Dysmorphic neuromuscular junctions associated with motor ability in cerebral palsy.

abstract：INTRODUCTION:Amyloidosis derived from transthyretin (TTR) molecules is typically caused by mutations of the TTR gene. METHODS:We describe an elderly patient with a severe length-dependent polyneuropathy that unexpectedly proved to be caused by wild-type transthyretin amyloidosis. RESULTS:The diagnosis was made by mus...

journal_title：Muscle & nerve

authors： Lam L,Margeta M,Layzer R

更新日期：2015-07-01 00:00:00

abstract：:It has been suggested that vibration causes small changes in muscle length, but to the best of our knowledge, these have yet to be demonstrated during whole-body vibration (WBV). This was an observational study to determine whether acute WBV would result in muscle lengthening. We hypothesized that acute WBV would incr...

journal_title：Muscle & nerve

authors： Cochrane DJ,Loram ID,Stannard SR,Rittweger J

更新日期：2009-09-01 00:00:00

abstract：:Of 185 patients with myopathy, 22 showed abnormal muscle mitochondria. In 12 of the 22 patients, all of whom had ocular myopathy or the ophthalmoplegia-plus syndrome, muscle biopsies contained 5%-25% "ragged red" fibers. In 4 patients with a facioscapulohumeral distribution of weakness, ragged red fibers were less num...

journal_title：Muscle & nerve

authors： Kamieniecka Z,Schmalbruch H

更新日期：1978-09-01 00:00:00

abstract：:Injuries to the lumbosacral plexus during labor and delivery have been reported in the literature for years, but have lacked electrophysiologic testing to substantiate the location of the nerve injury. We report 2 cases with comprehensive electrophysiologic testing which localizes the site of this obstetrical paralysi...

journal_title：Muscle & nerve

authors： Feasby TE,Burton SR,Hahn AF

更新日期：1992-08-01 00:00:00

abstract：:We have assessed the reliability of hand-held myometry in 33 patients with spinal muscular atrophy (SMA), testing elbow flexion, handgrip, three-point pinch, knee flexion, knee extension, and foot dorsiflexion, and determining intraclass correlation coefficients (ICC). Interrater reliability was high for upper limbs, ...

journal_title：Muscle & nerve

authors： Merlini L,Mazzone ES,Solari A,Morandi L

更新日期：2002-07-01 00:00:00

abstract：:The clinical and neurophysiological features of multifocal motor neuropathy (MMN) indicate selective involvement of motor axons, but pathological abnormalities in sensory axons suggest a more widespread disease process. The present study was undertaken to determine whether the focal abnormalities are associated with w...

journal_title：Muscle & nerve

authors： Cappelen-Smith C,Kuwabara S,Lin CS,Burke D

更新日期：2002-12-01 00:00:00

abstract：:Factors that affect the shape of the so-called "normal cloud" of the turns and amplitude measurements of the electromyographic interference pattern are investigated. As the force of voluntary contraction increases from low to moderate levels, the number of turns in the signal increase faster than does the mean amplitu...

journal_title：Muscle & nerve

authors： Nandedkar SD,Sanders DB,Stalberg EV

更新日期：1991-01-01 00:00:00

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

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

更新日期：2011-04-01 00:00:00

abstract：BACKGROUND:Despite use of qualitative laryngeal electromyography (LEMG) guided botulinum toxin A (BoNT-A) injection for treatment of adductor spasmodic dysphonia (AdSD), unsatisfactory injections and complete "misses" remain problematic. We aimed to determine if the quantitative LEMG measure of number of small segments...

journal_title：Muscle & nerve

authors： Dwyer CD,Leclerc AA,Nandedkar SD,Young VN,Rosen CA

更新日期：2020-12-31 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

authors： Chan KM,Amirjani N,Sumrain M,Clarke A,Strohschein FJ

更新日期：2003-03-01 00:00:00

abstract：INTRODUCTION:Premotor potentials (PMPs) precede compound muscle action potentials evoked from the second lumbrical muscle after median nerve stimulation. Although PMP has been identified as a median sensory nerve action potential, few reports have documented the significance of PMP parameters for diagnosing carpal tunn...

journal_title：Muscle & nerve

authors： Kodama M,Sasao Y,Tochikura M,Kasahara T,Koyama Y,Aono K,Fujii C,Hanayama K,Takahashi O,Kobayashi Y,Masakado Y

更新日期：2012-12-01 00:00:00

abstract：:Epidemiological studies of the carpal tunnel syndrome have generally overlooked the possibility of a familial occurrence. A prospective study was undertaken to determine the prevalence and significance of a positive family history of carpal tunnel syndrome. Seventy-five of 253 women and 40 of 168 men with a confirmed ...

journal_title：Muscle & nerve

authors： Radecki P

更新日期：1994-03-01 00:00:00

abstract：:We studied membrane ultrastructural localization of anionic phospholipids (AP) and sialic acid (SA) calcium binding sites in muscle biopsies from Duchenne muscular dystrophy (DMD) and 3 Becker's muscular dystrophy (BMD) patients using polymyxin B (PXB) and limulus polyphemus (LP) as cytochemical markers. We found that...

journal_title：Muscle & nerve

authors： Moggio M,Fagiolari G,Prelle A,Gallanti A,Sciacco M,Scarlato G

更新日期：1992-03-01 00:00:00

abstract：:Hereditary neuropathy with liability to pressure palsy (HNPP) is typified as isolated nerve palsies caused by trivial compression or trauma. It rarely presents in two extremities and even more infrequently affects all four limbs simultaneously. We present a patient who concurrently experienced right shoulder, left han...

journal_title：Muscle & nerve

authors： Crum BA,Sorenson EJ,Abad GA,Dyck PJ

更新日期：2000-06-01 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

authors： Hackman P,Juvonen V,Sarparanta J,Penttinen M,Aärimaa T,Uusitalo M,Auranen M,Pihko H,Alén R,Junes M,Lönnqvist T,Kalimo H,Udd B

更新日期：2005-02-01 00:00:00

abstract：:The development of the personal computer has simplified the process of quantitating sensory thresholds using various testing algorithms. We reviewed the technical aspects and reproducibility of different methods to determine threshold for light touch-pressure, vibration, thermal, and pain stimuli. Clinical uses and li...

journal_title：Muscle & nerve

authors： Chong PS,Cros DP

更新日期：2004-05-01 00:00:00

abstract：INTRODUCTION:Merkel cell carcinoma is a rare cutaneous, aggressive tumor. Although it shares many neuroendocrine features with small cell lung carcinoma, it has only occasionally been reported with paraneoplastic neurological syndromes. METHODS:A healthy 67-year-old man developed acute ataxia, vertigo, and nausea. Sub...

journal_title：Muscle & nerve

authors： Pavolucci L,Giannini G,Giannoccaro MP,Foschini MP,Lang B,Avoni P,Tinuper P,Vincent A,Liguori R

更新日期：2017-11-01 00:00:00

abstract：:False motor points (FMPs) can occur in intrinsic foot or hand muscles, causing spuriously prolonged distal motor latencies by misrepresenting the compound muscle action potential (CMAP) onset. We investigated the motor point (MP) and possible FMPs in abductor hallucis (AH) by three methods: (1) electrophysiologic mapp...

journal_title：Muscle & nerve

authors： Del Toro DR,Park TA

更新日期：1996-09-01 00:00:00

abstract：:Little attention has been paid to small-fiber dysfunction in carpal tunnel syndrome (CTS) although its symptoms are common. This study investigates vasomotor dysfunction, which is controlled by small nerve fibers, in patients with CTS compared with control subjects. Vasomotor function was quantified by measuring, with...

journal_title：Muscle & nerve

authors： Wilder-Smith EP,Fook-Chong S,Chew SE,Chow A,Guo Y

更新日期：2003-11-01 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

authors： Carter GT,Fritz RC

更新日期：1997-09-01 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

authors： Plishker GA,Appel SH

更新日期：1980-01-01 00:00:00

abstract：INTRODUCTION:We examined the effects of pain site and intensity on function in patients with myotonic dystrophy type 1 (DM1) and facioscapulohumeral muscular dystrophy (FSHD) and chronic pain. METHODS:Questionnaires assessing pain sites, pain extent (number of sites), pain intensity, and pain interference were complet...

journal_title：Muscle & nerve

authors： Miró J,Gertz KJ,Carter GT,Jensen MP

更新日期：2014-06-01 00:00:00

abstract：:We compared the compound motor action potentials (CMAPs) evoked in the biceps, triceps, and abductor digiti minimi (ADM) muscles by conventional electrical stimulation at Erb's point (EP), and by magnetic coil stimulation of the supraclavicular region in 11 normal subjects. We found that magnetic coil stimulation was ...

journal_title：Muscle & nerve

authors： Cros D,Gominak S,Shahani B,Fang J,Day B

更新日期：1992-05-01 00:00:00

abstract：:Injection of low-dose arachidonic acid into the rat femoral artery occludes the vasa nervorum of the tibial nerve and produces focal and generalized ischemia with transient effects on nerve conduction. Across a severely ischemic segment of the proximal tibial nerve there is a marked fall in amplitude of the compound m...

journal_title：Muscle & nerve

authors： Parry GJ,Linn DJ

更新日期：1986-05-01 00:00:00

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

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

更新日期：2003-11-01 00:00:00

abstract：:A case study of a 50-year-old man who awakened from aortobifemoral bypass graft surgery with painful burning and numbness of the left foot is reported. Angiography showed embolic occlusion of the left popliteal artery proximal to the knee. Nerve conduction studies performed 1 year after occlusion showed loss or severe...

journal_title：Muscle & nerve

authors： Levin KH

更新日期：1989-10-01 00:00:00

abstract：INTRODUCTION:Voltage-gated Kv1 potassium channel complex (VGKC) autoantibodies subtyped for leucine-rich glioma-inactivated 1 (LGI1), contactin-associated-proteinlike 2 (CASPR2), and Kv IgGs have a spectrum of neurological presentations. Painful polyneuropathy is seen in some patients, but nerve pathology descriptions ...

journal_title：Muscle & nerve

authors： Lahoria R,Pittock SJ,Gadoth A,Engelstad JK,Lennon VA,Klein CJ

更新日期：2017-04-01 00:00:00

abstract：INTRODUCTION:The ability to individualize recommendations or expectations of disease progression based on a patient's unique characteristics has merit for use in sporadic inclusion body myositis (sIBM). METHODS:Fifty-five subjects with sIBM completed a battery of strength and functional outcomes at 2 study visits. The...

journal_title：Muscle & nerve

authors： Alfano LN,Yin H,Dvorchik I,Maus EG,Flanigan KM,Mendell JR,Lowes LP

更新日期：2017-04-01 00:00:00

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

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

更新日期：1995-02-01 00:00:00

abstract：:In recent years, the following ideas have been expressed: (a) that all cases of a discrete, inherited neuromuscular syndrome should prove to be due to a single biochemical defect, (b) that any single biochemical defect should give rise only to one syndrome, and (c) that an enzymatic defect cannot give rise to a diseas...

journal_title：Muscle & nerve

authors： Kark RA,Becker DM

更新日期：1981-01-01 00:00:00