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Demyelinating polyneuropathy

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https://www.readbyqxmd.com/read/28820724/neurologic-complications-in-persons-with-hiv-infection-in-the-era-of-antiretroviral-therapy
#1
Dennis Kolson
Neurologic complications in persons with HIV infection are less severe in the era of potent antiretroviral therapy but remain highly prevalent. Prior to the use of antiretroviral therapy, opportunistic infections of the central nervous system (CNS) and CNS malignancy were common. Progressive multifocal leukoencephalopathy (PML), however, remains a diagnostic challenge in HIV-infected individuals, and no effective antiviral treatment for PML is currently available. Primary neurologic complications of acute HIV infection include aseptic meningitis and acute inflammatory demyelinating polyneuropathy...
July 2017: Topics in Antiviral Medicine
https://www.readbyqxmd.com/read/28820624/retinopathy-and-optic-atrophy-expanding-the-phenotypic-spectrum-of-pathogenic-variants-in-the-aars2-gene
#2
Jason H Peragallo, Stephanie Keller, Marjo S van der Knaap, Bruno P Soares, Suma P Shankar
BACKGROUND: Optic atrophy may be the sequela of optic nerve injury due to any insult, including isolated and syndromic genetic diseases. Alanyl-tRNA synthetase 2 (AARS2) pathogenic variants have been reported to cause leukodystrophy with ovarian failure, and cardiomyopathy (#615889) as well as combined oxidative phosphorylation deficiency-8 (#614096). We report a young child who presented with decreased vision due to optic atrophy and was found to harbor missense variants in the AARS2 gene expanding the phenotypic expression of the AARS2 gene...
August 18, 2017: Ophthalmic Genetics
https://www.readbyqxmd.com/read/28811143/nadph-oxidases-as-drug-targets-and-biomarkers-in-neurodegenerative-diseases-what-is-the-evidence
#3
REVIEW
Silvia Sorce, Roland Stocker, Tamara Seredenina, Rikard Holmdahl, Adriano Aguzzi, Adriano Chio, Antoine Depaulis, Freddy Heitz, Peter Olofsson, Tomas Olsson, Venceslas Duveau, Despina Sanoudou, Sara Skosgater, Antonia Vlahou, Dominique Wasquel, Karl-Heinz Krause, Vincent Jaquet
Neurodegenerative disease are frequently characterized by microglia activation and/or leukocyte infiltration in the parenchyma of the central nervous system and at the molecular level by increased oxidative modifications of proteins, lipids and nucleic acids. NADPH oxidases (NOX) emerged as a novel promising class of pharmacological targets for the treatment of neurodegeneration due to their role in oxidant generation and presumably in regulating microglia activation. The unique function of NOX is the generation of superoxide anion (O2(•-)) and hydrogen peroxide (H2O2)...
August 12, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28806453/neurologic-complications-associated-with-the-zika-virus-in-brazilian-adults
#4
Ivan Rocha Ferreira da Silva, Jennifer A Frontera, Ana Maria Bispo de Filippis, Osvaldo Jose Moreira do Nascimento
Importance: There are no prospective cohort studies assessing the incidence and spectrum of neurologic manifestations secondary to Zika virus (ZIKV) infection in adults. Objective: To evaluate the rates of acute ZIKV infection among patients hospitalized with Guillain-Barré syndrome (GBS), meningoencephalitis, or transverse myelitis. Design, Setting, and Participants: A prospective, observational cohort study was conducted at a tertiary referral center for neurological diseases in Rio de Janeiro, Brazil, between December 5, 2015, and May 10, 2016, among consecutive hospitalized adults (>18 years of age) with new-onset acute parainfectious or neuroinflammatory disease...
August 14, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28798722/autonomic-neuropathy-and-albuminocytologic-dissociation-in-cerebrospinal-fluid-as-the-presenting-features-of-primary-amyloidosis-a-case-report
#5
Jingjing Li, Yi Li, Hongbing Chen, Shihui Xing, Huiyu Feng, Dawei Liu, Dilong Wang, Jinsheng Zeng, Yuhua Fan
OBJECTIVE: Primary amyloidosis is a disease with a poor prognosis and multi-organ involvement. Here, we report the clinical and pathological features of a patient with primary amyloidosis featuring autonomic neuropathy as the initial symptom and albuminocytologic dissociation in the cerebrospinal fluid (CSF). METHODS: The patient was a 60-year-old Chinese male with numbness, orthostatic hypotension, and gastrointestinal symptoms. For diagnosis, we performed an electromyogram (EMG), lumbar puncture, Bence Jones protein urine test, serum electrophoresis blood test, sural nerve and rectal membrane biopsies, transthyretin (TTR) gene sequencing, and bone marrow puncture...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28796344/diagnostic-value-of-the-near-nerve-needle-sensory-nerve-conduction-in-sensory-inflammatory-demyelinating-polyneuropathy
#6
Zeki Odabasi, Shin J Oh
INTRODUCTION: We report here the diagnostic value of the near-nerve needle sensory nerve conduction study (NNN-SNCS) in sensory inflammatory demyelinating polyneuropathy (IDP) in which the routine nerve conduction study was normal or nondiagnostic. METHODS: The NNN-SNCS was performed to identify demyelination in the plantar nerves in 14 patients and in the median or ulnar nerve in two patients with sensory IDP . RESULTS: In 16 patients with sensory IDP, routine NCSs were either normal or non-diagnostic for demyelination...
August 10, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28796305/electron-microscopic-abnormality-and-therapeutic-efficacy-in-chronic-inflammatory-demyelinating-polyneuropathy-with-anti-neurofascin155-igg4-antibody
#7
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/28795614/shifting-borders-crossing-boundaries-the-case-of-combined-central-and-peripheral-demyelination
#8
Orhan Aktas
The case report by Puthenparampil et al. suggests that heterogeneity of demyelinating diseases may be more complex than expected. The authors describe a female patient starting with isolated but relapsing myelitis (in the absence of oligoclonal bands or anti-aquaporin 4 antibodies) and later experiencing the simultaneous occurrence of inflammatory demyelinating polyneuropathy and of a tumefactive hippocampal brain lesion responsive to immunotherapy. As testing for antibodies against established and novel auto-antigens including neurofascin-155 was negative, this case may be regarded as an example for the unfolding spectrum of combined central and peripheral demyelination (CCPD) syndromes with so far unknown immunological target(s)...
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28795610/acute-simultaneous-development-of-brain-tumour-like-lesion-and-demyelinating-polyneuropathy-in-a-patient-with-chronic-relapsing-myelitis
#9
Marco Puthenparampil, Alberto Terrin, Lisa Federle, Matteo Gizzi, Paola Perini, Paolo Gallo
Combined central and peripheral demyelination (CCPD) is a rare chronic inflammatory disorder of the nervous system. We describe the case of a patient with a history of recurrent myelitis that acutely and simultaneously developed a brain tumour-like lesion and a sensitive-motor demyelinating polyneuropathy. The diagnosis of CCPD was supported by a detailed diagnostic workup. Up to date, no similar cases have been reported in the literature.
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28795553/-chronic-demyelinating-polyradiculoneuropathy-revealing-a-solitary-plasmacytoma-of-the-right-iliac-bone
#10
J Oumerzouk, A Bourazza
Solitary bone plasmacytoma (pso) is a rare tumor, made of malignant plasma cells observed in a single bone, and without systemic proliferation. We report the case of a 33 year-old man who was admitted with chronic demyelinating sensorimotor polyneuropathy. Immunoelectrophoresis with immunofixation of serum proteins showed a monoclonal peak igg with lambda light chain and the workup was in favour of right hipbone secreting solitary plasmacytoma. The patient was treated with radiotherapy and steroids, and the evolution has been marked by a partial recovery of the motor deficit...
July 2017: Revue Médicale de Liège
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
#11
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/28764223/frequency-of-polyneuropathy-in-patients-on-long-term-peritoneal-dialysis-treatment
#12
Taner Basturk, Yener Koc, Aarzu Ozdemir Kayalar, Figen Yilmaz, Nuri Baris Hasbal, Tamer Sakaci, Elbis Ahbap, Abdulkadir Unsal
INTRODUCTION: Uremic polyneuropathy is very common among patients with Chronic Kidney Disease (CKD). The patients have electrophysiologic signs of impaired nerve function, although a lower percentage of patients are symptomatic. Electrophysiological parameters are quantitative indices of Polyneuropathy (PNP) severity. AIM: To assess the frequency of PNP in patients on long term Peritoneal Dialysis (PD) treatment. MATERIALS AND METHODS: Twenty three PD patients were analysed, who were receiving dialysis for at least five years and the study population divided into two groups according to duration of PD treatment...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28763304/chronic-inflammatory-demyelinating-polyneuropathy
#13
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/28747620/chronic-inflammatory-demyelinating-polyneuropathy-variant-with-creatine-kinase-elevation-and-vanishing-effect-of-immunoglobulins
#14
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/28714395/five-decades-of-cuprizone-an-updated-model-to-replicate-demyelinating-diseases
#15
Jose M Vega-Riquer, Gerardo Mendez-Victoriano, Raul A Morales-Luckie, Oscar Gonzalez-Perez
Multiple sclerosis, Guillain-Barré syndrome, peripheral nerve polyneuropathy and others comprise a group of demyelinating diseases characterized by progressive (and eventually irreversible) loss of oligodendrocytes and myelin sheaths in the white matter of the brain. To date, the etiology of these disorders is not well known and no effective treatments are currently available. Therefore, further research is needed to gain a better understand and treat these patients. To accomplish this goal, it is necessary to have appropriate animal models that closely resemble the pathophysiology of these diseases...
July 17, 2017: Current Neuropharmacology
https://www.readbyqxmd.com/read/28708133/autoantibodies-in-chronic-inflammatory-neuropathies-diagnostic-and-therapeutic-implications
#16
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/28694071/first-clinical-and-genetic-description-of-a-family-diagnosed-with-late-onset-pompe-disease-from-costa-rica
#17
Gabriel Torrealba-Acosta, María Consuelo Rodríguez-Roblero, Sixto Bogantes-Ledezma, Kenneth Carazo-Céspedes, Claude Desnuelle
Glycogen storage disease type II, also known as Pompe disease, is an autosomal recessive disorder caused by deficiency of enzymatic activity of acid alpha-glucosidase. The wide phenotypical variation of this disease relates to the amount of residual enzymatic activity depending on the combination of mutations on each allele. We confirmed Pompe disease in a patient that presented with progressive weakness, recurrent episodes of respiratory failure associated with pneumonia, a predominantly demyelinating mixed sensorimotor polyneuropathy and paraspinal complex repetitive discharges...
June 20, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28682959/paraneoplastic-neuropathies
#18
Jean-Christophe Antoine, Jean-Philippe Camdessanché
PURPOSE OF REVIEW: To review recent advances in paraneoplastic neuropathies with emphasis on their definition, different forms and therapeutic development. RECENT FINDINGS: A strict definition of definite paraneoplastic neuropathies is necessary to avoid confusion. With carcinoma, seronegative sensory neuronopathies and neuronopathies and anti-Hu and anti-CV2/Contactin Response Mediator Protein 5 antibodies are the most frequent. With lymphomas, most neuropathies occur with monoclonal gammopathy including AL amyloidosis, Polyneuropathy-Organomegaly-Endocrinopathy-M component-Skin changes (POEMS) syndrome, type I cryoglobulinemia and antimyelin-associated glycoprotein (MAG) neuropathies and Waldenström's disease...
July 5, 2017: Current Opinion in 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
#19
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/28639596/guillain-barre-syndrome-demographics-clinical-profile-seasonal-variation-in-a-tertiary-care-centre-of-central-india
#20
Manisha Shrivastava, Shah Nehal, Navaid Seema
BACKGROUND & OBJECTIVES: Guillain-Barre syndrome (GBS) is an autoimmune disease and a recognized cause of generalized progressive paralysis worldwide. The present study was aimed to document the clinical findings, demographics and seasonal variations amongst the patients with GBS during the hospital stay. METHODS: A retrospective analysis of 66 referred cases diagnosed as GBS was conducted. Medical records and the data related to age, sex, antecedent illness, duration of symptoms before admission, muscle power graded by the Medical Research Council scale, functional scores, details of Intensive Care Unit complications and need for ventilation were obtained...
February 2017: Indian Journal of Medical Research
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