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Demyelinating polyneuropathy

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https://www.readbyqxmd.com/read/29164611/serial-electrophysiology-in-guillain-barr%C3%A3-syndrome-a-retrospective-cohort-and-case-by-case-multicentre-analysis
#1
J Ibrahim, A-M Grapperon, F Manfredonia, P Y van den Bergh, S Attarian, Y A Rajabally
OBJECTIVES: To assess the usefulness of serial electrophysiology in Guillain-Barré syndrome (GBS) in a multicenter setting and the reasons for change in electrodiagnostic subtypes with serial studies. METHODS: We retrospectively analysed serial electrophysiology of 51 patients with GBS from 4 European centres. Proportions of subtypes were determined at each timing. Individual case analyses were also performed where diagnostic changes occurred with either criteria, to ascertain if changes were due to disease progression or criteria inadequacy...
November 21, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29157616/the-peripheral-neuropathies-of-poems-syndrome-and-castleman-disease
#2
REVIEW
Michelle L Mauermann
Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome is a rare paraneoplastic disorder. The polyneuropathy can be the presenting symptom and is typically a painful, motor-predominant polyradiculoneuropathy often mimicking chronic inflammatory demyelinating polyradiculoneuropathy. The presence of a lambda monoclonal protein, elevated vascular endothelial growth factor, systemic features, and treatment resistance are clues to the diagnosis. Castleman disease (CD) is seen in a subset of these patients, and when present the neuropathy is similar but less severe...
February 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29153605/associations-between-tumor-necrosis-factor-%C3%AE-gene-polymorphisms-and-the-risk-of-guillain-barr%C3%A3-syndrome-and-its-subtypes-a-systematic-review-and-meta-analysis
#3
Ju Liu, Zhiyun Lian, Hongxi Chen, Ziyan Shi, Huiru Feng, Qin Du, Qin Zhang, Hongyu Zhou
This meta-analysis aimed to assess the relationship between tumor necrosis factor-α (TNF-α) polymorphisms and Guillain-Barré syndrome (GBS) or its subtypes of acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor-sensory axonal neuropathy (AMSAN). A total of six studies with 1013 cases and 1029 controls were included. Our pooled data indicated that TNF-α 308G/A polymorphism was significantly associated with GBS, AMAN, and AMSAN but not with AIDP; TNF-α 857C/T polymorphism was significantly associated with AMAN but not with GBS or AIDP...
December 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29137918/spinobulbar-muscular-atrophy-combined-with-atypical-hereditary-neuropathy-with-liability-to-pressure-palsy
#4
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
https://www.readbyqxmd.com/read/29137897/elevated-serum-levels-of-endothelin-1-in-patients-with-chronic-inflammatory-demyelinating-polyneuropathy
#5
Chun-Wei Chang, Hsiu-Chuan Wu, Rong-Kuo Lyu, Yen-Shi Lo, Chiung-Mei Chen, Long-Sun Ro, Hong-Shiu Chang, Ching-Chang Huang, Ming-Feng Liao, Yih-Ru Wu, Hung-Chou Kuo, Chun-Che Chu, Yi-Ching Weng, Pei-Tsi Wei, Ai-Lun Lo, Kuo-Hsuan Chang
INTRODUCTION: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, or non-hereditary, chronic demyelinating neuropathy. Currently, there is no reliable molecular biomarker that can identify CIDP patients as well as monitor disease severity. MATERIAL AND METHODS: We measured serum levels of endothelin-1 (ET-1), a factors involved in vasoconstrictive, inflammatory and nerve regenerative processes, in 20 CIDP, 21 acute inflammatory demyelinating polyneuropathy (AIDP), 37 multiple sclerosis (MS), and 10 Alzheimer's disease (AD) patients, as well as 26 healthy control (HC) subjects...
November 11, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29130507/neurofascin-155-igg4-neuropathy-pathophysiological-insights-spectrum-of-clinical-severity-and-response-to-treatment
#6
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
https://www.readbyqxmd.com/read/29125079/-relapse-of-chronic-inflammatory-demyelinating-polyneuropathy-following-treatment-with-zoledronic-acid
#7
M Loef, E C T Geijteman, K J Beelen, M Bornebroek, D H Schweitzer
BACKGROUND: Zoledronic acid is a nitrogen-containing bisphosphonate that is frequently used in the treatment of osteoporosis. Many patients experience a so-called acute-phase reaction during initial treatment; this is characterized by flu-like symptoms and fever. CASE DESCRIPTION: We describe a 61-year-old woman who suffered from chronic inflammatory demyelinating polyneuropathy (CIDP), and who was started on intravenous zoledronic acid treatment as adjuvant therapy for breast cancer...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/29122523/subcutaneous-immunoglobulin-for-maintenance-treatment-in-chronic-inflammatory-demyelinating-polyneuropathy-path-a-randomised-double-blind-placebo-controlled-phase-3-trial
#8
Ivo N van Schaik, Vera Bril, Nan van Geloven, Hans-Peter Hartung, Richard A Lewis, Gen Sobue, John-Philip Lawo, Michaela Praus, Orell Mielke, Billie L Durn, David R Cornblath, Ingemar S J Merkies
BACKGROUND: Approximately two-thirds of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) need long-term intravenous immunoglobulin. Subcutaneous immunoglobulin (SCIg) is an alternative option for immunoglobulin delivery, but has not previously been investigated in a large trial of CIDP. The PATH study compared relapse rates in patients given SCIg versus placebo. METHODS: Between March 12, 2012, and Sept 20, 2016, we studied patients from 69 neuromuscular centres in North America, Europe, Israel, Australia, and Japan...
November 6, 2017: Lancet Neurology
https://www.readbyqxmd.com/read/29122522/subcutaneous-igg-for-chronic-inflammatory-demyelinating-polyneuropathy
#9
Marinos C Dalakas
No abstract text is available yet for this article.
November 6, 2017: Lancet Neurology
https://www.readbyqxmd.com/read/29113857/a-rare-neurological-complication-of-waldenstrom-s-macroglobulinemia
#10
Gabriel Torrealba-Acosta, Rajan Gadhia, Thabele Leslie-Mazwi
Bilateral and simultaneous facial nerve palsy (FNP) is a rare clinical condition occurring in 0.3-2.0% of facial palsy cases and is typically a manifestation of an underlying systemic disease. We here describe a case of a 67-year-old Hispanic man with a known history of Waldenstrom's Macroglobulinemia (WM) who presented to the clinic with a sub-acute onset of bilateral facial weakness. No alternate etiology for the facial weakness was identified after a thorough diagnostic approach. WM is a rare hematological condition due to low-grade B cell lymphoma, where lymphoplasmacytoid cells infiltrate different tissues and secrete monoclonal IgM...
November 4, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29113155/poems-syndrome-presentation-with-progressive-weakness-in-upper-and-lower-limbs-a-case-report
#11
Chuang-Jie Cao, Cheng-Yun Dou, Ke-Hua Zhou, Jin-Bo Liu, Hong Lai
Polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes (POEMS) syndrome is a rare variant of plasma cell disorders with multiple systemic manifestations. A 50-year-old female patient presented with progressive weakness in her upper and lower limbs; tingling, numbness and burning in her feet; polyneuropathy (demyelinating in the majority of cases of POEMS syndrome); monoclonal plasma cell disorder (typicallyλ-restricted in cases of POEMS syndrome); sclerotic lesions on the spine and pelvis; organomegaly, including hepatomegaly, splenomegaly and lymphadenopathy; edema; pleural effusion; adrenal, thyroidal, pituitary, gonadal and pancreatic endocrinopathy; skin changes, including hyperpigmentation, dry skin and hypertrichosis; thrombocytosis; pulmonary hypertension; low vitamin B12 and weight loss...
November 2017: Oncology Letters
https://www.readbyqxmd.com/read/29112586/the-role-of-immunoglobulin-in-the-treatment-of-immune-mediated-peripheral-neuropathies
#12
Alejandro Tobon
Immune-mediated neuropathies are a group of peripheral nerve disorders characterized by motor or sensory deficits caused by inflammation leading to demyelination or axonal injury. Intravenous immunoglobulin (IVIg) has been demonstrated to be an effective therapy for the 3 most common immune-mediated neuropathies: Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. This review summarizes current data on the rationale for the use of IVIg in immune-mediated neuropathies, addressing mechanism of action, clinical evidence, and practical considerations for its use...
November 2017: Journal of Infusion Nursing: the Official Publication of the Infusion Nurses Society
https://www.readbyqxmd.com/read/29110767/cerebrospinal-fluid-findings-in-guillain-barr%C3%A3-syndrome-and-chronic-inflammatory-demyelinating-polyneuropathies
#13
Zsolt Illes, Morten Blaabjerg
The classic immunologic alteration of the cerebrospinal fluid (CSF) in Guillain-Barré syndrome (GBS), albuminocytologic dissociation, has been known since the original paper by Guillain, Barré, and Strohl. Albuminocytologic dissociation has been also described in other forms of the GBS spectrum, such as axonal motor or motor-sensory forms (AMAN, AMSAN), the anti-GQ1b spectrum of Miller Fisher syndrome, and Bickerstaff brainstem encephalitis. Cytokines, chemokines, antibodies, complement components, and molecules with a putative neuroprotective role or indicating axonal damage have also been examined using different methods...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29108667/clinical-electrophysiological-genetic-and-imaging-features-of-six-chinese-han-patients-with-hereditary-neuropathy-with-liability-to-pressure-palsies-hnpp
#14
Bin Chen, Songtao Niu, Xingao Wang, Wei Li, Na Chen, Zaiqiang Zhang
Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant peripheral neuropathy caused by mutations in the peripheral myelin protein 22 (PMP22) gene. This study summarizes the clinical, electrophysiological, genetic, and imaging features of six unrelated Chinese Han patients with HNPP. Age of onset was within the second decade in five patients, and 46 years of age in one patient. Weakness or numbness in a unilateral lower extremity was the most common symptom in 5 patients, and bilateral sensorineural hearing loss was also detected in one patient...
November 3, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29095325/charcot-marie-tooth-disease-1x-simulating-paraparetic-guillain-barre-syndrome
#15
Dimitrios Parissis, Panagiotis Ioannidis, Georgios Papadopoulos, Dimitrios Karacostas
X-linked Charcot-Marie-Tooth disease (CMT 1X) is the second most common form of inherited demyelinating neuropathy. It is established that patients suffering from CMT 1X can have episodes of hemiparesis, paraparesis, quadriparesis, ataxia, aphasia, and dysarthria, which can be fully reversible, and 'trigger' factors for these episodes are usually febrile illness, high altitudes, hyperventilation, and physical activity. We describe a 22-year-old patient with a history of viral infection and sleep deprivation who presented to our department because of acute difficulty in walking and neurophysiological findings suggesting Guillain-Barre syndrome...
November 2017: Neurologist
https://www.readbyqxmd.com/read/29093388/an-adult-case-of-recurrent-guillain-barr%C3%A3-syndrome-with-anti-galactocerebroside-antibodies
#16
Hisashi Takahashi, Tadashi Kimura, Natsuko Yuki, Akira Yoshioka
A 79-year-old woman with a history of Guillain-Barré syndrome (GBS) developed somnolence and tetraparesis after pneumonia. Based on clinical and laboratory findings, she was diagnosed with complications of acute inflammatory demyelinating polyneuropathy (AIDP) and acute disseminated encephalomyelitis (ADEM). Anti-galactocerebroside (Gal-C) IgG antibodies were detected in her serum. Cases of recurrent GBS in patients who are positive for this antibody are extremely rare. The anti-Gal-C IgG antibodies likely played an important role in the pathogenesis of the AIDP and ADEM...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29078790/hereditary-neuropathy-with-liability-to-pressure-palsy-hnpp-report-of-a-family-with-a-new-point-mutation-in-pmp22-gene
#17
Carlo Fusco, Carlotta Spagnoli, Grazia Gabriella Salerno, Elena Pavlidis, Daniele Frattini, Francesco Pisani
BACKGROUND: Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant disorder most commonly presenting with acute-onset, non-painful focal sensory and motor mononeuropathy. Approximately 80% of patients carry a 1.5 Mb deletion of chromosome 17p11.2 involving the peripheral myelin protein 22 gene (PMP22), the same duplicated in Charcot-Marie-Tooth 1A patients. In a small proportion of patients the disease is caused by PMP22 point mutations. CASE PRESENTATION: We report on a familial case harbouring a new point mutation in the PMP22 gene...
October 27, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29075571/quantitative-muscle-ultrasound-is-useful-for-evaluating-secondary-axonal-degeneration-in-chronic-inflammatory-demyelinating-polyneuropathy
#18
Keiichi Hokkoku, Kiyoshi Matsukura, Yudai Uchida, Midori Kuwabara, Yuichi Furukawa, Hiroshi Tsukamoto, Yuki Hatanaka, Masahiro Sonoo
INTRODUCTION: In chronic inflammatory demyelinating polyneuropathy (CIDP), exclusion of secondary axonal degeneration is challenging with conventional methods such as nerve conduction study (NCS), needle electromyography, and nerve biopsy. Increased echo intensity (EI) and decreased muscle thickness (MT) identified on muscle ultrasound (MUS) examination represent muscle denervation due to axonal degeneration in neurogenic disorders, suggesting MUS as a new tool to detect secondary axonal degeneration in patients with CIDP...
October 2017: Brain and Behavior
https://www.readbyqxmd.com/read/29073825/myasthenia-gravis-and-chronic-inflammatory-demyelinating-polyneuropathy-in-the-same-patient-a-case-report
#19
Weiwei Quan, Junhui Xia, Qiuling Tong, Jie Lin, Xiaolu Zheng, Xuezhi Yang, Dewei Xie, Yiyun Weng, Xu Zhang
We reported a misdiagnosed case of a 66-year-old man who concomitantly developed chronic inflammatory demyelinating polyneuropathy (CIDP) and myasthenia gravis (MG), which was rarely reported. This man presented with chronic progressive sensory symptoms, flaccid tetraparesis, areflexia and protein-cell dissociation of cerebrospinal fluid. Nerve conduction study was indicative of demyelinating neuropathy. He was suspected as CIDP and treated with high-dose glucocorticoids. However, his condition worsened. Four months later, he was admitted and was diagnosed as combination of CIDP and MG...
October 27, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29043889/unconventional-treatments-for-chronic-inflammatory-demyelinating-polyneuropathy
#20
Yusuf A Rajabally
This article focuses on the unconventional treatments used in chronic inflammatory demyelinating polyneuropathy (CIDP). First line, evidence-based treatments for CIDP include corticosteroids, immunoglobulins and plasma exchanges. Several unproven treatments are however given in treatment-refractory disease or to reduce requirements in validated therapies for reasons of side effects/practical delivery/cost. Despite methodological issues, IFN-α, azathioprine and methotrexate have not been shown to be useful in randomized controlled trials...
October 18, 2017: Neurodegenerative Disease Management
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