听力与言语-语言病理学

行为科学

医学伦理学

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  • Application of differentiated human tonsil-derived stem cells to trembler-J mice.

    abstract:INTRODUCTION:Mesenchymal stem cells (MSCs) can differentiate into various cell types. METHODS:In this study we investigated the potential of human tonsil-derived MSCs (T-MSCs) for neuromuscular regeneration in trembler-J (Tr-J) mice, a model for Charcot-Marie-Tooth disease type 1A (CMT1A). RESULTS:T-MSCs differentiat...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25763

    authors: Park S,Choi Y,Kwak G,Hong YB,Jung N,Kim J,Choi BO,Jung SC

    更新日期:2018-03-01 00:00:00

  • Thymus imaging in myasthenia gravis: The relevance in clinical practice.

    abstract:INTRODUCTION:The ability to distinguish between normal thymus, thymic hyperplasia, and thymoma should aid clinical management and decision making in patients with myasthenia gravis (MG). We sought to determine the accuracy of routine imaging in predicting thymic pathology. METHODS:We retrospectively analyzed records o...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.26096

    authors: Klimiec E,Quirke M,Leite MI,Hilton-Jones D

    更新日期:2018-02-09 00:00:00

  • Muscle changes in patients with diabetes and chronic exertional compartment syndrome before and after treatment with fasciotomy.

    abstract:INTRODUCTION:Muscle changes in patients with diabetes and lower leg pain due to chronic exertional compartment syndrome (CECS) were investigated before and after fasciotomy. METHODS:The tibialis anterior muscle was analyzed with histochemical and morphological techniques in 7 patients with diabetes and CECS before fas...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25715

    authors: Edmundsson DS,Toolanen GL,Stål PS

    更新日期:2018-02-01 00:00:00

  • Two-dimensional and shear wave elastography ultrasound: A reliable method to analyse spastic muscles?

    abstract:INTRODUCTION:Few data exist on the feasibility and reliability of measuring muscular atrophy in 2 dimensions (2D) by ultrasonography (US) and elasticity with shear wave elastography (SWE) in spastic muscles. METHODS:Fourteen patients with chronic stroke took part in 2 intersession reliability experiments performed wit...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25716

    authors: Mathevon L,Michel F,Aubry S,Testa R,Lapole T,Arnaudeau LF,Fernandez B,Parratte B,Calmels P

    更新日期:2018-02-01 00:00:00

  • Nerve ultrasound reliability of upper limbs: Effects of examiner training.

    abstract:INTRODUCTION:Duration of training to reliably measure nerve cross-sectional area with ultrasound is unknown. METHODS:A retrospective review was performed of ultrasound data, acquired and recorded by 2 examiners-an expert and either a trainee with 2 months (novice) or a trainee with 12 months (experienced) of experienc...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25980

    authors: Garcia-Santibanez R,Dietz AR,Bucelli RC,Zaidman CM

    更新日期:2018-02-01 00:00:00

  • Peripheral nerve atrophy together with higher cerebrospinal fluid progranulin indicate axonal damage in amyotrophic lateral sclerosis.

    abstract:INTRODUCTION:We aimed to investigate whether sonographic peripheral cross-sectional nerve area (CSA) and progranulin (PGRN), a neuritic growth factor, are related to each other and whether they interact to predict clinical and paraclinical measures in amyotrophic lateral sclerosis (ALS). METHODS:We included 55 ALS pat...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25682

    authors: Schreiber S,Debska-Vielhaber G,Abdulla S,Machts J,Schreiber F,Kropf S,KÖrtvelyessy P,KÖrner S,Kollewe K,Petri S,Dengler R,Kunz WS,Nestor PJ,Vielhaber S

    更新日期:2018-02-01 00:00:00

  • Effects of hemodialysis on intraneural blood flow in end-stage kidney disease.

    abstract:INTRODUCTION:We quantified intraneural blood flow (INBF) in 18 patients with end-stage kidney disease (ESKD) and examined its relationship with nerve size, neuropathy severity, and nerve excitability parameters. METHODS:Sonographic measurements of the median nerve were performed at the same site before and after hemod...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25704

    authors: Borire AA,Arnold R,Pussell BA,Kwai NC,Visser LH,Simon NG,Kiernan MC,Krishnan AV

    更新日期:2018-02-01 00:00:00

  • Adipose stem cells enhance myoblast proliferation via acetylcholine and extracellular signal-regulated kinase 1/2 signaling.

    abstract:INTRODUCTION:In this study we investigated the interaction between adipose tissue-derived stem cells (ASCs) and myoblasts in co-culture experiments. METHODS:Specific inductive media were used to differentiate ASCs in vitro into a Schwann cell-like phenotype (differentiated adipose tissue-derived stem cells, or dASCs) ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25741

    authors: El-Habta R,Kingham PJ,Backman LJ

    更新日期:2018-02-01 00:00:00

  • The conventional tuning fork as a quantitative tool for vibration threshold.

    abstract:INTRODUCTION:This study was undertaken to describe a method for quantifying vibration when using a conventional tuning fork (CTF) in comparison to a Rydel-Seiffer tuning fork (RSTF) and to provide reference values. METHODS:Vibration thresholds at index finger and big toe were obtained in 281 participants. Spearman's c...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25680

    authors: Alanazy MH,Alfurayh NA,Almweisheer SN,Aljafen BN,Muayqil T

    更新日期:2018-01-01 00:00:00

  • Organization of the motor-unit pool for different directions of isometric contraction of the first dorsal interosseous muscle.

    abstract:INTRODUCTION:Muscle force generation involves recruitment and firing rate modulation of motor units (MUs). The control of MUs in producing multidirectional forces remains unclear. METHODS:We studied MU recruitment and firing properties, recorded from the first dorsal interosseous muscle, for 3 different directions of ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25963

    authors: Lei Y,Suresh NL,Rymer WZ,Hu X

    更新日期:2018-01-01 00:00:00

  • Genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies.

    abstract::Muscular dystrophy is defined as the progressive wasting of skeletal muscles that is caused by inherited or spontaneous genetic mutations. Next-generation sequencing has greatly improved the accuracy and speed of diagnosis for different types of muscular dystrophy. Advancements in depth of coverage, convenience, and o...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审

    doi:10.1002/mus.25953

    authors: Hightower RM,Alexander MS

    更新日期:2018-01-01 00:00:00

  • Resistance training and aerobic training improve muscle strength and aerobic capacity in chronic inflammatory demyelinating polyneuropathy.

    abstract:INTRODUCTION:We investigated the effects of aerobic and resistance exercise in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS:Eighteen CIDP patients treated with subcutaneous immunoglobulin performed 12 weeks of aerobic exercise and 12 weeks of resistance exercise after a run-in period...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25652

    authors: Markvardsen LH,Overgaard K,Heje K,Sindrup SH,Christiansen I,Vissing J,Andersen H

    更新日期:2018-01-01 00:00:00

  • Subacute demyelinating polyradiculoneuropathy complicating Epstein-Barr virus infection in GATA2 haploinsufficiency.

    abstract:INTRODUCTION:Autosomal dominant haploinsufficiency of GATA2 causes monocytopenia and natural killer cell lymphopenia, resulting in predisposition to mycobacterial, fungal, and viral infections. METHODS:Herein we report on the clinical, serologic, electrophysiologic, and pathologic evaluations of a 29-year-old woman wi...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25581

    authors: Kazamel M,Klein CJ,Benarroch EE,Patnaik MM,Tracy JA

    更新日期:2018-01-01 00:00:00

  • Median and ulnar nerve injuries reduce volitional forelimb strength in rats.

    abstract:INTRODUCTION:Peripheral nerve injuries (PNI) are among the leading causes of physical disability in the United States. The majority of injuries occur in the upper extremities, and functional recovery is often limited. Robust animal models are critical first steps for developing effective therapies to restore function a...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25590

    authors: Meyers EC,Granja R,Solorzano BR,Romero-Ortega M,Kilgard MP,Rennaker RL 2nd,Hays S

    更新日期:2017-12-01 00:00:00

  • Association between wrist ratio and carpal tunnel syndrome: Effect modification by body mass index.

    abstract:INTRODUCTION:Previous studies have reported higher wrist ratios (WR) related to carpal tunnel syndrome (CTS) but have not assessed effect modification by obesity and may have inadequately controlled for confounders. METHODS:Baseline data of a multicenter prospective cohort study were analyzed. CTS was defined by nerve...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,多中心研究

    doi:10.1002/mus.25692

    authors: Thiese MS,Merryweather A,Koric A,Ott U,Wood EM,Kapellusch J,Foster J,Garg A,Deckow-Schaefer G,Tomich S,Kendall R,Drury DL,Wertsch J,Hegmann KT,Wistah Study Team.

    更新日期:2017-12-01 00:00:00

  • Detecting fasciculations in cranial nerve innervated muscles with ultrasound in amyotrophic lateral sclerosis.

    abstract:INTRODUCTION:Cranial muscle fasciculations may be difficult to detect in amyotrophic lateral sclerosis (ALS). Ultrasound (US) detection of fasciculations in these muscles may have clinical utility. METHODS:Patients with suspected ALS were prospectively enrolled. Nerve conduction studies, needle electromyography (EMG),...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,多中心研究

    doi:10.1002/mus.25676

    authors: O'gorman CM,Weikamp JG,Baria M,Van Den Engel-Hoek L,Kassardjian C,Van Alfen N,Boon AJ

    更新日期:2017-12-01 00:00:00

  • The quality of life in genetic neuromuscular disease questionnaire: Rasch validation of the French version.

    abstract:INTRODUCTION:Slowly progressive, genetic neuromuscular diseases (gNMDs) often lead to important motor deficiencies and functional limitations. The Quality of Life in Genetic Neuromuscular Disease Questionnaire (QoL-gNMD) is a new health-related quality-of-life questionnaire developed for these patients. The purpose of ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,多中心研究

    doi:10.1002/mus.25598

    authors: Dany A,Rapin A,Lavrard B,Saoût V,Réveillère C,Bassez G,Tiffreau V,Péréon Y,Sacconi S,Eymard B,Dramé M,Jolly D,Novella JL,Hardouin JB,Boyer FC

    更新日期:2017-12-01 00:00:00

  • Childhood autoimmune necrotizing myopathy with anti-signal recognition particle antibodies.

    abstract:INTRODUCTION:The aim of this study was to describe the therapeutic effects of immunomodulatory therapy in 3 patients with childhood autoimmune necrotizing myopathy with anti-signal recognition particle antibodies (SRP-ANM). METHODS:Before treatment, data on clinical features, muscle pathology, and thigh MRIs were obta...

    journal_title:Muscle & nerve

    pub_type:

    doi:10.1002/mus.25575

    authors: Zhao Y,Liu X,Zhang W,Yuan Y

    更新日期:2017-12-01 00:00:00

  • Low-load resistance training with low relative pressure produces muscular changes similar to high-load resistance training.

    abstract:INTRODUCTION:This study compares the acute and chronic response of high-load resistance training (HL) to low-load resistance training with low blood flow restriction (LL-BFR) pressure. METHODS:Participants completed elbow flexion with either HL or LL-BFR or nonexercise. In the chronic study, participants in the HL and...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25626

    authors: Kim D,Loenneke JP,Ye X,Bemben DA,Beck TW,Larson RD,Bemben MG

    更新日期:2017-12-01 00:00:00

  • Combined XIL-6R and urocortin-2 treatment restores MDX diaphragm muscle force.

    abstract:INTRODUCTION:Duchenne muscular dystrophy (DMD) is characterized by progressive muscle degeneration leading to immobility, respiratory failure, and premature death. As chronic inflammation and stress are implicated in DMD pathology, the efficacy of an anti-inflammatory and anti-stress intervention strategy in ameliorati...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25644

    authors: Manning J,Buckley MM,O'Halloran KD,O'Malley D

    更新日期:2017-12-01 00:00:00

  • Anti-LRP4 autoantibodies in Chinese patients with myasthenia gravis.

    abstract:INTRODUCTION:We assessed antibodies against low-density lipoprotein receptor-related protein 4 (LRP4-Ab) in a Chinese population with myasthenia gravis (MG). METHODS:Serum samples from 116 patients and 80 controls were collected. Acetylcholine receptor antibodies(AChR-Ab) and muscle-specific receptor tyrosine kinase a...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25591

    authors: Li Y,Zhang Y,Cai G,He D,Dai Q,Xu Z,Chu L

    更新日期:2017-11-01 00:00:00

  • Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial.

    abstract:INTRODUCTION:Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7Ionis ), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. METHODS:We assessed: (1) Proficiency of grading ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,多中心研究

    doi:10.1002/mus.25563

    authors: Dyck PJ,Kincaid JC,Dyck PJB,Chaudhry V,Goyal NA,Alves C,Salhi H,Wiesman JF,Labeyrie C,Robinson-Papp J,Cardoso M,Laura M,Ruzhansky K,Cortese A,Brannagan TH 3rd,Khoury J,Khella S,Waddington-Cruz M,Ferreira J,Wang AK,

    更新日期:2017-11-01 00:00:00

  • Repeatability, consistency, and accuracy of hand-held dynamometry with and without fixation for measuring ankle plantarflexion strength in healthy adolescents and adults.

    abstract:INTRODUCTION:Hand-held dynamometry (HHD) is commonly used to measure ankle plantarflexion strength but has variable reliability measuring higher forces. Fixed HHD is suggested to improve reliability. We, therefore, compared the reliability, consistency, and accuracy of measuring plantarflexion strength. METHODS:Planta...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25576

    authors: Davis PR,McKay MJ,Baldwin JN,Burns J,Pareyson D,Rose KJ

    更新日期:2017-11-01 00:00:00

  • Paraneoplastic cerebellar degeneration and lambert-eaton myasthenia in a patient with merkel cell carcinoma and voltage-gated calcium channel antibodies.

    abstract:INTRODUCTION:Merkel cell carcinoma is a rare cutaneous, aggressive tumor. Although it shares many neuroendocrine features with small cell lung carcinoma, it has only occasionally been reported with paraneoplastic neurological syndromes. METHODS:A healthy 67-year-old man developed acute ataxia, vertigo, and nausea. Sub...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25530

    authors: Pavolucci L,Giannini G,Giannoccaro MP,Foschini MP,Lang B,Avoni P,Tinuper P,Vincent A,Liguori R

    更新日期:2017-11-01 00:00:00

  • Mitochondrial capacity, muscle endurance, and low energy in friedreich ataxia.

    abstract:INTRODUCTION:In this study we noninvasively evaluated skeletal muscle mitochondrial capacity, muscle-specific endurance, and energy/fatigue feelings in persons with Friedreich ataxia (FRDA) and able-bodied controls (AB). METHODS:Forearm mitochondrial capacity was measured in FRDA (n = 16) and AB (n = 10) study partici...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25524

    authors: Bossie HM,Willingham TB,Schoick RAV,O'Connor PJ,McCully KK

    更新日期:2017-10-01 00:00:00

  • Assessment of small sensory fiber function across the spectrum of severity in carpal tunnel syndrome patients.

    abstract:INTRODUCTION:In compressive neuropathies, large myelinated nerve fibers are generally thought to be more susceptible. In this study, we investigated small myelinated Aδ and unmyelinated C fiber function in patients with mild, moderate, and severe carpal tunnel syndrome. METHODS:Forty-four healthy controls and 81 carpa...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25592

    authors: Clarke C,Christensen C,Curran MWT,Chan KM

    更新日期:2017-10-01 00:00:00

  • Eosinophilic fasciitis with subjacent myositis.

    abstract:INTRODUCTION:Eosinophilic fasciitis (EF) is a rare disorder that can present with muscle symptoms that mimic other neuromuscular diseases. METHODS:We report the case of a 43-year-old woman with chronic muscle aches, tightness, and stiffness with hypertrophied, well-defined muscles despite physical inactivity, and thic...

    journal_title:Muscle & nerve

    pub_type:

    doi:10.1002/mus.25492

    authors: Whitlock JB,Dimberg EL,Selcen D,Rubin DI

    更新日期:2017-09-01 00:00:00

  • Ultrasound elastography assessment of the median nerve in leprosy patients.

    abstract:INTRODUCTION:We sought to compare median nerve elasticity between leprosy patients (LPs) and healthy volunteers (HVs) using ultrasound elastography (UE). METHODS:Two radiologists independently measured the strain ratio of the median nerve/flexor digitorum superficialis muscle (MN/FDSM) of 18 LP and 18 HV using real-ti...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25510

    authors: Nogueira-Barbosa MH,Lugão HB,Gregio-Júnior E,Crema MD,Kobayashi MTT,Frade MAC,Pavan TZ,Carneiro AAO

    更新日期:2017-09-01 00:00:00

  • Two patients with GMPPB mutation: The overlapping phenotypes of limb-girdle myasthenic syndrome and limb-girdle muscular dystrophy dystroglycanopathy.

    abstract:INTRODUCTION:Mutations in the guanosine diphosphate-mannose pyrophosphorylase-B gene (GMPPB) have been identified in congenital muscular dystrophies, limb-girdle muscular dystrophy (LGMD2T), and congenital myasthenic syndromes (CMSs); overall, 41 patients have been described. METHODS:Two patients presented with a myas...

    journal_title:Muscle & nerve

    pub_type:

    doi:10.1002/mus.25485

    authors: Montagnese F,Klupp E,Karampinos DC,Biskup S,Gläser D,Kirschke JS,Schoser B

    更新日期:2017-08-01 00:00:00

  • A reliable technique for ultrasound-guided perineural injection in ulnar neuropathy at the elbow.

    abstract:INTRODUCTION:Ulnar neuropathy at the elbow (UNE) is a common peripheral compression neuropathy and, in most cases, occurs at 2 sites, the retroepicondylar groove or the cubital tunnel. With regard to a potential therapeutic approach with perineural corticosteroid injection, the aim of this study was to evaluate the dis...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25490

    authors: Hamscha UM,Tinhofer I,Heber S,Grisold W,Weninger WJ,Meng S

    更新日期:2017-08-01 00:00:00

  • Physical function and mobility in children with congenital myotonic dystrophy.

    abstract:INTRODUCTION:Congenital myotonic dystrophy (CDM) occurs when symptoms of myotonic dystrophy present at birth. In this study we evaluated the relationship between physical function, muscle mass, and age to provide an assessment of the disease and help prepare for therapeutic trials. METHODS:CDM participants performed t...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25482

    authors: Pucillo EM,Dibella DL,Hung M,Bounsanga J,Crockett B,Dixon M,Butterfield RJ,Campbell C,Johnson NE

    更新日期:2017-08-01 00:00:00

  • Impaired calcium signaling in muscle fibers from intercostal and foot skeletal muscle in a cigarette smoke-induced mouse model of COPD.

    abstract:INTRODUCTION:Respiratory and locomotor skeletal muscle dysfunction are common findings in chronic obstructive pulmonary disease (COPD); however, the mechanisms that cause muscle impairment in COPD are unclear. Because Ca2+ signaling in excitation-contraction (E-C) coupling is important for muscle activity, we hypothesi...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25466

    authors: Robison P,Sussan TE,Chen H,Biswal S,Schneider MF,Hernández-Ochoa EO

    更新日期:2017-08-01 00:00:00

  • Mechanomyographic responses during recruitment curves in the soleus muscle.

    abstract:INTRODUCTION:In this study we examined relationships among mechanomyographic (MMG), electromyographic (EMG), and peak twitch torque (PTT) responses as well as test-retest reliability when recorded during recruitment curves in the soleus muscle. METHODS:PTT, EMG (M-wave, H-reflex), and MMG responses were recorded durin...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25432

    authors: Miramonti AA,Jenkins NDM,Oza PD,Weir JP,Cramer JT

    更新日期:2017-07-01 00:00:00

  • Lower limb muscle strength impairment in late-onset and adult myotonic dystrophy type 1 phenotypes.

    abstract:INTRODUCTION:Lower limb strength has never been characterized separately for late-onset and adult myotonic dystrophy type 1 (DM1) phenotypes. METHODS:The purpose of this study was to: (1) describe and compare lower limb strength between the 2 DM1 phenotypes; and (2) compare the impairment profiles obtained from 2 asse...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25451

    authors: Petitclerc É,Hébert LJ,Mathieu J,Desrosiers J,Gagnon C

    更新日期:2017-07-01 00:00:00

  • Peripheral nerve ultrasound in cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS).

    abstract:INTRODUCTION:We report preliminary findings of nerve ultrasound in patients with cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS) who have sensory impairment due to dorsal root ganglionopathy. METHODS:The ultrasound cross-sectional area (CSA) of median and ulnar nerves of 7 CANVAS patients was compa...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25476

    authors: Pelosi L,Leadbetter R,Mulroy E,Chancellor AM,Mossman S,Roxburgh R

    更新日期:2017-07-01 00:00:00

  • A novel points system to predict the prognosis of ulnar neuropathy at the elbow.

    abstract:INTRODUCTION:In this study we aimed to identify prognostic factors of ulnar neuropathy at the elbow (UNE) and developed a scoring system to establish the prognosis. METHODS:We collected baseline clinical, electrophysiologic, and ultrasonographic data from 2 cohorts. The outcomes for all patients were determined on fol...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25406

    authors: Beekman R,Zijlstra W,Visser LH

    更新日期:2017-05-01 00:00:00

  • Inflammatory demyelinating neuropathy heralding accelerated chediak-higashi syndrome.

    abstract:INTRODUCTION:Chediak-Higashi syndrome (CHS) is a very rare autosomal recessive disorder (gene CHS1/LYST) characterized by partial albinism, recurrent infections, and easy bruising. Survivors develop a constellation of slowly progressive neurological manifestations. METHODS:We describe clinical, laboratory, electrophys...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25414

    authors: Faber IV,Prota JRM,Martinez ARM,Nucci A,Lopes-Cendes I,Júnior MCF

    更新日期:2017-05-01 00:00:00

  • Chronic peripheral nerve compression disrupts paranodal axoglial junctions.

    abstract:INTRODUCTION:Peripheral nerves are often exposed to mechanical stress leading to compression neuropathies. The pathophysiology underlying nerve dysfunction by chronic compression is largely unknown. METHODS:We analyzed molecular organization and fine structures at and near nodes of Ranvier in a compression neuropathy ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25273

    authors: Otani Y,Yermakov LM,Dupree JL,Susuki K

    更新日期:2017-04-01 00:00:00

  • Clinical-pathologic correlations in voltage-gated Kv1 potassium channel complex-subtyped autoimmune painful polyneuropathy.

    abstract:INTRODUCTION:Voltage-gated Kv1 potassium channel complex (VGKC) autoantibodies subtyped for leucine-rich glioma-inactivated 1 (LGI1), contactin-associated-proteinlike 2 (CASPR2), and Kv IgGs have a spectrum of neurological presentations. Painful polyneuropathy is seen in some patients, but nerve pathology descriptions ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25371

    authors: Lahoria R,Pittock SJ,Gadoth A,Engelstad JK,Lennon VA,Klein CJ

    更新日期:2017-04-01 00:00:00

  • Modeling functional decline over time in sporadic inclusion body myositis.

    abstract:INTRODUCTION:The ability to individualize recommendations or expectations of disease progression based on a patient's unique characteristics has merit for use in sporadic inclusion body myositis (sIBM). METHODS:Fifty-five subjects with sIBM completed a battery of strength and functional outcomes at 2 study visits. The...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25373

    authors: Alfano LN,Yin H,Dvorchik I,Maus EG,Flanigan KM,Mendell JR,Lowes LP

    更新日期:2017-04-01 00:00:00

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