Abstract:
INTRODUCTION:Spinal and bulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS) are motor neuron diseases. Sensory impairment is sometimes observed, and electrophysiological involvement has been described in the sensory nerves of SBMA patients. We hypothesized that a sensory nerve conduction study (NCS) could be used to discriminate SBMA from ALS. METHODS:We compared the results from NCSs in a total of 120 SBMA cases confirmed by genetic analysis, 188 ALS cases, and 50 normal subjects. RESULTS:Sensory nerve action potential (SNAP) amplitudes of the SBMA group were significantly lower than in the ALS and control groups. In addition, receiver-operating characteristic curve analysis for SNAP amplitude showed that a cut-off value of 13.8 μV for median, 10.7 μV for ulnar, and 9.9 μV for sural nerve best discriminated SBMA from ALS. CONCLUSIONS:The specific decrease of SNAP amplitude in SBMA provides another useful tool for the differential diagnosis of motor neuron diseases.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Hama T,Hirayama M,Hara T,Nakamura T,Atsuta N,Banno H,Suzuki K,Katsuno M,Tanaka F,Sobue Gdoi
10.1002/mus.22291subject
Has Abstractpub_date
2012-02-01 00:00:00pages
169-74issue
2eissn
0148-639Xissn
1097-4598journal_volume
45pub_type
杂志文章相关文献
MUSCLE & NERVE文献大全abstract:INTRODUCTION:We examined the extent to which fatiguing cycling exercise in the heat influences contractile function in modulating the force-frequency relationship. METHODS:Before (∽37.0 °C) and after (∽38.5 °C) exercise (ExH) and passive (PaH) hyperthermia, an 8-s train of stimulation at 10, 20, 50, and 100 Hz (2 s pe...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24228
更新日期:2014-11-01 00:00:00
abstract:INTRODUCTION:Previous studies of limb-girdle muscular dystrophy type 2A (LGMD2A) patients in many countries have suggested a heterogeneous genetic and clinical spectrum, but the genotypes and phenotypes of Chinese LGMD2A patients remain unclear. METHODS:We directly screened calpain-3 (CAPN3) in 18 Chinese Han subjects...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23381
更新日期:2012-11-01 00:00:00
abstract::The synthesized pyrimidine compound MS-818 has neurotrophic effects in several kinds of neuronal cells, but its effect with respect to muscle cells remains unknown. We therefore examined the effects of MS-818 on regeneration for 12 weeks in a wounded area (damaged and gap areas) of cut muscle in adult rats. The right ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.10028
更新日期:2002-02-01 00:00:00
abstract::This article describes nerve conduction studies of the deep temporal nerve (DTN) and the mylohyoid nerve (MHN) motor branches of the trigeminal nerve. These nerves were stimulated intraorally with a pediatric surface stimulator. Compound muscle action potentials were recorded over the temporalis and mylohyoid muscles ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880190302
更新日期:1996-03-01 00:00:00
abstract:BACKGROUND:We aimed to describe the electrophysiological progression rate of chronic idiopathic axonal polyneuropathy (CIAP) and look into the potential role of human leukocyte antigen (HLA) genetic susceptibility in its development. METHODS:We recruited 57 patients with CIAP (mean age at diagnosis 67, mean follow-up ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.27164
更新日期:2021-01-13 00:00:00
abstract::Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and membranous glomerulonephritis (MGN) are both autoimmune disorders that are rarely observed concurrently. We describe a patient who developed MGN nearly 20 years after the onset of CIDP, resulting in a secondary progression of his neuropathy. He respo...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20841
更新日期:2008-01-01 00:00:00
abstract::We studied the effects of a 12-week progressive resistance strength training program in weakened muscles of 5 patients with sporadic inclusion body myositis (IBM). Strength was evaluated with Medical Research Council (MRC) scale ratings and quantitative isometric and dynamic tests. Changes in serum creatine kinase (CK...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199710)20:10<1242::aid-mus
更新日期:1997-10-01 00:00:00
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 p...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23681
更新日期:2013-03-01 00:00:00
abstract::The loss of muscle strength with aging appears to be greater in the lower than upper limbs, but strength and its neural control have never been compared in the same population of individuals in both upper and lower limbs. The aim of this study was to investigate differences between eight young (20-31 years) and eight ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20151
更新日期:2004-11-01 00:00:00
abstract:INTRODUCTION:Muscle weakness and functional disability have not been evaluated in a population-based study of patients with myasthenia gravis (MG). METHODS:All patients with MG in a well-defined catchment area were identified in the Danish National Patient Registry. Of the 175 eligible patients, 90 participated and we...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26356
更新日期:2019-02-01 00:00:00
abstract:INTRODUCTION:In this study we aimed to determine the maximal isometric muscle strength of a healthy, normal-weight, pediatric population between 6 and 15 years of age using hand-held dynamometry to establish strength reference values. The secondary objective was determining the relationship between strength and anthrop...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25180
更新日期:2017-01-01 00:00:00
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
更新日期:1992-02-01 00:00:00
abstract::Action potentials of rabbit masseter motor units (n = 42) were registered at different jaw angles to examine whether the shape of the action potential is related to length of muscle fibers in motor units and depends on the intramuscular location of the motor unit. Twitches were elicited by stimulating motoneurons in t...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.1182
更新日期:2001-11-01 00:00:00
abstract::Ultrastructural, biochemical, and electrophysiological analyses were done on 12-14-month-old mice deficient for myelin-associated glycoprotein (MAG) to further characterize the neuropathy that develops as they age. Electron microscopy demonstrated normal myelin compaction and axonal degeneration in a large number of m...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.1159
更新日期:2001-10-01 00:00:00
abstract::The purpose of this study was to investigate whether the previously demonstrated heterogeneity of myosin heavy chain (MHC) in type 2B fibers of the superficial portion of the lateral gastrocnemius (SLG) muscle of the rat may be due to presence of type 2D/X fibers. Immunohistochemical identification of MHC heterogeneit...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880151210
更新日期:1992-12-01 00:00:00
abstract::Familial amyotrophic lateral sclerosis (ALS) with the autosomal-recessively inherited D90A CuZn-superoxide dismutase (CuZn-SOD) mutation is characterized by a stereotypic slowly progressive, distinctive phenotype and very slow central motor conduction. To determine the basis of this slowing, we assessed corticomotoneu...
journal_title:Muscle & nerve
pub_type: 临床试验,杂志文章
doi:10.1002/1097-4598(200101)24:1<89::aid-mus10>3.0.co
更新日期:2001-01-01 00:00:00
abstract::Type 1 diabetes mellitus (T1DM) is associated with a peripheral neuropathy that reduces nerve conduction velocity. This may impair high motor-unit discharge frequencies (MUDF), decrease muscle activation, and curtail the ability to sustain repetitive contractile tasks. We examined (1) whether MUDF, the contractile pro...
journal_title:Muscle & nerve
pub_type: 临床试验,杂志文章
doi:10.1002/mus.20919
更新日期:2008-02-01 00:00:00
abstract::Obese individuals have slowed conduction in the median nerve across the wrist, but the mechanism for this is not established. This case-control study of 27 obese subjects and 16 thin subjects was designed to test the hypothesis that obese individuals have higher carpal canal pressures and more median nerve swelling th...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20125
更新日期:2004-10-01 00:00:00
abstract::Influenza vaccination has been associated with adverse events including Guillain-Barré syndrome. Because the safety of influenza vaccination in patients with myasthenia gravis (MG) has not been established, some clinicians discourage vaccination for these patients. We explored whether the administration of influenza v...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.21440
更新日期:2009-12-01 00:00:00
abstract:INTRODUCTION:The involvement of small myelinated fibers in the early stages of diabetic neuropathy (DN) has been suggested, but few objective methods can identify minimal neuropathy. The purpose of this study was to determine whether there are differences in contact heat evoked potential (CHEP) parameters between healt...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.22192
更新日期:2011-12-01 00:00:00
abstract::As applied to skeletal muscle, stem cell therapy is a reincarnation of myoblast transfer therapy that has resulted from recent advances in the cell biology of skeletal muscle. Both strategies envisage the reconstruction of damaged muscle from its precursors, but stem cell therapy employs precursors that are earlier in...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.10243
更新日期:2003-02-01 00:00:00
abstract::Two brothers with the childhood variant of type II glycogenosis (GSD-IIb) treated with nutrition and exercise therapy (NET) from a young age showed an unusually benign course. Muscle biopsy from the older brother, which showed characteristic vacuolar glycogen accumulation at age 2, had reverted to normal by age 16. A ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20473
更新日期:2006-04-01 00:00:00
abstract::We investigated the possible role of extracellular matrix in specifying the expression of superfast myosin during cat jaw muscle regeneration. Equal proportions of muscle tissue from jaw and limb were minced together after killing cellular elements from one source. We allowed the mince to regenerate in the bed of a fa...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880140503
更新日期:1991-05-01 00:00:00
abstract::To determine if short TI (time to inversion) recovery (STIR) magnetic resonance imaging (MRI) is useful in assessing lower extremity (LE) denervation in subacute lumbar radiculopathy (LR), 25 subjects underwent lumbar spine MRI, LE STIR MRI and needle electromyography (EMG). In 23 (92%) subjects there was a positive c...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199709)20:9<1191::aid-mus1
更新日期:1997-09-01 00:00:00
abstract::Twenty patients with neurogenic disorders, polymyositis, or muscular dystrophies were assessed clinically and by CT imaging of limb, limb girdle, and trunk muscles, using a standard protocol. On each side of these patients 26 movements were graded by the MRC scale, and 20 muscles were assessed by CT imaging. The clini...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880180706
更新日期:1995-07-01 00:00:00
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
更新日期:2018-02-09 00:00:00
abstract:INTRODUCTION:X-linked myotubular myopathy (XLMTM), characterized by severe hypotonia, weakness, respiratory distress, and early mortality, is rare and natural history studies are few. METHODS:RECENSUS is a multicenter chart review of male XLMTM patients characterizing disease burden and unmet medical needs. Data were ...
journal_title:Muscle & nerve
pub_type: 杂志文章,多中心研究
doi:10.1002/mus.26018
更新日期:2018-04-01 00:00:00
abstract::The electrical characteristics of myotonia in skeletal muscle appear remarkably similar in a variety of natural disorders and animal models. Recent experimental work has made it clear, however, that the pathophysiologic mechanisms underlying these myotonic discharges can be quite different. A mechanistic classificatio...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:
更新日期:1982-01-01 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::In this pilot study we use T2-weighted magnetic resonance imaging (MRI) to identify possible peripheral nerve inflammation in 4 patients with diffuse chronic pain. In all 4 patients, there was an increase in median and ulnar nerve T2 signal intensity at the wrist (P < 0.05 vs. controls). Positive clinical signs of per...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.22221
更新日期:2011-12-01 00:00:00