Pulmonary function and electromyographic study of respiratory muscles in myotonic dystrophy.

Abstract:

:Ten adult myotonic dystrophy patients underwent measurements of lung function, maximal dynamic and static ventilatory efforts, and respiratory muscle electromyography (EMG). EMG studies were performed during spontaneous breathing or when subjects breathed through high inspiratory or expiratory resistive loads. Present results show that (1) a moderate restriction of lung volumes with hypoxemia plus normocapnia is often observed; (2) patients sustain dynamic ventilatory efforts more easily than static work; and (3) abnormalities in respiratory muscle EMG exist with spontaneous expiratory and inspiratory intercostal activities during quiet breathing and changes in muscular response to resistive loads. Inspiratory loading evokes contraction of expiratory muscles, with a marked decrease in inspiratory activities. Expiratory resistive loads prolong the diaphragmatic contraction throughout the expiratory time, and in some patients, relaxation of the diaphragm does not occur during the loaded run. These EMG data suggest that the reciprocal inhibition among respiratory neurons is enhanced in myotonic dystrophy and that myotonia also occurs in the diaphragm when loads oppose its relaxation.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Jammes Y,Pouget J,Grimaud C,Serratrice G

doi

10.1002/mus.880080708

subject

Has Abstract

pub_date

1985-09-01 00:00:00

pages

586-94

issue

7

eissn

0148-639X

issn

1097-4598

journal_volume

8

pub_type

杂志文章
  • Persistent and transient "conduction block" in motor neuron diseases.

    abstract::Although conduction block indicates dysfunction of peripheral nerve, it may occur in patients with clinically typical motor neuron disease. There are no universally accepted criteria to identify conduction block, so diagnosis may be difficult. In some peripheral neuropathies, conduction block persists over long period...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880160903

    authors: Lange DJ,Trojaborg W,McDonald TD,Blake DM

    更新日期:1993-09-01 00:00:00

  • Analysis of youtube as a source of information for peripheral neuropathy.

    abstract:INTRODUCTION:YouTube is an important resource for patients. No study has evaluated the information on peripheral neuropathy disseminated by YouTube videos. In this study, our aim was to perform a systematic review of information on YouTube regarding peripheral neuropathy. METHODS:The Web site (www.youtube.com) was sea...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24916

    authors: Gupta HV,Lee RW,Raina SK,Behrle BL,Hinduja A,Mittal MK

    更新日期:2016-01-01 00:00:00

  • Single fiber macro versus concentric trigger macro EMG: a comparison of methods.

    abstract::The values obtained from two different macro EMG methods were compared, and found to be different. Normally, macro electromyography (EMG) is performed with a modified single fiber (SF) needle using the SF potential as a trigger; a new method recommends a modified concentric needle and triggers on the concentric EMG si...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880150211

    authors: Nix WA,Scherer A

    更新日期:1992-02-01 00:00:00

  • Protection of human muscle acetylcholinesterase from soman by pyridostigmine bromide.

    abstract:INTRODUCTION:Pretreatment with pyridostigmine bromide (PB) of human intercostal muscle fibers exposed to the irreversible acetylcholinesterase (AChE) inhibitor soman was investigated. METHODS:Muscles were pretreated with 3 × 10(-6) M PB or saline for 20 minutes, then exposed to 10(-7) M soman for 10 minutes. RESULTS:...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21922

    authors: Maselli RA,Henderson JD,Ng J,Follette D,Graves G,Wilson BW

    更新日期:2011-04-01 00:00:00

  • Electrodiagnostic errors contribute to chronic inflammatory demyelinating polyneuropathy misdiagnosis.

    abstract:INTRODUCTION:Documentation of peripheral nerve demyelination is an important part of the chronic inflammatory demyelinating polyneuropathy (CIDP) diagnostic process. METHODS:We performed a retrospective analysis of patients referred with a diagnosis of CIDP who were found to have a different condition. Electrodiagnost...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25997

    authors: Allen JA,Ney J,Lewis RA

    更新日期:2018-04-01 00:00:00

  • Potential implications of a ciliary neurotrophic factor gene mutation in a German population of patients with motor neuron disease.

    abstract::The frequency of a recently described point mutation of the ciliary neurotrophic factor (CNTF) gene was investigated in a population of 154 German patients with motor neuron disease (MND). Twenty-two percent of the patients were heterozygous, 2% homozygous for the CNTF mutation. Since the gene defect is per se not lin...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(sici)1097-4598(199802)21:2<236::aid-mus12

    authors: Giess R,Goetz R,Schrank B,Ochs G,Sendtner M,Toyka K

    更新日期:1998-02-01 00:00:00

  • Permanent muscle weakness in McArdle disease.

    abstract::McArdle disease is an autosomal recessive muscle glycogenosis. In the typical clinical presentation, only exercise-related symptoms are noted. Nevertheless, permanent weakness may occur, usually late in life. In this study we report on the clinical and genetic features of fixed muscle weakness in McArdle disease. Amon...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21351

    authors: Nadaj-Pakleza AA,Vincitorio CM,Laforêt P,Eymard B,Dion E,Teijeira S,Vietez I,Jeanpierre M,Navarro C,Stojkovic T

    更新日期:2009-09-01 00:00:00

  • Use of a catchlike property of human skeletal muscle to reduce fatigue.

    abstract::This study compared the force output produced by variable frequency, short-duration trains (VFTs) of electrical pulses with the forces produced by constant frequency, short-duration trains (CFTs). Human quadriceps femoris muscle was stimulated with a 300-msec train of pulses once every second for 180 seconds. Each sub...

    journal_title:Muscle & nerve

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1002/mus.880140909

    authors: Binder-Macleod SA,Barker CB 3rd

    更新日期:1991-09-01 00:00:00

  • Spontaneous electromyographic activity of the tongue in amyotrophic lateral sclerosis.

    abstract:INTRODUCTION:Detection of denervation in muscles in the craniobulbar area is important to assure widespread lower motor neuron involvement in the diagnosis of amyotrophic lateral sclerosis (ALS). The value of spontaneous activity analysis in needle electromyography (EMG) of the tongue has been questioned in the recent ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.23781

    authors: Tankisi H,Otto M,Pugdahl K,Fuglsang-Frederiksen A

    更新日期:2013-08-01 00:00:00

  • Effects of insulin on protein synthesis in muscles from normal and dystrophic mice.

    abstract::Protein synthesis in soleus and extensor digitorum longus (EDL) muscles was measured in vitro to test the hypothesis that the lack of muscle protein accumulation in dystrophic conditions could be caused by a reduced sensitivity to insulin. We demonstrate that physiological insulin concentrations stimulate protein synt...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880060709

    authors: Ballard FJ,Nield MK,Tomas FM

    更新日期:1983-09-01 00:00:00

  • Sensory ganglionopathy with livedoid vasculopathy controlled by immunotherapy.

    abstract:INTRODUCTION:Livedoid vasculopathy is a rare dermatological condition characterized by painful ulceration, atrophic scarring, and persistent livedo reticularis. The pathogenesis is unclear. METHODS:We report a patient with biopsy-proven livedoid vasculopathy who developed a progressive sensory ganglionopathy with prof...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24452

    authors: Alix JJ,Hadjivassiliou M,Ali R,Slater D,Messenger AG,Rao DG

    更新日期:2015-02-01 00:00:00

  • McArdle's disease presenting as recurrent cryptogenic renal failure due to occult seizures.

    abstract::Myophosphorylase deficiency (McArdle's disease) may present with episodic renal failure following exertion. We present a case of adult-onset myophosphorylase deficiency in which recurrent bouts of renal failure could not be associated with any exertional events until a tonic seizure was witnessed after the fourth epis...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.10463

    authors: Walker AR,Tschetter K,Matsuo F,Flanigan KM

    更新日期:2003-11-01 00:00:00

  • Comparison of interpolation and central activation ratios as measures of muscle inactivation.

    abstract::The objective of this study was to investigate different methods of estimating muscle inactivation, derived from single and multiple voluntary contractions. Ten subjects performed maximal and submaximal leg extensor contractions to determine an interpolation (IT) or central activation ratio (CAR). A superimposed evoke...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.1090

    authors: Behm D,Power K,Drinkwater E

    更新日期:2001-07-01 00:00:00

  • Conduction block in neuralgic amyotrophy.

    abstract::We describe two cases of neuralgic amyotrophy with electrophysiological evidence of conduction block across the lower trunk of the brachial plexus. Low-output impedance stimulation of the cervical spinal roots in combination with collision was used to accurately demonstrate the conduction block. Complete electrophysio...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.1041

    authors: Watson BV,Nicolle MW,Brown JD

    更新日期:2001-04-01 00:00:00

  • Probabilistic muscle characterization using QEMG: application to neuropathic muscle.

    abstract::Clinicians who use electromyographic (EMG) signals to help determine the presence or absence of abnormality in a muscle often, with varying degrees of success, evaluate sets of motor unit potentials (MUPs) qualitatively and/or quantitatively to characterize the muscle in a clinically meaningful way. The resulting musc...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21456

    authors: Pino LJ,Stashuk DW,Boe SG,Doherty TJ

    更新日期:2010-01-01 00:00:00

  • Clinical electrophysiology of infantile botulism.

    abstract::Infantile botulism is a recently recognized cause of acute hypotonic paresis and respiratory failure in young infants. Electrophysiological testing has proven useful in early diagnosis in suspected cases by demonstrating abnormal neuromuscular transmission as is known to occur in botulism. Twenty-five infants with bac...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880060609

    authors: Cornblath DR,Sladky JT,Sumner AJ

    更新日期:1983-07-01 00:00:00

  • MRI of the brachial plexus in polyneuropathy associated with monoclonal gammopathy.

    abstract::On magnetic resonance (MR) imaging of the brachial plexus increased signal intensity and swelling of the brachial plexus has been found in chronic inflammatory demyelinating polyneuropathy (CIDP). Whether these proximal abnormalities are also present in the distal polyneuropathy associated with monoclonal gammopathy i...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.1149

    authors: Eurelings M,Notermans NC,Franssen H,Van Es HW,Ramos LM,Wokke JH,van den Berg LH

    更新日期:2001-10-01 00:00:00

  • TEA-sensitive potassium channels and inward rectification in regenerated rat sciatic nerve.

    abstract::Sucrose gap and intra-axonal recording techniques were used to identify the types of ion channels and inward rectification that are present in regenerated axons of adult (greater than 8 weeks) rat sciatic nerve after crush injury. In sucrose gap recordings, 4-aminopyridine (4-AP) led to slight broadening of the compou...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880140707

    authors: Gordon TR,Kocsis JD,Waxman SG

    更新日期:1991-07-01 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

  • Properties of extensor digitorum longus muscle and skinned fibers from adult and aged male and female Actn3 knockout mice.

    abstract::Absence of α-actinin-3, encoded by the ACTN3 "speed gene," is associated with poorer sprinting performance in athletes and a slowing of relaxation in fast-twitch muscles of Actn3 knockout (KO) mice. Our first aim was to investigate, at the individual-fiber level, possible mechanisms for this slowed relaxation. Our sec...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21778

    authors: Chan S,Seto JT,Houweling PJ,Yang N,North KN,Head SI

    更新日期:2011-01-01 00:00:00

  • The utility of various sensory nerve conduction responses in assessing brachial plexopathies.

    abstract::To determine which sensory nerve conduction studies (S-NCS) are helpful in detecting supraclavicular axon loss brachial plexopathies, we selected 53 cases (of 417 reviewed) in whom complicating factors were absent and which, by needle electrode examination findings, involved only a single "truncal" element (upper, mid...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880180813

    authors: Ferrante MA,Wilbourn AJ

    更新日期:1995-08-01 00:00:00

  • KANDID--an EMG decision support system--evaluated in a European multicenter trial.

    abstract::KANDID is an advanced EMG decision support system dedicated to the support of the clinical neurophysiologist during EMG examinations. It has facilities for test planning, automatized and structured data interpretation, EMG diagnosis, explanation, and reporting. In a prospective European multicenter field trial, the ag...

    journal_title:Muscle & nerve

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1002/mus.880160514

    authors: Vingtoft S,Fuglsang-Frederiksen A,Rønager J,Petrera J,Stigsby B,Willison RG,Jarratt JA,Fawcett PR,Schofield IS,Otte G

    更新日期:1993-05-01 00:00:00

  • Bcl-2 and bax immunohistochemistry in denervation-reinnervation and necrosis-regeneration of rat skeletal muscles.

    abstract::Bcl-2 and Bax immunohistochemistry was examined in the skeletal muscle of rats after cutting the sciatic nerve, as a model of denervation and reinnervation, and in the anterior tibialis muscle of rats after an intramuscular injection of metoclopramide, as a model of necrosis and regeneration of muscle fibers, to bette...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/1097-4598(200012)23:12<1862::aid-mus10>3.0

    authors: Olivé M,Ferrer I

    更新日期:2000-12-01 00:00:00

  • Electrical stimulation based on chronaxie reduces atrogin-1 and myoD gene expressions in denervated rat muscle.

    abstract::Denervation induces muscle fiber atrophy and changes in the gene expression rates of skeletal muscle. Electrical stimulation (ES) is a procedure generally used to treat denervated muscles in humans. This study evaluated the effect of ES based on chronaxie and rheobase on the expression of the myoD and atrogin-1 genes ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.20668

    authors: Russo TL,Peviani SM,Freria CM,Gigo-Benato D,Geuna S,Salvini TF

    更新日期:2007-01-01 00:00:00

  • Paraneoplastic lower motor neuronopathy associated with Hodgkin lymphoma.

    abstract:INTRODUCTION:Paraneoplastic lower motor neuronopathies have been reported rarely with Hodgkin lymphoma. METHODS:We report a case of rapidly progressive motor neuronopathy preceding the diagnosis of Hodgkin lymphoma. RESULTS:A 31-year-old woman developed subacute rapidly progressive quadriparesis. Electrodiagnostic st...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.23464

    authors: Flanagan EP,Sandroni P,Pittock SJ,Inwards DJ,Jones LK Jr

    更新日期:2012-11-01 00:00:00

  • Nemaline body myopathy caused by a novel mutation in troponin T1 (TNNT1).

    abstract:INTRODUCTION:Nemaline myopathy is a rare disorder characterized by skeletal muscle weakness of varying severity and onset, with the presence of nemaline rods on muscle biopsy. Congenital nemaline body myopathy due to mutations in TNNT1 has hitherto only been described as a result of a single founder mutation in patient...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24885

    authors: Abdulhaq UN,Daana M,Dor T,Fellig Y,Eylon S,Schuelke M,Shaag A,Elpeleg O,Edvardson S

    更新日期:2016-04-01 00:00:00

  • Central nervous system involvement in multifocal demyelinating neuropathy with persistent conduction block.

    abstract::We report the case of a 27-year-old man treated for bilateral optic neuritis 5 and 3 years before who within a few months developed sensorimotor disorders of the arms and legs characterized by asymmetric distribution and distal prominence. In addition to sensorimotor defects, which were particularly marked in the left...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880171106

    authors: Péréon Y,Jardel J,Guillon B,Guihéneuc P

    更新日期:1994-11-01 00:00:00

  • Function of thick and thin nerve fibers in carpal tunnel syndrome before and after surgical treatment.

    abstract::Present studies evaluated the functions of thick and thin nerve fibers in carpal tunnel syndrome (CTS) to determine whether data of thin nerve fibers can add further information to the diagnosis of CTS. Measurements were performed in 22 patients and 16 age-matched control subjects. Motor and sensory nerve conduction, ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880180210

    authors: Lang E,Spitzer A,Pfannmüller D,Claus D,Handwerker HO,Neundörfer B

    更新日期:1995-02-01 00:00:00

  • Analysis of force profile during a maximum voluntary isometric contraction task.

    abstract::This study analyses maximum voluntary isometric contraction (MVIC) and its measurement by recording the force profile during maximal-effort, 7-s hand-grip contractions. Six healthy subjects each performed three trials repeated at short intervals to study variation from fatigue. These three trials were performed during...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.10564

    authors: Househam E,McAuley J,Charles T,Lightfoot T,Swash M

    更新日期:2004-03-01 00:00:00

  • The utility of a point-of-care sural nerve conduction device for detection of diabetic polyneuropathy: A cross-sectional study.

    abstract:INTRODUCTION:Rapid and accessible methods for diagnosing diabetic polyneuropathy (DPN) have been developed, but not validated, in large cohorts of people with diabetes. METHODS:The performance of a point-of-care device (POCD) was studied in 168 patients with type 2 diabetes, estimating the sensitivity, specificity, po...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.26361

    authors: Kural MA,Andersen ST,Andersen NT,Andersen H,Charles M,Finnerup NB,Jensen TS,Tankisi H

    更新日期:2019-02-01 00:00:00