Recombinant growth hormone treatment of amyotrophic lateral sclerosis.

Abstract:

:Based on the known trophic effects of growth hormone (GH) on nerve and muscle 75 patients with ALS were treated for up to 18 months with synthetic human growth hormone (Protropin) or a placebo. The course of ALS was assessed serially using a quantitative (TQNE) neuromuscular and manual exam (MRC) and laboratory chemistries. Average insulin-related growth factor (IGF-I) values increased from 1.2 to 2.3 U/mL in the treated group. Surprisingly, serum insulin levels did not increase. Hyperglycemia was noted in only 2 patients of the 38 patients receiving hGH, and this resolved with cessation of treatment. Over the 12 months of treatment there were 11 deaths (6 controls, 5 treated). Survival analysis, performed approximately 12 months following cessation of treatment, did not reveal a difference between the treatment and placebo group. The TQNE scores declined inexorably in both the control and treated group. Retrospective analysis of the TQNE data indicated a poor prognosis for patients who lost arm strength early. A correlation between the TQNE and MRC scores was evident at early stages of motor unit loss, less so when muscle weakness was advanced.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Smith RA,Melmed S,Sherman B,Frane J,Munsat TL,Festoff BW

doi

10.1002/mus.880160608

subject

Has Abstract

pub_date

1993-06-01 00:00:00

pages

624-33

issue

6

eissn

0148-639X

issn

1097-4598

journal_volume

16

pub_type

临床试验,杂志文章,多中心研究,随机对照试验
  • Electrodiagnostic errors contribute to chronic inflammatory demyelinating polyneuropathy misdiagnosis.

    abstract:INTRODUCTION:Documentation of peripheral nerve demyelination is an important part of the chronic inflammatory demyelinating polyneuropathy (CIDP) diagnostic process. METHODS:We performed a retrospective analysis of patients referred with a diagnosis of CIDP who were found to have a different condition. Electrodiagnost...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25997

    authors: Allen JA,Ney J,Lewis RA

    更新日期:2018-04-01 00:00:00

  • Contractile speed and fatigue of adductor pollicis muscle in multiple sclerosis.

    abstract::The purpose of the study was to investigate differences in contractile speed, force, and fatigability of the adductor pollicis muscle between 12 patients with multiple sclerosis (MS) and 8 sedentary control subjects matched for age and gender. There were no differences between the patients with MS and control subjects...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.1129

    authors: de Ruiter CJ,Jongen PJ,van der Woude LH,de Haan A

    更新日期:2001-09-01 00:00:00

  • Feasibility of contact heat evoked potentials for detection of diabetic neuropathy.

    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

    authors: Wong MC,Chung JW

    更新日期:2011-12-01 00:00:00

  • Estimating electromyographic and heart rate fatigue thresholds from a single treadmill test.

    abstract:INTRODUCTION:The purposes of this study were to (1) develop a fatigue threshold based on electromyography (EMG) and heart rate (HR) responses for treadmill running from a single incremental test; and (2) propose a new fatigue threshold called the RV(EMGFT) and RV(HRFT). METHODS:Eleven men performed incremental treadmi...

    journal_title:Muscle & nerve

    pub_type: 临床试验,杂志文章

    doi:10.1002/mus.23345

    authors: Guffey DR,Gervasi BJ,Maes AA,Malek MH

    更新日期:2012-10-01 00:00:00

  • Ultrasound elastography assessment of the median nerve in leprosy patients.

    abstract:INTRODUCTION:We sought to compare median nerve elasticity between leprosy patients (LPs) and healthy volunteers (HVs) using ultrasound elastography (UE). METHODS:Two radiologists independently measured the strain ratio of the median nerve/flexor digitorum superficialis muscle (MN/FDSM) of 18 LP and 18 HV using real-ti...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25510

    authors: Nogueira-Barbosa MH,Lugão HB,Gregio-Júnior E,Crema MD,Kobayashi MTT,Frade MAC,Pavan TZ,Carneiro AAO

    更新日期:2017-09-01 00:00:00

  • Ultrasonography of sciatic nerve endometriosis.

    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

    authors: Arányi Z,Polyák I,Tóth N,Vermes G,Göcsei Z

    更新日期:2016-09-01 00:00:00

  • Red blood cell alterations in muscular dystrophy: the role of lipids.

    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

    authors: Plishker GA,Appel SH

    更新日期:1980-01-01 00:00:00

  • Quantitative measurement of muscle fiber composition in a normal population.

    abstract::To obtain normative muscle morphology data on a healthy population recruited from a population survey, we examined vastus lateralis biopsies from 58 men and 33 women, aged 26-67 years. Biopsies were measured with automated, computer-aided techniques. Data were analyzed according to gender and age, and the influence of...

    journal_title:Muscle & nerve

    pub_type: 临床试验,杂志文章

    doi:10.1002/mus.10373

    authors: Toft I,Lindal S,Bønaa KH,Jenssen T

    更新日期:2003-07-01 00:00:00

  • Skin biopsy in assessing meralgia paresthetica.

    abstract:INTRODUCTION:Meralgia paresthetica is a focal neuropathy caused by compression of the lateral femoral cutaneous nerve (LFCN). The disease can be difficult to assess by neurophysiological or imaging studies. METHODS:We studied 5 patients who presented to our neuromuscular clinic from April 2012 to December 2014 with a ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25044

    authors: Wongmek A,Shin S,Zhou L

    更新日期:2016-04-01 00:00:00

  • Posture-related changes of soleus H-reflex excitability.

    abstract::We investigated whether the modulatory effects of segmental and descending inputs on the soleus H reflex are modified by postural conditions. Fourteen healthy volunteers received a transcranial magnetic stimulus (TMS) or percutaneous electrical stimulation of the posterior tibial nerve (PTN), preceding by 0 to 400 ms ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(sici)1097-4598(200006)23:6<925::aid-mus13

    authors: Goulart F,Valls-Solé J,Alvarez R

    更新日期:2000-06-01 00:00:00

  • Genetic disorders of neuromuscular ion channels.

    abstract::Ion channels are complex proteins that span the lipid bilayer of the cell membrane, where they orchestrate the electrical signals necessary for normal function of the central nervous system, peripheral nerve, and both skeletal and cardiac muscle. The role of ion channel defects in the pathogenesis of numerous disorder...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审

    doi:10.1002/mus.10164

    authors: Kleopa KA,Barchi RL

    更新日期:2002-09-01 00:00:00

  • Clinical utility of reflex studies in assessing cervical radiculopathy.

    abstract::We prospectively studied the diagnostic utility of upper limb segmental reflexes in patients with suspected cervical radiculopathy (CR). Fifty-three patients (29 men and 24 women), referred for electrodiagnostic testing, were positive for at least one of four clinical criteria for CR: abnormal (1) history, (2) motor (...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(sici)1097-4598(199908)22:8<1075::aid-mus1

    authors: Miller TA,Pardo R,Yaworski R

    更新日期:1999-08-01 00:00:00

  • AAEM Minimonograph #13: H reflexes and F waves: physiology and clinical indications.

    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

    pub_type: 杂志文章,评审

    doi:10.1002/mus.880151102

    authors: Fisher MA

    更新日期:1992-11-01 00:00:00

  • Axonal transport in neuropathy.

    abstract::Recent studies on the distribution of labeled endogenous proteins in the experimental neuropathies induced by streoptozotocin diabetes, galactose feeding, zinc pyridinethione, 2,5-hexanedione, acrylamide, and p-bromophenylacetylurea (BPAU) have demonstrated an impaired build up of retrogradely transported material der...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880060214

    authors: Jakobsen J,Brimijoin S,Sidenius P

    更新日期:1983-02-01 00:00:00

  • Myopathies with abnormal mitochondria: a clinicopathologic classification.

    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

    pub_type: 杂志文章

    doi:10.1002/mus.880010513

    authors: Kamieniecka Z,Schmalbruch H

    更新日期:1978-09-01 00:00:00

  • Correlation of single-breath count test and neck flexor muscle strength with spirometry in myasthenia gravis.

    abstract:INTRODUCTION:Although formal spirometry is the gold standard for monitoring respiratory function in patients with myasthenia gravis (MG), such testing is often delayed or unavailable. There is a need for a simple bedside test that can accurately measure respiratory function. METHODS:We conducted a prospective, cross-s...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24929

    authors: Elsheikh B,Arnold WD,Gharibshahi S,Reynolds J,Freimer M,Kissel JT

    更新日期:2016-01-01 00:00:00

  • Switching stimulation patterns improves performance of paralyzed human quadriceps muscle.

    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

    authors: Scott WB,Lee SC,Johnston TE,Binder-Macleod SA

    更新日期:2005-05-01 00:00:00

  • Spinal irradiation does not inhibit distal axonal sprouting.

    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

    authors: Pamphlett RS

    更新日期:1988-05-01 00:00:00

  • Multiple forms of anti-acetylcholine receptor antibody in myasthenia gravis.

    abstract::Sera of patients with myasthenia gravis (MG) contain anti-acetylcholine receptor (AChR) IgG antibodies (Ab) which have different antigenic specificities. Three Ab types were detected: (1) MG-I, which forms immune complexes with AChR; (2) MG-C, which decreases binding of AChR to concanavalin A; and MG-B, which blocks a...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880040105

    authors: Mittag T,Massa T,Kornfeld P,Papatestas A,Bender A,Genkins G

    更新日期:1981-01-01 00:00:00

  • Limitations of 6-minute walk test reference values for spinal muscular atrophy.

    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

    authors: Goodwin AM,Cornett KMD,McKay MJ,Burns J,Garber CE,De Vivo DC,Montes J

    更新日期:2020-03-01 00:00:00

  • A novel CLCN1 mutation: P480T in a Japanese family with Thomsen's myotonia congenita.

    abstract::At least 50 disease-causing mutations in the skeletal muscle voltage-gated chloride channel gene (CLCN1), almost all of which originate from Caucasian families, have been identified. We investigated a Japanese family with Thomsen's myotonia congenita that included 16 affected individuals (8 men and 8 women) through fi...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/1097-4598(200103)24:3<357::aid-mus1006>3.0

    authors: Sasaki R,Ito N,Shimamura M,Murakami T,Kuzuhara S,Uchino M,Uyama E

    更新日期:2001-03-01 00:00:00

  • Ultrasound elastography for the evaluation of peripheral nerves: A systematic review.

    abstract::Peripheral nerve disorders are commonly encountered in clinical practice. Electrodiagnostic studies remain the cornerstone of the evaluation of nerve disorders. More recently, ultrasound has played an increasing complementary role in the neuromuscular clinic. Ultrasound elastography is a technique that measures the el...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.26624

    authors: Wee TC,Simon NG

    更新日期:2019-11-01 00:00:00

  • Permanent muscle weakness in McArdle disease.

    abstract::McArdle disease is an autosomal recessive muscle glycogenosis. In the typical clinical presentation, only exercise-related symptoms are noted. Nevertheless, permanent weakness may occur, usually late in life. In this study we report on the clinical and genetic features of fixed muscle weakness in McArdle disease. Amon...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21351

    authors: Nadaj-Pakleza AA,Vincitorio CM,Laforêt P,Eymard B,Dion E,Teijeira S,Vietez I,Jeanpierre M,Navarro C,Stojkovic T

    更新日期:2009-09-01 00:00:00

  • Safety of influenza and H1N1 vaccinations in patients with myasthenia gravis, and patient compliance.

    abstract::Myasthenia gravis (MG) exacerbations may be triggered by infections.In the winter of 2009/2010, the Israeli Ministry of Health recommended that each individual with a chronic condition be vaccinated against both the seasonal influenza and the H1N1 virus. We examined vaccination practices in 74 MG patients. Only 38 pat...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.22077

    authors: Auriel E,Regev K,Dori A,Karni A

    更新日期:2011-06-01 00:00:00

  • Sources of error in the diagnosis of Guillain-Barre syndrome.

    abstract::A careful monitoring of the accuracy of diagnosis in six cases of Guillain-Barre syndrome has shown that a substantial proportion of these patients initially diagnosed as having Guillain-Barre syndrome on the basis of characteristic clinical findings and an elevated level of protein in the spinal fluid had a neuropath...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880050206

    authors: Feit H,Tindall RS,Glasberg M

    更新日期:1982-02-01 00:00:00

  • Animal models of alcoholic neuropathy: morphologic, electrophysiologic, and biochemical findings.

    abstract::A chronic high alcohol intake was induced in rats through the use of two procedures: the schedule-induced polydipsia technique and the liquid diet technique. Rats consumed 11-12 g of ethanol per kilogram body weight per day for 16 to 18 weeks. Morphologic evidence of a mild distal axonal neuropathy in the ventral caud...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880020208

    authors: Bosch EP,Pelham RW,Rasool CG,Chatterjee A,Lash RW,Brown L,Munsat TL,Bradley WG

    更新日期:1979-03-01 00:00:00

  • Marked increase in CD44-highly positive cells in hyperplastic thymuses from patients with Myasthenia gravis.

    abstract::To investigate the role of the thymus in the pathogenesis of myasthenia gravis (MG), immunohistochemical expression of CD44, CD45R0, B7-1, and IL-2 was studied in: (1) hyperplastic thymuses of patients with MG whose symptoms markedly improved after thymectomy, (2) remnant thymuses of patients with MG whose symptoms di...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(sici)1097-4598(200004)23:4<507::aid-mus8>

    authors: Utsugisawa K,Nagane Y,Tohgi H

    更新日期:2000-04-01 00:00:00

  • Visualization of the diaphragm muscle with ultrasound improves diagnostic accuracy of phrenic nerve conduction studies.

    abstract:INTRODUCTION:Evaluation of phrenic neuropathy (PN) with phrenic nerve conduction studies (PNCS) is associated with false negatives. Visualization of diaphragmatic muscle twitch with diaphragm ultrasound (DUS) when performing PNCS may help to solve this problem. METHODS:We performed bilateral, simultaneous DUS-PNCS in ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24059

    authors: Johnson NE,Utz M,Patrick E,Rheinwald N,Downs M,Dilek N,Dogra V,Logigian EL

    更新日期:2014-05-01 00:00:00

  • Acute morphologic changes in orbicularis oculi muscle after doxorubicin injection into the eyelid.

    abstract::This study attempts to gain a better understanding of the primary cause of doxorubicin myotoxicity to aid in the development of a protocol to increase its effectiveness in treating muscle spasm diseases. The time course of acute injury to the orbicularis oculi muscle after injection of doxorubicin into the eyelids of ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880160708

    authors: McLoon LK,Luo XX,Wirtschafter J

    更新日期:1993-07-01 00:00:00

  • Measurement of intramuscular fat by muscle echo intensity.

    abstract:INTRODUCTION:The aim of this study was to compare ultrasound echo intensity (EI) with high-resolution T1 -weighted MRI and to establish calibration equations to estimate percent intramuscular fat from EI. METHODS:Thirty-one participants underwent both ultrasound and MRI testing of 4 muscles: rectus femoris (RF); bicep...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24656

    authors: Young HJ,Jenkins NT,Zhao Q,Mccully KK

    更新日期:2015-12-01 00:00:00