Abstract:
INTRODUCTION:In this study we compare the ultrasound features in the median nerve in patients with different types of Charcot-Marie-Tooth (CMT) disease and hereditary neuropathies with liability to pressure palsies (HNPP) as a typical entrapment neuropathy. METHODS:Median nerve ultrasound and conduction studies were performed in patients with CMT1A (n = 12), MFN2-associated CMT2A (n = 7), CMTX (n = 5), and HNPP (n = 5), and in controls (n = 28). RESULTS:Median nerve cross-sectional area (CSA) was significantly increased in CMT1A, whereas, in axonal CMT2A, fascicle diameter (FD) was enlarged. CSA correlated with nerve conduction slowing in CMT1A and with axonal loss, as shown by motor and sensory nerve amplitudes in both CMT1A and CMT2A. A relatively low wrist-to-forearm-ratio (WFR <0.8) or a relatively high WFR (>1.8) appeared to be unlikely in MFN2 and Cx32 mutations of CMT2A and CMTX, respectively. CONCLUSION:Differences in CSA, FD, and WFR of the median nerve can be helpful in defining subtypes of hereditary neuropathies.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Schreiber S,Oldag A,Kornblum C,Kollewe K,Kropf S,Schoenfeld A,Feistner H,Jakubiczka S,Kunz WS,Scherlach C,Tempelmann C,Mawrin C,Dengler R,Schreiber F,Goertler M,Vielhaber Sdoi
10.1002/mus.23681subject
Has Abstractpub_date
2013-03-01 00:00:00pages
385-95issue
3eissn
0148-639Xissn
1097-4598journal_volume
47pub_type
杂志文章相关文献
MUSCLE & NERVE文献大全abstract::Calpain 3 (C3) is the only muscle-specific member of the calcium-dependent protease family. Although neither its physiological function nor its in vivo substrates are known, C3 must be an important protein for normal muscle function as mutations in the C3 gene result in limb-girdle muscular dystrophy type 2A. Previous...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.10465
更新日期:2003-10-01 00:00:00
abstract::The clinical efficacy of functional electrical stimulation (FES) is limited by the rapid onset of fatigue. Functional electrical stimulation applications typically stimulate skeletal muscles with constant-frequency trains (CFTs). Our laboratory has identified trains that we call doublet-frequency trains (DFTs) and tha...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20300
更新日期:2005-05-01 00:00:00
abstract:INTRODUCTION:Similar to magnetic resonance imaging (MRI), electrical impedance myography (EIM) is dependent on the presence and location of water in muscle to assess neuromuscular diseases. We compared the 2 technologies in mdx mice to better understand their relationship. METHODS:EIM and MRI, using T2 relaxation and ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24573
更新日期:2015-10-01 00:00:00
abstract:INTRODUCTION:Low-intensity electrical stimuli of digital nerves may generate a double peak potential (DPp), composed of a cathodal (caAP) and an anodal (anAP) potential in orthodromic recordings. METHODS:We studied the effects on caAP and anAP of stimuli of variable intensity, duration, and frequency. We also applied ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24957
更新日期:2016-06-01 00:00:00
abstract::Morvan's syndrome is a rare disorder characterized by neuromyotonia, hyperhidrosis, and central nervous system dysfunction. We report a patient with features of this syndrome, but who initially presented with breathing difficulties. Concentric needle electromyography showed an abundance of myokymic and neuromyotonic d...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20081
更新日期:2004-08-01 00:00:00
abstract:INTRODUCTION:Nebulin is a giant actin-binding protein in the thin filament of the skeletal muscle sarcomere. Studies of nebulin interactions are limited by the size, complexity, and poor solubility of the protein. We divided the nebulin super-repeat region into a super-repeat panel, and studied nebulin/actin interactio...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26350
更新日期:2019-01-01 00:00:00
abstract:INTRODUCTION:Changes in subsynovial connective tissue (SSCT) in carpal tunnel syndrome (CTS) patients may result in altered dynamics; consequently, quantification of these dynamics might support objective diagnosis of CTS. METHODS:We measured and compared longitudinal excursion of the flexor digitorum superficialis an...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23246
更新日期:2012-05-01 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::Impaired torque production is a major physical impairment following stroke, and has been studied extensively in isometric conditions. However, functional use of a limb requires torque production during movement, and the effects of velocity on maximal torque production may be abnormally enhanced in the paretic limb. Th...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20157
更新日期:2004-12-01 00:00:00
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
更新日期:2017-12-01 00:00:00
abstract:BACKGROUND:Our pilot study tested the feasibility and performance of an eye-controlled power wheelchair for amyotrophic lateral sclerosis (ALS) patients. METHODS:In this prospective pilot study, participants drove the wheelchair three times around an indoor course. We assessed the time to complete the course; starting...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26655
更新日期:2019-11-01 00:00:00
abstract::Congenital myasthenic syndromes (CMS) stem from defects in presynaptic, synaptic basal lamina, and postsynaptic proteins. The presynaptic CMS are associated with defects that curtail the evoked release of acetylcholine (ACh) quanta or ACh resynthesis. Defects in ACh resynthesis have now been traced to mutations in cho...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.10269
更新日期:2003-01-01 00:00:00
abstract::The dystrophin protein complex (DPC), composed of at least 10 proteins that associate with dystrophin, is critical for the maintenance of normal muscle fiber structure and physiology. In this study, we used immunohistochemistry and confocal microscopy to examine the relative abundance and distribution of several of th...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/1097-4598(200102)24:2<262::aid-mus120>3.0.
更新日期:2001-02-01 00:00:00
abstract::Normal motor units (MUs) were simulated and their architecture altered to simulate the changes produced by myopathy. The concentric needle electromyographic recordings of motor unit action potentials (MUAPs) from the MUs were then also simulated. These simulated MUAPs showed features that are seen in myopathy: normal ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880120306
更新日期:1989-03-01 00:00:00
abstract::In an attempt to determine the relative importance of the nerve cell body and of the axon in initiating and controlling axonal regeneration, nerve cell bodies were irradiated and the ability of the distal axon to sprout was examined. Mice were subjected to either 25 or 50 Gray (Gy) of x-irradiation localized to the lu...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880110513
更新日期:1988-05-01 00:00:00
abstract::To elucidate whether the frontal components of scalp somatosensory evoked potentials (SEPs) depend on the type of peripheral input, we compared scalp SEPs in response to electrical stimuli applied to: (i) the proximal phalanx of the thumb, involving both deep and cutaneous afferents; and (ii) the distal phalanx of the...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199907)22:7<910::aid-mus15
更新日期:1999-07-01 00:00:00
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
更新日期:2017-07-01 00:00:00
abstract:BACKGROUND:Anti-cytosolic 5'-nucleotidase 1A (cN1A) antibodies are commonly detected in patients with sporadic inclusion body myositis (sIBM). However, their pathogenic role has not been established. Moreover, efforts toward identifying sIBM distinct clinicopathologic characteristics associated with these antibodies ha...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.27157
更新日期:2020-12-29 00:00:00
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
更新日期:2017-08-01 00:00:00
abstract:INTRODUCTION:We describe the ultrasonographic findings of sciatic nerve endometriosis. METHODS:Two premenopausal women with catamenial sciatica symptoms were examined, the first without a history of endometriosis, the second with previously confirmed endometriosis of the ovary. Ultrasonography, extending from the scia...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25152
更新日期:2016-09-01 00:00:00
abstract::Beta-chemokines attract and activate T cells and monocytes and have a key role in chronic inflammation. Certain beta-chemokines, such as monocyte chemoattractant protein-1 (MCP-1), have been reported to be upregulated in the idiopathic inflammatory myopathies (IIM). We studied the distribution of beta-chemokine recept...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20294
更新日期:2005-05-01 00:00:00
abstract::Cerebral palsy (CP) is the most prevalent neurologic disease in children and a leading cause of severe physical disability. Research and clinical experience indicate that children with CP have abnormal neuromuscular junctions (NMJs), and we present evidence that nonapposition of neuromuscular junction components is as...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20401
更新日期:2005-11-01 00:00:00
abstract::Phospholipid methylation is thought to modulate such vital cellular processes as calcium transport, receptor function, and membrane microviscosity. As these processes are fundamental to the function of muscle cells and are thought to be altered in disease states, we have characterized several features of phospholipid ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880080513
更新日期:1985-06-01 00:00:00
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
更新日期:2017-12-01 00:00:00
abstract::Limb-girdle muscular dystrophy 2D (LGMD2D) is caused by mutations in the alpha-sarcoglycan gene (SGCA). The most frequently reported mutation, 229CGC>TGC (R77C) in exon 3 of SGCA, results in the substitution of arginine by cysteine. We present here the clinical, immunohistochemical, and genetic data of 11 Finnish pati...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20267
更新日期:2005-02-01 00:00:00
abstract:INTRODUCTION:Cerebral palsy (CP) is associated with reduced muscle volumes, but previous studies have reported deficits in only a small number of muscles. The extent of volume deficits across lower limb muscles is not known. This study presents an imaging-based assessment of muscle volume and length deficits in 35 lowe...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24972
更新日期:2016-06-01 00:00:00
abstract::Spatial distribution of motor endplates affects the shape of the electrical activity recorded from muscle. In order to provide information for realistic models of action potential propagation within muscles, we assembled three-dimensional maps of the motor endplates of the rat medial gastrocnemius (MGM) and lateral ga...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20378
更新日期:2005-09-01 00:00:00
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
更新日期:2017-10-01 00:00:00
abstract::Acetylcholine receptor (AChR) metabolism was studied in muscle from juvenile and adult dogs with congenital myasthenia gravis (CMG) and their unaffected littermates. Although the amount of AChR in the junctional region of innervated CMG muscle fibers was 25% of normal, or less, denervation of CMG fibers resulted in th...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880070905
更新日期:1984-11-01 00:00:00
abstract::Ischemia-reperfusion injury is implicated in the failure of free flap and replant surgeries and is associated with the pathogenesis of a wide variety of clinical diseases including stroke, myocardial infarction, spinal injury, and compartment syndromes. We used a skeletal muscle flap model to test if the induction of ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199903)22:3<390::aid-mus12
更新日期:1999-03-01 00:00:00