Branched-chain amino acids and amyotrophic lateral sclerosis: a treatment failure? The Italian ALS Study Group.

Abstract:

:We initiated a double-blind, placebo-controlled trial to test the efficacy and safety of branched-chain amino acids (BCAA) (L-leucine 12 g, L-isoleucine 6 g, and L-valine 6 g daily) in amyotrophic lateral sclerosis (ALS) patients. There was an excess mortality in subjects randomized to active treatment (24 BCAA, 13 placebo) when a total of 126 ALS patients had been recruited. This finding, associated with the lack of efficacy of BCAA (measured by comparing the disability scales in the two treatment groups), led the Data Monitoring Committee to require cessation of the trial.

journal_name

Neurology

journal_title

Neurology

authors

doi

10.1212/wnl.43.12.2466

subject

Has Abstract

pub_date

1993-12-01 00:00:00

pages

2466-70

issue

12

eissn

0028-3878

issn

1526-632X

journal_volume

43

pub_type

临床试验,杂志文章,随机对照试验
  • Midbrain deafness with normal brainstem auditory evoked potentials.

    abstract::The authors report two cases of patients with word deafness. The word deafness occurred after a head injury for the first patient and after an arterio venous malformation embolization for the second patient. MRI demonstrated bilateral lesions of the inferior colliculi but brainstem auditory-evoked potentials (BAEP) we...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.58.6.970

    authors: Vitte E,Tankéré F,Bernat I,Zouaoui A,Lamas G,Soudant J

    更新日期:2002-03-26 00:00:00

  • Prospective natural history study of C9orf72 ALS clinical characteristics and biomarkers.

    abstract:OBJECTIVE:To define the natural history of the C9orf72 amyotrophic lateral sclerosis (C9ALS) patient population, develop disease biomarkers, and characterize patient pathologies. METHODS:We prospectively collected clinical and demographic data from 116 symptomatic C9ALS and 12 non-amyotrophic lateral sclerosis (ALS) f...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000008359

    authors: Cammack AJ,Atassi N,Hyman T,van den Berg LH,Harms M,Baloh RH,Brown RH,van Es MA,Veldink JH,de Vries BS,Rothstein JD,Drain C,Jockel-Balsarotti J,Malcolm A,Boodram S,Salter A,Wightman N,Yu H,Sherman AV,Esparza TJ,Mc

    更新日期:2019-10-22 00:00:00

  • Cutaneous innervation in sensory neuropathies: evaluation by skin biopsy.

    abstract:OBJECTIVE:To use punch skin biopsies to evaluate the loss of intra-epidermal nerve fibers in sensory neuropathies. BACKGROUND:Previous assessments of epidermal nerve fibers have been constrained by relatively insensitive staining techniques and variability in quantification. METHODS:Punch skin biopsies were performed...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.45.10.1848

    authors: McCarthy BG,Hsieh ST,Stocks A,Hauer P,Macko C,Cornblath DR,Griffin JW,McArthur JC

    更新日期:1995-10-01 00:00:00

  • Restless legs syndrome: revisiting the dopamine hypothesis from the spinal cord perspective.

    abstract::Restless legs syndrome (RLS) involves abnormal limb sensations that diminish with motor activity, worsen at rest, have a circadian peak in expression in the evening and at night, and can severely disrupt sleep. Primary treatment is directed at CNS dopaminergic systems, particularly activation of D(2)-like (D(2), D(3),...

    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:10.1212/01.wnl.0000223316.53428.c9

    authors: Clemens S,Rye D,Hochman S

    更新日期:2006-07-11 00:00:00

  • Elevated cortical zinc in Alzheimer disease.

    abstract:OBJECTIVE:To determine whether changes in brain biometals in Alzheimer disease (AD) and in normal brain tissue are tandemly associated with amyloid beta-peptide (Abeta) burden and dementia severity. METHODS:The authors measured zinc, copper, iron, manganese, and aluminum and Abeta levels in postmortem neocortical tiss...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000223644.08653.b5

    authors: Religa D,Strozyk D,Cherny RA,Volitakis I,Haroutunian V,Winblad B,Naslund J,Bush AI

    更新日期:2006-07-11 00:00:00

  • Diffuse cerebral vasoconstriction (Call-Fleming syndrome) and stroke associated with antidepressants.

    abstract::Call-Fleming syndrome is a reversible segmental vasoconstriction of cerebral arteries manifested by a "thunderclap" headache and focal neurologic symptoms. Although of unknown etiology, it has been reported in association with vasoactive sympathomimetic drugs. The authors report Call-Fleming syndrome in two patients w...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000223648.76430.27

    authors: Noskin O,Jafarimojarrad E,Libman RB,Nelson JL

    更新日期:2006-07-11 00:00:00

  • Tetanus vaccination and risk of multiple sclerosis: a systematic review.

    abstract:OBJECTIVE:To conduct a systematic review on the association between tetanus vaccination and the risk of multiple sclerosis (MS). METHODS:The authors searched the databases Medline, LILACS, EMBASE, and Science Citation Index including the period 1966 to September 1, 2005. Eligible studies had to meet the following incl...

    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:10.1212/01.wnl.0000225079.51201.f9

    authors: Hernán MA,Alonso A,Hernández-Díaz S

    更新日期:2006-07-25 00:00:00

  • Kearns-Sayre syndrome and complex II deficiency.

    abstract::A 25-year-old woman with Kearns-Sayre syndrome (KSS) had complete external ophthalmoplegia, short stature, ataxia, cardiac conduction defects, and pigmentary retinopathy. Muscle biopsy revealed ragged-red fibers. Electron microscopy showed increased numbers of mitochondria with disordered structure and paracrystalline...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.39.5.693

    authors: Rivner MH,Shamsnia M,Swift TR,Trefz J,Roesel RA,Carter AL,Yanamura W,Hommes FA

    更新日期:1989-05-01 00:00:00

  • Cerebral microbleeds are a risk factor for warfarin-related intracerebral hemorrhage.

    abstract:BACKGROUND:Cerebral microbleeds are known to be indicative of bleeding-prone microangiopathy and may predict incident intracerebral hemorrhage (ICH). In this study, we investigated whether microbleeds are associated with the incidence of warfarin-related ICH. METHODS:Twenty-four patients with ICH while on outpatient t...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000339060.11702.dd

    authors: Lee SH,Ryu WS,Roh JK

    更新日期:2009-01-13 00:00:00

  • Estimation of the temporal probability of human immunodeficiency virus (HIV) dementia after risk stratification for HIV-infected persons.

    abstract::We developed a scheme using routinely available data to estimate the risk of human immunodeficiency virus (HIV) dementia in HIV-infected persons over time. We performed a longitudinal review of medical records from more than 100 medical facilities in 11 U.S. cities. A total of 19,462 HIV-infected persons without histo...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.50.2.392

    authors: Qureshi AI,Hanson DL,Jones JL,Janssen RS

    更新日期:1998-02-01 00:00:00

  • Vimentin and desmin in maturing skeletal muscle and developmental myopathies.

    abstract::I studied vimentin and desmin immunoreactivities in the skeletal muscle of 30 human fetuses and children ranging from 8 weeks' gestation to 2 years of age, and in 45 infants and children and five adults with developmental neuromuscular diseases. Acridine orange-RNA fluorescence also identified regenerating myofibers i...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.42.8.1616

    authors: Sarnat HB

    更新日期:1992-08-01 00:00:00

  • Lapse of consciousness and automatisms in temporal lobe epilepsy: a videotape analysis.

    abstract::Videotape analysis of 76 attacks in 14 epileptics showed two electro-clinical types of psychomotor attacks. The first and most common type had three clinical phases, consisting of an initial motionless stare, stereotyped movements, and reactive automatisms during impaired consciousness. The second and less common type...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.27.2.144

    authors: Escueta AV,Kunze U,Waddell G,Boxley J,Nadel A

    更新日期:1977-02-01 00:00:00

  • Autoimmune encephalitis: A costly condition.

    abstract:OBJECTIVE:To assess the inpatient hospitalization burden and costs of patients with autoimmune encephalitis (AE) at a tertiary care institution. METHODS:Adult inpatients with AE were identified retrospectively from July 1, 2005, to June 30, 2015. Demographic and clinical data were collected and analyzed. Billing data ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000006990

    authors: Cohen J,Sotoca J,Gandhi S,Yeshokumar AK,Gordon-Lipkin E,Geocadin RG,Frick KD,Probasco JC,Venkatesan A

    更新日期:2019-02-26 00:00:00

  • CSF1R mutations link POLD and HDLS as a single disease entity.

    abstract:OBJECTIVE:Pigmented orthochromatic leukodystrophy (POLD) and hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) are rare neurodegenerative disorders characterized by cerebral white matter abnormalities, myelin loss, and axonal swellings. The striking overlap of clinical and pathologic features of these...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e31828726a7

    authors: Nicholson AM,Baker MC,Finch NA,Rutherford NJ,Wider C,Graff-Radford NR,Nelson PT,Clark HB,Wszolek ZK,Dickson DW,Knopman DS,Rademakers R

    更新日期:2013-03-12 00:00:00

  • Cerebral small-vessel disease and progression of brain atrophy: the SMART-MR study.

    abstract:OBJECTIVES:To investigate whether severity and progression of periventricular and deep white matter lesions (WML) and lacunar infarcts were associated with progression of brain atrophy. METHODS:Within the SMART-MR study, a prospective cohort on MRI changes in patients with symptomatic atherosclerotic disease, 565 pati...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3182749f02

    authors: Kloppenborg RP,Nederkoorn PJ,Grool AM,Vincken KL,Mali WP,Vermeulen M,van der Graaf Y,Geerlings MI,SMART Study Group.

    更新日期:2012-11-13 00:00:00

  • Immunocytochemical analysis of the cellular infiltrate in brain lesions in subacute sclerosing panencephalitis.

    abstract::We studied lymphocyte subsets and major histocompatibility complex (MHC) class II antigens in frozen brain tissues from three patients with subacute sclerosing panencephalitis (SSPE) using immunocytochemical techniques. Perivascular cuffs consisted predominantly of T cells and MHC class II-positive cells. In the paren...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.41.10.1639

    authors: Nagano I,Nakamura S,Yoshioka M,Kogure K

    更新日期:1991-10-01 00:00:00

  • Simple MRI score aids prediction of dementia in cerebral small vessel disease.

    abstract:OBJECTIVE:To determine whether a simple small vessel disease (SVD) score, which uses information available on rapid visual assessment of clinical MRI scans, predicts risk of cognitive decline and dementia, above that provided by simple clinical measures. METHODS:Three prospective longitudinal cohort studies (SCANS [St...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000009141

    authors: Amin Al Olama A,Wason JMS,Tuladhar AM,van Leijsen EMC,Koini M,Hofer E,Morris RG,Schmidt R,de Leeuw FE,Markus HS

    更新日期:2020-03-24 00:00:00

  • Memory complaints and APOE-epsilon4 accelerate cognitive decline in cognitively normal elderly.

    abstract:OBJECTIVE:To investigate to what extent subjective memory complaints and APOE-epsilon4 allele carriage predict future cognitive decline in cognitively intact elderly persons, by evaluating both their separate and combined effects. METHODS:We selected 1,168 subjects from the population-based Longitudinal Aging Study Am...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.57.12.2217

    authors: Dik MG,Jonker C,Comijs HC,Bouter LM,Twisk JW,van Kamp GJ,Deeg DJ

    更新日期:2001-12-26 00:00:00

  • Physiologic events initiating REM sleep in patients with the postpolio syndrome.

    abstract:BACKGROUND:We previously studied the occurrence of muscle tone reduction (MTR), sawtooth waves (STW), and REM in sleep, and found a stereotypical sequence of these events in normal subjects. Patients with the postpolio syndrome may have involvement of the reticular formation in the brainstem, an area known to mediate i...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.52.3.516

    authors: Siegel H,McCutchen C,Dalakas MC,Freeman A,Graham B,Alling D,Sato S

    更新日期:1999-02-01 00:00:00

  • An autosomal dominant disorder with episodic ataxia, vertigo, and tinnitus.

    abstract::The authors report an autosomal dominant episodic ataxia that is clinically distinct from the other episodic ataxias. Vestibular ataxia, vertigo, tinnitus, and interictal myokymia are prominent; attacks are diminished by acetazolamide. Linkage analyses of markers flanking the EA1 and EA2 loci demonstrate genetic exclu...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.57.8.1499

    authors: Steckley JL,Ebers GC,Cader MZ,McLachlan RS

    更新日期:2001-10-23 00:00:00

  • Subarachnoid and intracerebral hemorrhage: natural history, prognosis, and precursive factors in the Framingham Study.

    abstract::No uniformly accepted hypothesis explains the genesis and rupture of intracranial aneurysms. We followed 5,184 men and women prospectively for 26 years; 36 cases of aneurysmal subarachnoid hemorrhage (SAH) accounted for 62% of all intracranial hemorrhages. Blood pressure before SAH was higher in these patients than in...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.34.7.847

    authors: Sacco RL,Wolf PA,Bharucha NE,Meeks SL,Kannel WB,Charette LJ,McNamara PM,Palmer EP,D'Agostino R

    更新日期:1984-07-01 00:00:00

  • Diagnosis of vestibular imbalance in the blink of an eye.

    abstract:BACKGROUND:In a recent study, the authors found that blinks in healthy volunteers always triggered ocular torsion quick phases during dynamic roll movements of the head. On the basis of this observation, they hypothesized that blinks in patients with a vestibular tone imbalance would also trigger torsional quick phases...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000141144.02666.8c

    authors: Schneider E,Glasauer S,Dieterich M,Kalla R,Brandt T

    更新日期:2004-10-12 00:00:00

  • A dose-comparison trial of topiramate as monotherapy in recently diagnosed partial epilepsy.

    abstract:OBJECTIVE:To evaluate topiramate as monotherapy in adults and children with recently diagnosed, localization-related epilepsy, comparing two dosages of topiramate in a multicenter, randomized, double-blind study. METHODS:Adults and children (>/=3 years of age) were eligible if the maximum interval since epilepsy diagn...

    journal_title:Neurology

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验

    doi:10.1212/01.wnl.0000048200.12663.bc

    authors: Gilliam FG,Veloso F,Bomhof MA,Gazda SK,Biton V,Ter Bruggen JP,Neto W,Bailey C,Pledger G,Wu SC

    更新日期:2003-01-28 00:00:00

  • The natural history of asymptomatic meningiomas in Olmsted County, Minnesota.

    abstract::To define the natural history of asymptomatic meningioma found incidentally on a neuroimaging study, we performed a retrospective analysis of 35 such patients. There were 32 women and three men, with a mean age of 67 years and a mean follow-up of 74 months. Four tumors had progressed on subsequent imaging, and one pat...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.51.6.1718

    authors: Go RS,Taylor BV,Kimmel DW

    更新日期:1998-12-01 00:00:00

  • Management and outcome of CSF-JC virus PCR-negative PML in a natalizumab-treated patient with MS.

    abstract:OBJECTIVE:To describe the diagnosis and management of a 49-year-old woman with multiple sclerosis (MS) developing a progressive hemiparesis and expanding MRI lesion suspicious of progressive multifocal leukoencephalopathy (PML) 19 months after starting natalizumab. RESULTS:Polyomavirus JC (JCV)-specific qPCR in CSF wa...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e31823b9b27

    authors: Kuhle J,Gosert R,Bühler R,Derfuss T,Sutter R,Yaldizli O,Radue EW,Ryschkewitsch C,Major EO,Kappos L,Frank S,Hirsch HH

    更新日期:2011-12-06 00:00:00

  • The effects of myasthenic immunoglobulins on neuromuscular transmission and thymus histology in mice.

    abstract::An autoimmune reaction to the endplate acetylcholine receptor (AChR) is thought to be responsible for the muscular weakness of myasthenia gravis (MG). However, the significance of antithymic antibodies and the thymic AChR-like protein is still uncertain. We transferred immunoglobulins (Igs) from patients with MG to mi...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.33.10.1356

    authors: Dalkara T,Onur R,Ruacan SA,Topaktaş S

    更新日期:1983-10-01 00:00:00

  • Primary systemic carnitine deficiency: I. Carnitine biosynthesis.

    abstract::[Methyl-3H]epsilon-N-trimethyl-L-lysine was administered to patients with primary systemic carnitine deficiency and to controls. In both groups, labeled carnitine appeared in blood and urine within 2 hours, and the specific radioactivity of urinary carnitine peaked between 2 and 6 hours. The specific radioactivity of ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.31.7.813

    authors: Rebouche CJ,Engel AG

    更新日期:1981-07-01 00:00:00

  • Immediate-release and controlled-release carbidopa/levodopa in PD: a 5-year randomized multicenter study. Carbidopa/Levodopa Study Group.

    abstract:OBJECTIVE:To compare effects of immediate-release (IR) and sustained-release (CR) carbidopa/levodopa in levodopa-naive PD patients. BACKGROUND:It was hypothesized that the long-acting preparation would be associated with fewer long-term complications. METHODS:A total of 618 patients were studied in 36 centers worldwi...

    journal_title:Neurology

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验

    doi:10.1212/wnl.53.5.1012

    authors: Koller WC,Hutton JT,Tolosa E,Capilldeo R

    更新日期:1999-09-22 00:00:00

  • Development and validation of risk index for cognitive decline using blood-derived markers.

    abstract:OBJECTIVE:We sought to develop and validate a risk index for prospective cognitive decline in older adults based on blood-derived markers. METHODS:The index was based on 8 markers that have been previously associated with cognitive aging: APOE genotype, plasma β-amyloid 42/40 ratio, telomere length, cystatin C, glucos...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000001263

    authors: Nettiksimmons J,Ayonayon H,Harris T,Phillips C,Rosano C,Satterfield S,Yaffe K,Health ABC Study.

    更新日期:2015-02-17 00:00:00

  • Brain death worldwide: accepted fact but no global consensus in diagnostic criteria.

    abstract:OBJECTIVE:To survey brain death criteria throughout the world. BACKGROUND:The clinical diagnosis of brain death allows organ donation or withdrawal of support. Declaration of brain death follows a certain set of examinations. The code of practice throughout the world has not been systematically investigated. METHODS:...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.58.1.20

    authors: Wijdicks EF

    更新日期:2002-01-08 00:00:00