Congenital myasthenia: further evidence of disease heterogeneity.

abstract：INTRODUCTION:In mitochondrial disease, it is likely that energy substrate depletion leads to paralysis of ATPase-dependent pumps, resulting in membrane depolarization. Axonal depolarization has been demonstrated in a crisis, but not in the resting state. We, therefore, stressed axons using ischemia to see if this would...

journal_title：Muscle & nerve

authors： Ng K,Kumar KR,Sue C,Burke D

更新日期：2013-05-01 00:00:00

abstract：:A patient with neuromyotonia, peripheral neuropathy and myasthenia gravis (MG) is described. Neurophysiological studies, at rest, showed continuous muscle discharges of motor unit action potentials (MUAPs) in duplets and triplets. Motor (MNCV) and sensory (SNCV) nerve conduction studies revealed mild axonal and demyel...

journal_title：Muscle & nerve

authors： Martinelli P,Patuelli A,Minardi C,Cau A,Riviera AM,Dal Pozzo F

更新日期：1996-04-01 00:00:00

abstract：:Eleven patients with previous polio were studied. The concentration of energy-related metabolites and energy charge was measured from the vastus lateralis muscle, as was isometric muscle strength of knee extension. Cross-sectional area of the quadriceps femoris muscle was calculated from magnetic resonance imaging. Re...

journal_title：Muscle & nerve

authors： Nordgren B,Falck B,Stålberg E,Ronquist G,Waldenström A,Ahlström H,Hemmingson A

更新日期：1997-11-01 00:00:00

abstract：:The spectrum of neuromuscular disorders among intensive care unit (ICU) patients has shifted toward disorders acquired within the ICU and away from "traditional" neuromuscular disorders that lead to ICU admission. We sought to assess this spectrum by determining the causes and relative frequencies of neuromuscular dis...

journal_title：Muscle & nerve

authors： Lacomis D,Petrella JT,Giuliani MJ

更新日期：1998-05-01 00:00:00

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

authors： Guyon JR,Kudryashova E,Potts A,Dalkilic I,Brosius MA,Thompson TG,Beckmann JS,Kunkel LM,Spencer MJ

更新日期：2003-10-01 00:00:00

abstract：INTRODUCTION:We investigated the possible role of intercostal surgical neurolysis in relieving chronic neuropathic pain refractory to other nonsurgical treatments in patients with postsurgical thoracic pain. METHODS:We retrospectively collected clinical data on patients referred to the Neurosurgery Unit of Policlinic ...

journal_title：Muscle & nerve

authors： Cappellari AM,Tiberio F,Alicandro G,Spagnoli D,Grimoldi N

更新日期：2018-11-01 00:00:00

abstract：:Changes in contractile rate of force development (RFD), measured within a short time interval from contraction initiation, were measured after a period of strength training that led to increases in muscle fascicle length but no measurable change in neuromuscular activity. The relationship between training-induced shif...

journal_title：Muscle & nerve

authors： Blazevich AJ,Cannavan D,Horne S,Coleman DR,Aagaard P

更新日期：2009-04-01 00:00:00

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

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

更新日期：2017-09-01 00:00:00

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

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

更新日期：2015-02-01 00:00:00

abstract：INTRODUCTION:Skin-derived precursor cells (SKPs) are neural crest progenitor cells that can attain a Schwann cell-like phenotype through in vitro techniques (SKP-SCs). We hypothesized that SKP-SCs could produce mature myelin and, in doing so, facilitate the recovery of a focal demyelination injury. METHODS:We unilater...

journal_title：Muscle & nerve

authors： Grochmal J,Dhaliwal S,Stys PK,van Minnen J,Midha R

更新日期：2014-08-01 00:00:00

abstract：:Data are scarce relating to the plasticity with strength training of dynamic muscle performance in older humans. Hence, we investigated alterations in the torque-velocity relation with strength training in old age, and their origin. Knee extension and leg-press exercises were performed three times per week for 14 week...

journal_title：Muscle & nerve

authors： Reeves ND,Maganaris CN,Narici MV

更新日期：2005-03-01 00:00:00

abstract：INTRODUCTION:We sought to define the whole-body MRI (WB-MRI) fingerprint of muscle involvement in pediatric LMNA-related dystrophy (LMNA-RD) and to compare it with SEPN1-related myopathy (SEPN1-RM). METHODS:Signal abnormality and atrophy in 109 muscles were scored by semiquantitative scales in 8 children with LMNA-RD ...

journal_title：Muscle & nerve

authors： Gómez-Andrés D,Dabaj I,Mompoint D,Hankiewicz K,Azzi V,Ioos C,Romero NB,Ben Yaou R,Bergounioux J,Bonne G,Richard P,Estournet B,Yves-Carlier R,Quijano-Roy S

更新日期：2016-08-01 00:00:00

abstract：:The first dorsal interosseous (FDI) and abductor digiti minimi (ADM) muscles are innervated by the same ulnar nerve, but studies have shown that the former is much more severely affected in amyotrophic lateral sclerosis. In this study, threshold tracking was used to investigate whether membrane properties differ betwe...

journal_title：Muscle & nerve

authors： Bae JS,Sawai S,Misawa S,Kanai K,Isose S,Kuwabara S

更新日期：2009-03-01 00:00:00

abstract：:The objective of this study was to examine the contribution of lower motor neuron (LMN) and upper motor neuron (UMN) dysfunction to weakness and impaired motor control in 27 patients with amyotrophic lateral sclerosis (ALS). Isometric strength was measured by dorsiflexor maximum voluntary contraction force (MVC). LMN ...

journal_title：Muscle & nerve

authors： Kent-Braun JA,Walker CH,Weiner MW,Miller RG

更新日期：1998-06-01 00:00:00

abstract：INTRODUCTION:The molecular mechanism of immune-mediated necrotizing myopathy (IMNM) remains unknown. Autophagy impairment, described in autoimmune diseases, is a key process in myofiber protein degradation flux and muscle integrity and has not been studied in IMNM. METHODS:Muscle biopsies from patients with IMNM (n = ...

journal_title：Muscle & nerve

authors： Girolamo F,Lia A,Annese T,Giannini M,Amati A,D'Abbicco D,Tampoia M,Virgintino D,Ribatti D,Serlenga L,Iannone F,Trojano M

更新日期：2019-09-01 00:00:00

abstract：:The sternocleidomastoid muscle (SCM) is one of the major muscles involved in producing abnormal head position in cervical dystonia patients. This study tested whether doxorubicin chemomyectomy, direct injection of doxorubicin into the SCM to permanently remove muscle fibers, has the potential to be a nonsurgical, perm...

journal_title：Muscle & nerve

authors： McLoon LK,Falkenberg JH,Dykstra D,Iaizzo PA

更新日期：1998-11-01 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

authors： Sawchak JA,Leung B,Shafiq SA

更新日期：1992-12-01 00:00:00

abstract：:Electrodiagnostic evaluation of patients with suspected polyneuropathy is useful for detecting and documenting peripheral abnormalities, identifying the predominant pathophysiology, and determining the prognosis for certain disorders. The electrodiagnostic classification of polyneuropathy is associated with morphologi...

journal_title：Muscle & nerve

authors： Donofrio PD,Albers JW

更新日期：1990-10-01 00:00:00

abstract：:To determine the role of personal variables as risk factors for carpal tunnel syndrome (CTS) and their relationship to severity of nerve conduction abnormality, we studied 210 consecutive symptomatic CTS patients and 320 controls subjects without CTS symptomatology or known systemic disorders. The CTS group was classi...

journal_title：Muscle & nerve

authors： Kouyoumdjian JA,Zanetta DM,Morita MP

更新日期：2002-01-01 00:00:00

abstract：INTRODUCTION:Measurement precision and accuracy of spinal reflexes plays an essential role in the clinical neurological examination. Reflexes are conventionally assessed either electromyographically or with rating scales. In this study we compared objective kinematic T-reflex and subjective assessments of patellar refl...

journal_title：Muscle & nerve

authors： Dafkin C,Green A,Kerr S,Veliotes D,McKinon W

更新日期：2013-01-01 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

authors： Showalter CJ,Engel AG

更新日期：1997-03-01 00:00:00

abstract：:The aim of this study was to define normal ranges of histological features in pediatric muscle in comparison with muscle demonstrating inflammatory changes. Sixteen pediatric muscle biopsy samples, considered normal by standard histology, were analyzed for the presence of inflammatory cells, and the expression of neon...

journal_title：Muscle & nerve

authors： Varsani H,Newton KR,Li CK,Harding B,Holton JL,Wedderburn LR

更新日期：2008-02-01 00:00:00

abstract：:There are several pathogenic mechanisms of peripheral nerve involvement in patients with monoclonal dysglobulinemia. Intranervous proliferation of malignant cells, immunoglobulin, or amyloid deposits in the endoneurial space can only be determined by examination of nerve biopsy specimens. We present clinical, electrop...

journal_title：Muscle & nerve

authors： Vallat JM,Magy L,Richard L,Piaser M,Sindou P,Calvo J,Ghorab K,Cros D

更新日期：2008-07-01 00:00:00

abstract：:Motoneurons can be activated both reflexly and antidromically following electrical stimulation of peripheral nerves. These H reflexes and F waves are clinically useful responses which interface at the level of the peripheral nerves and the spinal cord. Because these responses are commonly employed in the electrodiagno...

journal_title：Muscle & nerve

authors： Fisher MA

更新日期：1992-11-01 00:00:00

abstract：:Twenty-four adult albino rats were injected with tetanus toxin into the right gastrocnemius muscle and then subjected to sustained dorsiflexion of the right ankle joint for 2 to 14 days. Histologic examinations of the soleus after this procedure showed myopathic changes, characterized by variations in fiber diameters,...

journal_title：Muscle & nerve

authors： Mizuno Y,Chou SM

更新日期：1990-10-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to examine the coping strategies of 49 patients with amyotrophic lateral sclerosis (ALS) and the relationships of these strategies to their perceived health-related quality of life (HRQoL). METHODS:Forty-nine subjects were assessed for collection of demographic and medical data. ...

journal_title：Muscle & nerve

authors： Montel S,Albertini L,Spitz E

更新日期：2012-01-01 00:00:00

abstract：INTRODUCTION:Doppler ultrasonography (DU) has recently been shown to be useful in imaging carpal tunnel syndrome (CTS). In this study, we aim to characterize the changes seen after exercise and electrical stimulation. METHODS:Five patients with CTS were recruited with 5 age-matched subjects. DU was used to visualize t...

journal_title：Muscle & nerve

authors： Ng ES,Ng KW,Wilder-Smith EP

更新日期：2013-01-01 00:00:00

abstract：:Although the effect of low temperature on the peripheral nervous system has been systematically studied, the effect of high temperature has not. We investigated the effect of elevating limb temperature from 32 degrees C to 42 degrees C by performing sequential motor studies, antidromic sensory studies, and 3-Hz repeti...

journal_title：Muscle & nerve

authors： Rutkove SB,Kothari MJ,Shefner JM

更新日期：1997-04-01 00:00:00

abstract：:Morphological changes of the subneural apparatus (SNA) during denervation and reinnervation are demonstrated using the hamster peroneus longus muscle. One week after nerve section, synaptic grooves (SGs) were more shallow, with lowered sarcoplasmic ridges. After 4 weeks, the whole SNA area was elevated above the sarco...

journal_title：Muscle & nerve

authors： Matsuda Y,Oki S,Kitaoka K,Nagano Y,Nojima M,Desaki J

更新日期：1988-12-01 00:00:00

abstract：:Chronic inflammatory demyelinating polyneuropathy (CIDP) is usually characterized by prominent motor deficits. A pure sensory presentation, labeled chronic sensory demyelinating neuropathy (CSDN), has been reported, but it is unclear if this neuropathy is a distinct clinical and immunologic entity or merely the sensor...

journal_title：Muscle & nerve

authors： Berger AR,Herskovitz S,Kaplan J

更新日期：1995-04-01 00:00:00