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 bacteriologically proven botulism were studied by uniform methods in our laboratory and characteristic electrophysiological abnormalities were found. Repetitive stimulation at 20 and 50 Hz was the most specific single test; 23 patients (92%) showed incremental responses. Stimulation at low rates was less specific. Concentric needle electromyography provided useful supplemental information. Short-duration, low-amplitude motor unit potentials were prominent in 22 patients (92%) accompanied by abnormal spontaneous activity in 13 patients (54%). Compound muscle action potential amplitudes were usually reduced, but motor and sensory conduction studies were otherwise normal. Electrodiagnostic testing demonstrated one or more characteristic abnormalities in all cases of infantile botulism. This constellation of electrophysiological abnormalities, combined with an appropriate clinical picture, was so distinctive as to allow early presumptive diagnosis of infant botulism, before the results of bacteriological testing were available.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Cornblath DR,Sladky JT,Sumner AJdoi
10.1002/mus.880060609subject
Has Abstractpub_date
1983-07-01 00:00:00pages
448-52issue
6eissn
0148-639Xissn
1097-4598journal_volume
6pub_type
杂志文章相关文献
MUSCLE & NERVE文献大全abstract:BACKGROUND:Diagnosing cervical radiculopathy (CR) can be difficult because of symptomatic overlap with peripheral neuropathies. In this retrospective observational study, we aimed to determine whether short-tau inversion recovery (STIR) magnetic resonance imaging (MRI) sequences are useful for detecting signs of denerv...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.27142
更新日期:2020-12-13 00:00:00
abstract::Many post-polio patients develop new muscle weakness decades after the initial illness. However, its mechanism and treatment are controversial. The purpose of this study was to test the hypotheses that: (1) after strength training, post-polio patients show strength improvement comparable to that seen in the healthy el...
journal_title:Muscle & nerve
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/mus.10327
更新日期:2003-03-01 00:00:00
abstract::Weakness is common in both hyper- and hypothyroidism, and skeletal muscle L-carnitine may play a role in this regard, as suggested by studies indicating abnormal levels of carnitine in serum and urine of patients with thyroid dysfunction. Skeletal muscle samples were obtained for carnitine analysis from control subjec...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20336
更新日期:2005-09-01 00:00:00
abstract::Immunocytochemical analysis of muscle specimens from 5 patients with acute quadriplegic myopathy indictes that depletion of either fast or slow myosin occurs in this disorder. The initial lesion consists of focal myosin loss in nonatrophic fibers. Other structural proteins (actin, titin, nebulin) are spared or affecte...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(SICI)1097-4598(199703)20:3<316::AID-MUS8>
更新日期:1997-03-01 00:00:00
abstract::The myogenic regulatory factor MyoD plays an important role in embryonic and adult skeletal muscle growth. Even though it is best known as a marker for activated satellite cells, it is also expressed in myonuclei, and its expression can be induced by a variety of different conditions. Several model systems have been u...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.21087
更新日期:2008-09-01 00:00:00
abstract::Epidemiological data suggest that prolonged exposure to cyclic lumbar flexion elicits a chronic neuromuscular disorder and disability in workers. This study provides a physiological and biomechanical assessment of various repetitions of cyclic lumbar flexion sessions as a risk factor for development of an acute neurom...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20629
更新日期:2006-11-01 00:00:00
abstract:INTRODUCTION:Tarsal tunnel syndrome (TTS) arises from tibial nerve damage under the flexor retinaculum of the fibro-osseus tunnel at the medial malleolus. It is notoriously difficult to diagnose, as many other foot pathologies result in a similar clinical picture. We examined the additional value of nerve ultrasound in...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24963
更新日期:2016-06-01 00:00:00
abstract::A review of our current knowledge of the etiology and pathogenesis of myasthenia gravis is presented, with particular emphasis on the immunological aspects of the disease. Part 1, published in this issue, deals with the clinical and genetic features of myasthenia gravis which led to the autoimmune theory of the etiolo...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.880010107
更新日期:1978-01-01 00:00:00
abstract::The aim of this study was to estimate normal jitter in voluntarily activated extensor digitorum communis (EDC) and orbicularis oculi (OOc) muscles using a disposable concentric needle electrode (CNE). The EDC of 67 normal subjects (22 males and 45 females, mean age 35.5 +/- 10.2 years) and the OOc of 50 normal subject...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.21043
更新日期:2008-06-01 00:00:00
abstract::Muscle biopsy and computerized tomography indicated that unilateral enlargement of the gastrocnemius muscle in a 12-year-old girl was the result of an infiltrating lipoma. This rare condition may cause diagnostic confusion before EMG and muscle biopsy studies are performed, but it can be readily distinguished from oth...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880010111
更新日期:1978-01-01 00:00:00
abstract:INTRODUCTION:Although there are numerous protocols to adjust the amplitude of the Hoffmann reflex (H-reflex) relative to the size of the direct motor response (M-response), the optimal stimulating location has not been described. We sought to determine the optimal positioning of the stimulating cathode when evoking the...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26308
更新日期:2018-12-01 00:00:00
abstract::We describe a 58-year-old male with a few years history of multifocal weakness in the upper limbs with minimal to absent sensory complaints. He was diagnosed as having multiple compressive neuropathies, which required repeated decompressive surgeries. Electrodiagnostic studies prior to diagnosis were limited to a few ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199806)21:6<813::aid-mus18
更新日期:1998-06-01 00:00:00
abstract::In median nerve lesions of the hand, electrophysiologic evaluation of the second lumbrical muscle can help to differentiate between lesions of the common digital nerves and the proper digital nerves, as illustrated by this case report. Median nerve conduction studies to the second lumbrical muscle can be performed by ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880090303
更新日期:1986-03-01 00:00:00
abstract:INTRODUCTION:The 6-minute walk test (6MWT) is a well-established clinical assessment of functional endurance, validated as a measure of walking ability in spinal muscular atrophy (SMA). The current availability of disease-modifying therapies for SMA indicates a growing need for normative reference data to compare SMA p...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26794
更新日期:2020-03-01 00:00:00
abstract:INTRODUCTION:Our aim in this study was to determine whether intravenous immunoglobulin (IVIg) or plasma exchange (PLEx) for treatment of neurologic disease is a trigger for thrombotic events. METHODS:Using administrative data from 2005 to 2014, we identified index admissions for thrombotic events. We performed case-cr...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26884
更新日期:2020-09-01 00:00:00
abstract::Dystrophic changes of Schwann cells and demyelination occurred in rats with chronic nerve edema induced by feeding a galactose-rich diet for two years. The mechanism for edema is the sorbitol pathway which generates osmotically active polyols from galactose or glucose. The blood-nerve barrier impedes diffusion of macr...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880060309
更新日期:1983-03-01 00:00:00
abstract:INTRODUCTION:An enlarged tongue (macroglossia) has been reported in advanced-stage patients with amyotrophic lateral sclerosis (ALS). METHODS:In this study we examined the prevalence of macroglossia and analyzed clinical correlations in 65 ALS patients on tracheostomy-invasive ventilation (TIV). RESULTS:Macroglossia ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25058
更新日期:2016-09-01 00:00:00
abstract:INTRODUCTION:Weakness in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) may be caused by decreases in muscle quantity and quality, but this has not been explored. METHODS:Twelve patients with CIDP (mean age 61 years) and 10 age-matched (mean age 59 years) control subjects were assessed for ankl...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26159
更新日期:2018-09-01 00:00:00
abstract:INTRODUCTION:The aim of this study was to assess whether peripheral neuropathy is a feature of glycogen storage disease type IIIa (GSD IIIa) in adult patients. METHODS:Medical records of a cohort of adult GSD IIIa patients who underwent electromyography (EMG) and nerve conduction studies (NCS) were reviewed, and the r...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24977
更新日期:2016-02-01 00:00:00
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
更新日期:2018-01-01 00:00:00
abstract:INTRODUCTION:Duchenne muscular dystrophy (DMD) is characterized by absence of the subsarcolemmal protein dystrophin, present in skeletal muscles and cardiomyocytes. We hypothesized that progressive respiratory and left ventricular (LV) insufficiencies in DMD could be parallel and interrelated phenomena. METHODS:We con...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26623
更新日期:2019-09-01 00:00:00
abstract:INTRODUCTION:Ulnar nerve displacement at the elbow causes overestimation of nerve conduction velocity. We hypothesized that this overestimation may cause false-negative results when the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) diagnostic criteria are used. METHODS:We investigated 9 ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.22131
更新日期:2011-10-01 00:00:00
abstract::In order to assess the clinical utility of trigemino-facial reflexes in lower facial muscles, we studied perioral reflexes to mechanical and electrical stimulation in 13 patients with spasmodic dysphonia and orofacial dyskinesia and in 7 healthy subjects. Mechanical stimulation of the upper lip of all patients and ele...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880150906
更新日期:1992-09-01 00:00:00
abstract::Mutations in the myelin protein zero gene (MPZ) are associated with certain demyelinating neuropathies, and in particular with Charcot-Marie-Tooth disease type 1B (CMT1B), Dejerine-Sottas syndrome, and congenital hypomyelination. MPZ mutations affecting the protein's transmembrane domain are generally associated with ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20034
更新日期:2004-06-01 00:00:00
abstract::Intramuscular injection of botulinum toxin A is used to treat focal dystonias. Because immunoresistance has been documented in some patients, other molecular forms of the toxin have been evaluated clinically. The present investigation compared the time course and extent of neuromuscular blockade and recovery of botuli...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.10213
更新日期:2002-09-01 00:00:00
abstract:BACKGROUND:Ankle foot orthoses (AFOs) are commonly prescribed to individuals with Charcot-Marie-Tooth disease (CMT). The aim of this study was to evaluate patient reported satisfaction with orthotic devices and services in individuals with CMT to provide preliminary data for advancing AFO development and improving clin...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.27027
更新日期:2021-01-01 00:00:00
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
更新日期:2017-05-01 00:00:00
abstract::Biochemical, morphologic, and biophysical studies support the concept that the red blood cell (RBC) membrane is altered in both myotonic muscular dystrophy (MyD) and Duchenne muscular dystrophy (DMD). These studies have not identified a primary metabolic defect that would explain the various alterations of membrane pr...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.880030108
更新日期:1980-01-01 00:00:00
abstract::Inflammatory myopathies (IMs) occur relatively frequently in dogs, and, with the exception of masticatory muscle myositis (MMM), have not been characterized. This study analyzed the distribution and types of cellular infiltrates in 21 cases of generalized IM, 3 cases of focal IM (MMM), and 1 case with features of both...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20043
更新日期:2004-06-01 00:00:00
abstract::The neuromuscular junction (NMJ) is a complex structure that serves to efficiently communicate the electrical impulse from the motor neuron to the skeletal muscle to signal contraction. Over the last 200 years, technological advances in microscopy allowed visualization of the existence of a gap between the motor neuro...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.20440
更新日期:2006-04-01 00:00:00