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Demyelinating polyneuropathy

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https://www.readbyqxmd.com/read/28914883/cidp-and-other-inflammatory-neuropathies-in-diabetes-diagnosis-and-management
#1
REVIEW
Yusuf A Rajabally, Mark Stettner, Bernd C Kieseier, Hans-Peter Hartung, Rayaz A Malik
Distal symmetric polyneuropathy (DSPN) is the most common neuropathy to occur in diabetes mellitus. However, patients with diabetes can also develop inflammatory neuropathies, the most common and most treatable of which is chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Whether diabetes is a risk factor for CIDP remains under debate. Early studies suggested that patients with diabetes were at increased risk of CIDP, but epidemiological studies failed to confirm the association, and subsequent data have re-opened the debate...
September 15, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28904571/transcranial-magnetic-stimulation-as-an-additional-diagnostic-tool-in-children-with-acute-inflammatory-demyelinating-polyneuropathy
#2
Voitenkov Vladislav Voitenkov, Klimkin Andrey, Skripchenko Natalia, Aksenova Anastasia
CONTEXT: The diagnosis of polyneuropathy may be challenging at the early stages of the disease. Despite electromyography (EMG) efficacy in the establishment of polyneuropathy diagnosis, in some cases, results are dubious and neurophysiologists may implement additional techniques to ensure that conduction is affected. AIMS: The aim of the study was to evaluate motor-evoked potential (MEP) characteristics in children with acute inflammatory demyelinating polyneuropathy (AIDP)...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28904461/the-prevalence-and-severity-of-autonomic-dysfunction-in-chronic-inflammatory-demyelinating-polyneuropathy
#3
Suresh Babu Pasangulapati, T V Murthy, Ajith Sivadasan, L Rynjah Gideon, A T Prabhakar, Aaron Sanjith, Vivek Mathew, Mathew Alexander
INTRODUCTION: In chronic inflammatory demyelinating polyneuropathy (CIDP), emphasis has been on motor disabilities, and autonomic dysfunction in these patients has not been addressed systematically. MATERIALS AND METHODS: Autonomic function was prospectively analyzed in 38 patients with CIDP. Quantitative autonomic function testing was done using Finometer(®) PRO and severity of adrenergic and cardiovagal dysfunction graded according to composite autonomic severity score and sudomotor dysfunction assessed using sympathetic skin response...
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28894082/chronic-inflammatory-demyelinating-polyneuropathy-cidp-an-uncommon-manifestation-of-systemic-lupus-erythematosus-sle
#4
Hrudya Abraham, Jose Kuzhively, Syed W Rizvi
BACKGROUND Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon manifestation of systemic lupus erythematosus (SLE). We report a case of SLE presenting as CIDP and discuss the diagnosis, management, and prognosis of CIDP. CASE REPORT A 40-year-old woman with a past medical history of SLE treated with hydroxychloroquine presented with bilateral, progressive, ascending, sensory and motor neuropathy. Physical examination showed weakness and reduced temperature of all extremities, reduced pinprick and vibration sense of the distal extremities, loss of reflexes, and walking with a wide-based unsteady gait...
September 12, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28884726/-polyneuropathies-in-intestinal-diseases
#5
I V Damulin, D A Degterev
Different aspects of polyneuropathies (PN) developed due to the deficit of group B vitamins in intestinal diseases are considered. Neurological disturbances related to intestinal diseases are caused by malabsorption that leads to the deficit of some compounds extremely important for normal cell metabolism, pathological changes of mucous coat of the stomach and intestine and higher sensitivity to the plant protein gluten. Vitamin B12 deficit can lead to a number of neurological disturbances; patients with pernicious anemia most often develop myelopathy and PN...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28881389/comparisons-in-fluctuation-of-muscle-strength-and-function-in-patients-with-immune-mediated-neuropathy-treated-with-intravenous-versus-subcutaneous-immunoglobulin
#6
Ingelise Christiansen, Lars H Markvardsen, Johannes Jakobsen
INTRODUCTION: Variation of muscle strength and function have not been studied in patients with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy whose treatment regimen has been changed from intravenous to subcutaneous immunoglobulin (IVIG to SCIG). METHODS: In a prospective open label study, patients were changed from monthly IVIG to weekly SCIG. The primary endpoint was the variation of isokinetic muscle strength (cIKS). Secondary endpoints were variation of Medical Research Council (MRC) score, grip strength (GS), 9-hole-peg test (9-HPT), 40-meter-walk test (40-MWT)...
September 7, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28866625/autologous-haematopoietic-stem-cell-transplantation-for-neurological-diseases
#7
REVIEW
Joachim Burman, Andreas Tolf, Hans Hägglund, Håkan Askmark
Neuroinflammatory diseases such as multiple sclerosis, neuromyelitis optica, chronic inflammatory demyelinating polyneuropathy and myasthenia gravis are leading causes of physical disability in people of working age. In the last decades significant therapeutic advances have been made that can ameliorate the disease course. Nevertheless, many affected will continue to deteriorate despite treatment, and the costs associated with disease-modifying drugs constitute a significant fiscal burden on healthcare in developed countries...
September 2, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28859335/a-new-mutation-in-fig4-causes-a-severe-form-of-cmt4j-involving-trpv4-in-the-pathogenic-cascade
#8
Benoit J Gentil, Erin O'Ferrall, Colin Chalk, Luis F Santana, Heather D Durham, Rami Massie
Mutations in FIG4, coding for a phosphoinositol(3,5) bisphosphate 5' phosphatase and involved in vesicular trafficking and fusion, have been shown causing a recessive form of Charcot-Marie-Tooth (CMT). We have identified a novel intronic mutation in the FIG4 in a wheel-chair bound patient presenting with a severe form of CMT4J and provide a longitudinal study. Investigations indicated a demyelinating sensorimotor polyneuropathy with diffuse active denervation and severe axonal loss. Genetic testing revealed that the patient is heterozygous for 2 FIG4 mutations, p...
September 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28855494/charcot-marie-tooth-disease-type-2-caused-by-homozygous-mme-gene-mutation-superimposed-by-chronic-inflammatory-demyelinating-polyneuropathy
#9
Miwako Fujisawa, Yasuteru Sano, Masatoshi Omoto, Jyun-Ichi Ogasawara, Michiaki Koga, Hiroshi Takashima, Takashi Kanda
We report a 59-year-old Japanese male who developed gradually worsening weakness and numbness of distal four extremities since age 50. His parents were first cousins, and blood and cerebral spinal examinations were unremarkable. Homozygous mutation of MME gene was detected and thus he was diagnosed as autosomal-recessive Charcot-Marie-Tooth disease 2T (AR-CMT2T); however, electrophysiological examinations revealed scattered demyelinative changes including elongated terminal latency in several peripheral nerve trunks...
August 31, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28835984/relapsing-remitting-type-1-diabetes
#10
Kayleigh M van Megen, Matthew P Spindler, Fleur M Keij, Ineke Bosch, Fleur Sprangers, Annet van Royen-Kerkhof, Tatjana Nikolic, Bart O Roep
AIMS/HYPOTHESIS: Type 1 diabetes is believed to be an autoimmune disease associated with irreversible loss of insulin secretory function that follows a chronic progressive course. However, it has been speculated that relapsing/remitting disease progression may occur in type 1 diabetes. METHODS: We report the case of an 18-year-old girl with Graves' disease, chronic inflammatory demyelinating polyneuropathy (CIDP) and multiple islet autoantibodies, presenting with relapsing/remitting hyperglycaemia...
August 23, 2017: Diabetologia
https://www.readbyqxmd.com/read/28835897/clinical-and-pathological-variation-of-charcot-marie-tooth-1a-in-a-large-chinese-cohort
#11
Rui Wu, He Lv, Wei Zhang, Zhaoxia Wang, Yuehuan Zuo, Jing Liu, Yun Yuan
Charcot-Marie-Tooth 1A (CMT1A) caused by peripheral myelin protein 22 (PMP22) gene duplication is the most common form of hereditary polyneuropathy. Twenty-four genetically confirmed CMT1A patients with sural nerve biopsies were enrolled in this study. The clinical picture included a great variability of phenotype with mean onset age of 22.2 ± 14.5 years (1-55 years). Pathologically, we observed a severe reduction in myelinated fiber density showing three types of changes: pure onion bulb formation in 3 cases (12...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28833261/abstracts
#12
I N van Schaik, V Bril, N van Geloven, H P Hartung, R A Lewis, G Sobue, J P Lawo, O Mielke, B L Durn, D R Cornblath, I S J Merkies, M M Dimachkie
INTRODUCTION: Patients with chronic inflammatory demyelinating polyneuropathy (CIDP) often require long-term intravenous immunoglobulin (IVIG) maintenance therapy. Subcutaneous immunoglobulin (SCIG) offers an alternative administration option with anticipated improvements in patient quality of life, convenience, and flexibility. OBJECTIVES: To evaluate IgPro20 (SCIG) as a maintenance treatment in CIDP. METHODS: A randomized, double-blind trial in CIDP patients (n=172) investigated 0...
August 22, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28820724/neurologic-complications-in-persons-with-hiv-infection-in-the-era-of-antiretroviral-therapy
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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-immunoglobulin-g4-antibody
#19
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 immunoglobulin G4 (IgG4) antibody-positive CIDP who underwent sural nerve biopsies. RESULTS: All patients were relatively young at onset. Three patients experienced tremors, and 2 patients had severe ataxia. Although the response to intravenous immunoglobulin was poor in all patients, plasma exchange 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
#20
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
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