield
Campylobacter jejuni is a leading cause of bacterial gastroenteritis linked to several serious autoimmune sequelae such as the peripheral neuropathies Guillain Barré syndrome (GBS) and Miller Fisher syndrome (MFS). We hypothesized that GBS and MFS can result in NOD wild type (WT) mice or their congenic interleukin (IL)-10 or B7-2 knockouts secondary to C. jejuni infection. Mice were gavaged orally with C. jejuni strains HB93-13 and 260.94 from patients with GBS or CF93-6 from a patient with MFS and assessed for clinical neurological signs and phenotypes, anti-ganglioside antibodies, and cellular infiltrates and lesions in gut and peripheral nerve tissues...
February 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27931132/guillain-barr%C3%A3-syndrome-during-adalimumab-therapy-for-crohn%C3%A2-s-disease-coincidence-or-consequence
#20
Guilherme Grossi Lopes Cançado, Eduardo Garcia Vilela
We report the case of a 64-year-old patient diagnosed with extensive ileal Crohn´s disease who developed Guillain-Barré syndrome after starting biological therapy with adalimumab. Neurologic involvement associated with inflammatory bowel diseases is recognized as an extra-intestinal manifestation. After the breakthrough of antitumor necrosis factor alpha (anti-TNF-α) agents, an increasing number of cases of acute inflammatory demyelinating polyneuropathies have been reported; however, only one case has been described in a patient with Crohn´s disease...
April 2017: Scandinavian Journal of Gastroenterology
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